Rheumatology Flashcards
1
Q
What is rheumatoid arthritis?
A
- An autoimmune condition causing chronic inflammation in the synovial lining of joints, tendon sheaths, and bursae.
- A type of inflammatory arthritis.
2
Q
What are the common symptoms of rheumatoid arthritis?
A
- Joint pain, stiffness, and swelling.
- Typically affects multiple small joints symmetrically (symmetrical polyarthritis).
- Commonly involves metacarpophalangeal (MCP) joints, proximal interphalangeal (PIP) joints, wrists, and metatarsophalangeal (MTP) joints.
- Associated systemic symptoms include fatigue, weight loss, flu-like illness, muscle aches, and weakness.
3
Q
What factors contribute to the development of rheumatoid arthritis?
A
- More common in women (2-3 times) than men.
- Often develops in middle age but can present at any age.
- Risk factors include **smoking and obesity. **
- Family history increases risk; HLA DR4 gene is commonly associated.
4
Q
What is the pathophysiology of rheumatoid arthritis?
A
- Autoimmune response targets the synovial lining, leading to chronic inflammation (synovitis).
- Inflammation extends to tendon sheaths and bursae.
- Persistent inflammation can cause joint damage and deformities.
5
Q
How prevalent is rheumatoid arthritis?
A
- Affects approximately 1% of the global population.
- More prevalent in women than men.
- Commonly presents in middle-aged individuals but can occur at any age.
6
Q
What are the risk factors for rheumatoid arthritis?
A
Female gender
Middle age onset
Smoking
Obesity
Family history of rheumatoid arthritis
Presence of HLA DR4 gene.
7
Q
What are common clinical examination findings in rheumatoid arthritis?
A
- Tenderness and synovial thickening in affected joints, giving a “boggy” feel.
- Symmetrical involvement of small joints, especially hands and feet.
- Possible deformities in advanced cases.
8
Q
What investigations are used to diagnose rheumatoid arthritis?
A
- Blood tests for rheumatoid factor (RF) and anti-cyclic citrullinated peptide (anti-CCP) antibodies. -
- Imaging studies like X-rays to assess joint damage. - MRI or ultrasound may be used for detailed evaluation.
9
Q
What is the significance of rheumatoid factor (RF) in rheumatoid arthritis?
A
- An autoantibody present in about 70% of patients.
Causes immune system activation against the patient’s own IgG, leading to systemic inflammation.
- Most often of the IgM type but can be other immunoglobulin classes.
10
Q
What is the role of anti-cyclic citrullinated peptide (anti-CCP) antibodies in rheumatoid arthritis?
A
- More sensitive and specific than rheumatoid factor. - Positive in approximately 80% of patients.
- Can predate the development of symptoms, indicating a likelihood of developing the condition.
11
Q
What are the differential diagnoses for rheumatoid arthritis?
A
- Osteoarthritis.
- Psoriatic arthritis.
- Systemic lupus erythematosus.
- Gout.
- Reactive arthritis.
12
Q
What are the types of management for rheumatoid arthritis?
A
- Conservative: Patient education, physical therapy, and lifestyle modifications.
- Medical: Disease-modifying antirheumatic drugs (DMARDs), biologic agents, and corticosteroids.
- Surgical: Joint replacement or synovectomy in severe cases.
13
Q
How does rheumatoid arthritis typically present?
A
- Symmetrical distal polyarthritis affecting small joints.
- Gradual onset of joint pain, stiffness, and swelling.
- Morning stiffness lasting more than 30 minutes.
- Systemic symptoms like fatigue and weight loss.
14
Q
What is synovitis?
A
- Inflammation of the synovial membrane lining the joints.
- Leads to joint swelling, pain, and stiffness.
- A hallmark feature of rheumatoid arthritis.
15
Q
How does smoking influence the risk of developing rheumatoid arthritis?
A
- Smoking is a significant risk factor.
- Increases the likelihood of developing the condition.
- May worsen disease severity and reduce treatment effectiveness.
16
Q
What is the typical age of onset for rheumatoid arthritis?
A
- Most commonly develops in middle age.
- Can present at any age.
- More frequent in women aged 40-60 years.
17
Q
How does obesity affect rheumatoid arthritis?
A
- Obesity is a modifiable risk factor.
- Increases the risk of developing the condition.
- May exacerbate symptoms and complicate management.
18
Q
What is the significance of the HLA DR4 gene in rheumatoid arthritis?
A
- The most common gene associated with the condition. - Presence increases susceptibility.
- Suggests a genetic predisposition in affected individuals.
19
Q
What are the systemic manifestations of rheumatoid arthritis?
A
- Fatigue.
- Weight loss.
- Flu-like symptoms.
- Muscle aches and weakness.
- Potential involvement of other organs in severe cases.
20
Q
How does rheumatoid arthritis affect the cervical spine?
A
- Can involve the cervical spine, particularly the atlantoaxial joint.
- May lead to neck pain and stiffness. - Unlike osteoarthritis, it does not affect the lumbar spine.
21
Q
What is the significance of morning stiffness in rheumatoid arthritis?
A
- Morning stiffness lasting more than 30 minutes is characteristic - Symptoms improve with activity -
- Helps differentiate from osteoarthritis, where stiffness worsens with activity.
22
Q
How does rheumatoid arthritis differ from osteoarthritis in joint involvement?
A
- Rheumatoid arthritis: Symmetrical involvement of small joints, sparing distal interphalangeal joints. -
- Osteoarthritis: Often affects weight-bearing joints and distal interphalangeal joints, with asymmetrical involvement.
23
Q
What is the role of imaging in rheumatoid arthritis?
A
- X-rays to assess joint damage and erosions.
- MRI or ultrasound for detailed evaluation of synovitis and early changes.
- Helps in monitoring disease progression and response to treatment.
24
Q
How does family history influence the risk of rheumatoid arthritis?
A
- Family history increases the risk.
- No clear inheritance pattern.
- Suggests a genetic predisposition, especially with HLA DR4 gene association.
25
What is psoriatic arthritis?
- An inflammatory arthritis associated with psoriasis.
- Severity ranges from mild joint stiffness and soreness to severe joint destruction (arthritis mutilans).
26
How common is psoriatic arthritis among patients with psoriasis?
- Occurs in 10-20% of patients with psoriasis.
- Typically develops within 10 years of the onset of psoriasis.
- In some cases, arthritis may precede skin manifestations.
27
What are the common patterns of joint involvement in psoriatic arthritis?
- Asymmetrical oligoarthritis: Affects 1-4 joints, often on one side.
- Symmetrical polyarthritis: Resembles rheumatoid arthritis, involving multiple joints like hands, wrists, and ankles.
- Distal interphalangeal (DIP) predominant: Primarily affects DIP joints.
- Spondylitis: Involves the spine and sacroiliac joints, causing back stiffness and pain.
- Arthritis mutilans: Severe form leading to bone destruction and shortening of digits.
28
What are the typical signs and symptoms of psoriatic arthritis?
- Skin manifestations: Psoriatic plaques.
- Nail changes: Pitting (small indentations) and onycholysis (nail detachment).
- Dactylitis: Inflammation of an entire finger or toe, leading to a "sausage-like" appearance.
- Enthesitis: Inflammation at tendon insertion points, causing localized pain and tenderness.
29
What is the Psoriasis Epidemiological Screening Tool (PEST)?
- A screening questionnaire for psoriatic arthritis in patients with psoriasis.
- Includes questions about joint pain, swelling, history of arthritis, and nail pitting.
- A high score suggests the need for rheumatology referral.
30
What are the characteristic X-ray findings in psoriatic arthritis?
- Periostitis: Inflammation of the periosteum, leading to a thickened and irregular bone outline.
- Ankylosis: Fusion of bones at the joint.
- Osteolysis: Bone destruction.
- Dactylitis: Soft tissue swelling of an entire digit.
- "Pencil-in-cup" deformity: Erosion of bone ends, giving a pencil-in-cup appearance on X-ray.
31
How does psoriatic arthritis differ from rheumatoid arthritis in terms of joint involvement?
- Psoriatic arthritis often affects DIP joints and the axial skeleton (spine and sacroiliac joints).
- Rheumatoid arthritis typically spares DIP joints and primarily involves peripheral joints.
32
What is arthritis mutilans?
- The most severe form of psoriatic arthritis.
- Involves osteolysis of phalanges, leading to shortening of fingers or toes.
- Results in a "telescoping" appearance due to skin folds over shortened digits.
33
What is dactylitis, and how is it related to psoriatic arthritis?
- Inflammation of an entire finger or toe.
- Causes uniform swelling, giving a "sausage-like" appearance.
- Commonly associated with psoriatic arthritis.
34
What is enthesitis, and which areas are commonly affected in psoriatic arthritis?
- Inflammation at the sites where tendons or ligaments insert into bone.
- Common sites include the Achilles tendon insertion and plantar fascia.
- Leads to localized pain and tenderness.
35
How does spondylitis present in psoriatic arthritis?
- Involves inflammation of the spine and sacroiliac joints.
- Causes back stiffness and pain.
- May lead to reduced spinal mobility over time.
36
What nail changes are commonly seen in psoriatic arthritis?
- Nail pitting: Small depressions on the nail surface.
- Onycholysis: Separation of the nail from the nail bed.
- These changes are indicative of psoriatic involvement.
37
What does the "pencil-in-cup" deformity indicate in psoriatic arthritis?
- A specific X-ray finding where the end of a bone becomes pointed (pencil-like) and fits into a cup-shaped erosion on the adjacent bone.
- Indicates severe joint erosion and damage.
- Commonly observed in the fingers.
38
What are the extra-articular manifestations associated with psoriatic arthritis?
- Uveitis: Inflammation of the uveal tract of the eye, causing redness and pain.
- Inflammatory bowel disease: Conditions like Crohn's disease or ulcerative colitis.
- These manifestations highlight the systemic nature of psoriatic arthritis.
39
What are the common risk factors for developing psoriatic arthritis?
- Having psoriasis, especially with severe skin involvement.
- Family history of psoriatic arthritis.
- Certain genetic markers, such as HLA-B27.
- Environmental factors, including infections or physical trauma.
40
What is the typical age of onset for psoriatic arthritis?
- Most commonly develops in middle age.
- Can occur at any age, including childhood.
- Both genders are affected, with a slight predilection for males.
41
How is psoriatic arthritis managed?
Conservative Management:
- Patient education on disease course and flare-ups.
- Exercise and physiotherapy to maintain joint function. - Weight loss to reduce joint stress.
- Smoking cessation to lower inflammation.
Medical Management:
- Nonsteroidal anti-inflammatory drugs (NSAIDs): First-line for mild cases to relieve pain and stiffness.
- Disease-modifying antirheumatic drugs (DMARDs): Methotrexate, sulfasalazine, or leflunomide for moderate to severe disease.
- Biologic agents: TNF inhibitors (e.g., etanercept, adalimumab) and IL-17 inhibitors (e.g., secukinumab) for cases unresponsive to DMARDs.
- JAK inhibitors: Tofacitinib as an alternative in severe cases.
- Corticosteroids: Used sparingly for short-term flare control, as they may trigger psoriasis flares.
Surgical Management: Reserved for severe joint damage or deformities.
- Joint replacement (e.g., hip or knee) for end-stage disease.
42
What role do genetic factors play in psoriatic arthritis?
- Genetic predisposition plays a significant role.
- Strong association with **HLA-B27,** particularly in cases with axial involvement.
- Other genes, such as HLA-Cw6, have been linked to both psoriasis and psoriatic arthritis.
- A family history of psoriasis or psoriatic arthritis increases the risk.
43
What is osteoarthritis?
- Often described as "wear and tear" in the joints.
- Affects synovial joints.
- Results from genetic factors, overuse, and injury.
44
What are the common symptoms of osteoarthritis?
- Joint pain and stiffness.
- Worsens with activity and at the end of the day.
- Deformity, instability, and reduced function of the joint.
45
What factors contribute to the development of osteoarthritis?
- Imbalance between cartilage damage and chondrocyte response.
- Leads to structural joint issues.
46
Which joints are most commonly affected by osteoarthritis?
- Hips.
- Knees.
- Distal interphalangeal (DIP) joints in the hands.
- Carpometacarpal (CMC) joint at the base of the thumb.
- Lumbar spine.
- Cervical spine (cervical spondylosis).
47
What are the key X-ray changes associated with osteoarthritis?
- LOSS mnemonic:
- Loss of joint space.
- Osteophytes (bone spurs).
- Subarticular sclerosis (increased bone density along the joint line).
- Subchondral cysts (fluid-filled holes in the bone).
48
How do X-ray findings correlate with osteoarthritis symptoms?
- X-ray changes do not necessarily correlate with symptoms.
- A patient may have significant X-ray changes but minimal symptoms, or vice versa.
49
What are Heberden's nodes?
- Bony enlargements found in the distal interphalangeal (DIP) joints of the hands.
- Indicative of osteoarthritis.
50
What are Bouchard's nodes?
- Bony enlargements found in the proximal interphalangeal (PIP) joints of the hands.
- Associated with osteoarthritis.
51
What is the significance of squaring at the base of the thumb in osteoarthritis?
- Indicates osteoarthritis affecting the carpometacarpal (CMC) joint.
52
What are common clinical examination findings in osteoarthritis?
- Bulky, bony enlargement of the joint.
- Restricted range of motion.
- Crepitus on movement.
- Effusions (fluid) around the joint.
53
How does osteoarthritis pain typically present?
- Pain worsens with activity and at the end of the day.
- Unlike inflammatory arthritis, which is worse in the morning and improves with activity.
54
What is the NICE guideline for diagnosing osteoarthritis without investigations?
- Diagnosis can be made if:
- The patient is over 45.
- Has typical pain associated with activity.
- Has no morning stiffness or stiffness lasting under 30 minutes.
55
What non-pharmacological management strategies are recommended for osteoarthritis?
- Patient education.
- Therapeutic exercise to improve strength and function. - Weight loss if overweight.
- Occupational therapy for support in daily activities.
56
What is the first-line pharmacological treatment for osteoarthritis according to NICE guidelines?
- Topical NSAIDs are recommended as the first-line treatment.
57
What role does weight loss play in managing osteoarthritis?
- Reduces load on weight-bearing joints.
- May decrease pain and improve function.
58
How does therapeutic exercise benefit osteoarthritis patients?
- Helps improve strength and function.
- Reduces pain.
59
What is the purpose of occupational therapy in osteoarthritis management?
- Supports daily activities and function.
- May include walking aids and home adaptations.
60
What are osteophytes?
- Bone spurs that develop at the edges of a joint affected by osteoarthritis.
61
What is subarticular sclerosis?
- Increased bone density along the joint line, visible on X-rays.
62
What are subchondral cysts?
- Fluid-filled holes that develop in the bone beneath the cartilage in osteoarthritic joints.
63
How does morning stiffness in osteoarthritis differ from that in inflammatory arthritis?
- In osteoarthritis: - Morning stiffness is either absent or lasts less than 30 minutes.
- In inflammatory arthritis: - Stiffness is more prolonged and severe in the morning.
64
What is crepitus, and how is it related to osteoarthritis?
- Crackling or grating sound/sensation in a joint.
- Often present in osteoarthritic joints during movement.
65
Why might a patient with hip osteoarthritis experience knee pain?
- Pain from hip osteoarthritis can be referred to the knee.
- Leads to perceived knee pain.
66
What is the typical age group affected by osteoarthritis?
- Commonly affects individuals over the age of 45.
67
How does occupational activity influence the risk of developing osteoarthritis?
- Repetitive joint use or heavy manual labour increases the risk.
68
What is reactive arthritis?
- An inflammatory joint condition (synovitis) occurring in response to an infection elsewhere in the body.
- Typically presents as acute monoarthritis, often affecting a single joint like the knee.
- Characterized by a warm, swollen, and painful joint without direct infection in the joint itself.
69
What are the common triggers for reactive arthritis?
- Gastroenteritis: Infections of the gastrointestinal tract. - Sexually transmitted infections (STIs): Particularly chlamydia.
- Note: Gonorrhoea more commonly leads to septic arthritis rather than reactive arthritis.
70
What is the association between reactive arthritis and HLA-B27?
- Reactive arthritis is classified as a seronegative spondyloarthropathy.
- There is a significant link with the HLA-B27 gene.
- Patients with the HLA-B27 gene have a higher predisposition to developing reactive arthritis.
71
What are the typical clinical features of reactive arthritis?
- Joint involvement: Acute onset of a warm, swollen, and painful joint, commonly the knee.
- Ocular symptoms: Bilateral non-infective conjunctivitis and anterior uveitis.
- Urogenital symptoms: Non-gonococcal urethritis. - Dermatological manifestation: Circinate balanitis (dermatitis of the glans penis).
72
What mnemonic helps recall the classic triad of reactive arthritis symptoms?
- "Can't see, pee, or climb a tree": Refers to conjunctivitis (can't see), urethritis (can't pee), and arthritis (can't climb a tree).
73
How is reactive arthritis diagnosed?
- Primarily a clinical diagnosis based on history and presentation.
- Important to exclude other causes of acute arthritis, especially septic arthritis.
- Joint aspiration and synovial fluid analysis are essential to rule out infection and crystal arthropathies.
74
What are the key differential diagnoses for reactive arthritis?
- Septic arthritis: Infection within the joint space.
- Gout: Deposition of urate crystals in joints. - Pseudogout: Deposition of calcium pyrophosphate crystals.
- Other spondyloarthropathies: Such as ankylosing spondylitis and psoriatic arthritis.
75
What is the role of joint aspiration in managing reactive arthritis?
- Essential for excluding septic arthritis.
- Synovial fluid is analyzed for: - Microscopy: To detect the presence of white blood cells and crystals.
- Culture and sensitivity: To identify any bacterial growth.
76
How is the triggering infection in reactive arthritis managed?
- Antibiotic therapy: Appropriate antibiotics are prescribed to treat the underlying infection, such as chlamydia.
- Note: Treating the infection does not always alleviate the arthritis symptoms but is crucial to prevent further complications.
77
What are the mainstays of symptomatic treatment for reactive arthritis?
- Nonsteroidal anti-inflammatory drugs (NSAIDs): - First-line for reducing pain and inflammation.
- Examples include ibuprofen and naproxen.
- Corticosteroids: - Intra-articular injections: Directly into the affected joint for localized relief.
- Systemic steroids: Oral or intravenous administration, especially when multiple joints are involved.
78
When are Disease-Modifying Antirheumatic Drugs (DMARDs) considered in reactive arthritis?
- In cases where symptoms persist beyond six months. - For recurrent episodes of reactive arthritis.
- Common DMARDs include sulfasalazine and methotrexate.
79
What is the prognosis for most patients with reactive arthritis?
- Majority experience resolution of symptoms within six months.
- Recurrences are possible but not common.
- Chronic arthritis or eye complications can occur in a minority of patients.
80
Why is it important to exclude HIV in patients presenting with reactive arthritis?
- Reactive arthritis is more prevalent in individuals with HIV.
- Ensuring accurate diagnosis and management of underlying conditions is crucial.
81
What is circinate balanitis, and how is it related to reactive arthritis?
- A dermatological condition characterized by painless, serpiginous dermatitis of the glans penis.
- Considered a specific manifestation of reactive arthritis.
82
How does anterior uveitis present in reactive arthritis?
- Inflammation of the anterior chamber of the eye.
- Symptoms include eye pain, redness, blurred vision, and photophobia.
- Requires prompt ophthalmological assessment and treatment.
83
What is the significance of the HLA-B27 gene in reactive arthritis?
- Presence of HLA-B27 increases susceptibility to reactive arthritis.
- Associated with more severe and prolonged disease courses.
84
What are the potential extra-articular manifestations of reactive arthritis?
- Ocular: Conjunctivitis and uveitis.
- Dermatological: Circinate balanitis and keratoderma blennorrhagicum (hyperkeratotic skin lesions).
- Urogenital: Urethritis and prostatitis.
- Gastrointestinal: Mild enteritis.
85
How does reactive arthritis differ from septic arthritis?
- Reactive arthritis -->No infection within the joint space.
- Triggered by an infection elsewhere in the body.
- Septic arthritis --> Direct infection of the joint space.
- Requires immediate antibiotic therapy and often surgical intervention.
86
What is the typical age group affected by reactive arthritis?
- Most commonly affects adults aged 20-40 years.
- Can occur in both men and women, though post-venereal cases are more common in men.
87
How does conjunctivitis present in reactive arthritis?
- Typically bilateral and non-infective.
- Symptoms include redness, irritation, and discharge.
- Usually self-limiting but may require topical treatments for comfort.
88
What is the role of physiotherapy in managing reactive arthritis?
- Helps maintain joint function and muscle strength. - Prevents stiffness and promotes mobility.
- Tailored exercise programs can aid in recovery and prevent chronicity.
89
Can reactive arthritis become a chronic condition?
- While most cases resolve within six months, a subset of patients may develop chronic symptoms.
- Chronic reactive arthritis may require long-term management strategies, including DMARDs.
90
What lifestyle modifications can aid in managing reactive arthritis?
- Rest during acute phases: To reduce joint stress.
- Regular exercise: To maintain joint mobility and muscle strength.
- Smoking cessation: Smoking may exacerbate symptoms and delay recovery.
- Balanced diet: Supports overall health and immune function.
91
Why is patient education important in reactive arthritis management?
- Empowers patients to manage symptoms effectively. - Enhances adherence to treatment plans.
- Increases awareness of potential triggers and preventive measures.
92
What follow-up care is recommended for patients with reactive arthritis?
- Regular monitoring to assess symptom progression and treatment efficacy.
- Ophthalmological evaluations if ocular symptoms are present.
- Continuous evaluation for potential
93
What is ankylosing spondylitis?
- An inflammatory condition affecting the axial skeleton, primarily the spine and sacroiliac joints.
- Causes progressive stiffness and pain.
- Also known as axial spondyloarthritis.
- Part of the seronegative spondyloarthropathy group, which includes psoriatic arthritis and reactive arthritis.
94
Which joints are primarily affected in ankylosing spondylitis?
- Sacroiliac joints. - Vertebral column joints. - Inflammation in these joints leads to pain and stiffness.
- Can progress to fusion of the spine and sacroiliac joints.
95
What is the association between ankylosing spondylitis and the HLA-B27 gene?
- Strong link: approximately 90% of patients with ankylosing spondylitis possess the HLA-B27 gene.
- However, less than 10% of individuals with the HLA-B27 gene develop the condition.
- More prevalent in men, but women can also be affected.
96
What are the typical presenting features of ankylosing spondylitis?
- Usually presents in young adult males in their 20s. - Symptoms develop gradually over at least three months.
- Main features: - Lower back pain and stiffness. - Sacroiliac pain (buttock region).
- Pain and stiffness worsen with rest and improve with movement.
- Symptoms are more pronounced at night and in the morning, potentially causing nocturnal awakenings.
- Morning stiffness lasts at least 30 minutes.
97
What additional symptoms may be associated with ankylosing spondylitis?
- Chest pain related to costovertebral and sternocostal joint involvement.
- Enthesitis: inflammation where tendons or ligaments insert into bone.
- Dactylitis: inflammation of an entire finger or toe.
- Vertebral fractures, presenting with sudden-onset neck or back pain.
- Shortness of breath due to restricted chest wall movement.
98
What mnemonic can help recall the key associations of ankylosing spondylitis?
The "5 As" mnemonic:
- Anterior uveitis.
- Aortic regurgitation.
- Atrioventricular block (heart block)
- Apical lung fibrosis (upper lobe lung fibrosis)
- Anaemia of chronic disease.
99
What is Schober's test, and how is it performed?
- A clinical assessment of spinal mobility.
Procedure: - With the patient standing straight, locate the L5 vertebra.
- Mark a point 10 cm above and 5 cm below this level (total 15 cm apart).
- Ask the patient to bend forward maximally and measure the distance between the two points.
- A distance of less than **20 cm** indicates restricted lumbar movement, supporting a diagnosis of ankylosing spondylitis.
100
What investigations are essential in diagnosing ankylosing spondylitis?
Inflammatory markers:
- Elevated C-reactive protein (CRP)
- erythrocyte sedimentation rate (ESR) may indicate disease activity.
- HLA-B27 genetic testing:
- X-rays of the spine and sacrum to detect structural changes.
- MRI of the spine can reveal bone marrow oedema
101
What are the characteristic X-ray findings in ankylosing spondylitis?
- In advanced stages, a "bamboo spine" appearance due to fusion of the sacroiliac and spinal joints.
- Early changes may include: - Sacroiliitis: inflammation of the sacroiliac joints.
- Squaring of vertebral bodies.
- Syndesmophytes: bony growths originating inside a ligament.
102
What are the primary goals in managing ankylosing spondylitis?
- Relieve symptoms.
- Maintain spinal flexibility and posture.
- Prevent or minimize complications.
- Enhance quality of life.
103
What conservative management strategies are employed for ankylosing spondylitis?
- Exercise and physiotherapy: - Regular exercises to maintain posture and spinal mobility.
- Strengthening exercises for back and abdominal muscles.
- Swimming is particularly beneficial due to its low-impact nature.
- Postural training: - Encourages proper alignment to prevent deformities.
- Patient education: - Informing patients about the disease process and self-management techniques.
104
What medical treatments are available for ankylosing spondylitis?
- Nonsteroidal anti-inflammatory drugs (NSAIDs): - First-line treatment to reduce pain and inflammation. - Examples: ibuprofen, naproxen.
- Tumour necrosis factor (TNF) inhibitors: - Biologic agents used when NSAIDs are insufficient.
- Examples: etanercept, infliximab. - Interleukin-17 (IL-17) inhibitors:
- Another class of biologics for patients not responding to TNF inhibitors.
- Example: secukinumab. - Analgesics: - Additional pain relief as needed.
105
Are there any surgical interventions for ankylosing spondylitis?
- Joint replacement surgery: - Indicated for severe joint damage, particularly in the hips.
- Spinal surgery: - Rarely, procedures to correct severe spinal deformities or fractures.
- Surgery is generally considered when conservative and medical treatments fail to manage symptoms adequately.
106
What lifestyle modifications can benefit individuals with ankylosing spondylitis?
- Smoking cessation:
- Smoking can exacerbate symptoms and increase the risk of complications.
- Balanced diet: - Maintaining a healthy weight reduces stress on joints.
- Regular physical activity: - Encourages flexibility and reduces stiffness.
- Proper sleep hygiene: - Ensures adequate rest and reduces fatigue.
107
How does anterior uveitis present in ankylosing spondylitis?
- Eye pain - Redness - Blurred vision - Photophobia (sensitivity to light).
- Requires prompt ophthalmological evaluation and treatment to prevent complications.
108
What cardiovascular complications are associated with ankylosing spondylitis?
- Aortic regurgitation: - Backflow of blood from the aorta into the left ventricle due to valve dysfunction.
- Atrioventricular (AV) block: - Impaired electrical conduction between the atria and ventricles, potentially leading to arrhythmias.
109
Why is early diagnosis and management crucial in ankylosing spondylitis?
- To prevent or slow the progression of spinal fusion and deformity.
- To maintain functional abilities and quality of life.
- Early intervention can reduce the risk of complications and improve long-term outcomes.
110
What role does MRI play in the diagnosis of ankylosing spondylitis?
- MRI is more sensitive than X-rays for early disease detection.
- Can identify bone marrow oedema in the sacroiliac joints before structural changes occur.
- Helps confirm the diagnosis in patients with suggestive symptoms but normal X-rays.
111
What is Systemic Lupus Erythematosus (SLE)?
- An inflammatory autoimmune connective tissue disorder.
- Termed "systemic" due to its impact on multiple organs and systems.
- "Erythematosus" refers to the characteristic red malar rash across the face.
112
Which populations are more commonly affected by SLE?
- Predominantly affects women.
- Higher prevalence in individuals of Asian, African, Caribbean, and Hispanic descent.
- Typically presents in young to middle-aged adults.
113
What is the typical course of SLE?
- Follows a relapsing-remitting pattern.
- Periods of symptom exacerbation (flares) alternate with periods of symptom remission.
- Chronic inflammation can reduce life expectancy, with cardiovascular disease and infections being significant complications.
114
What is the underlying pathophysiology of SLE?
- Characterized by the presence of anti-nuclear antibodies (ANA).
- These autoantibodies target proteins within the cell nucleus.
- The resulting chronic inflammatory response leads to the diverse manifestations of the disease.
115
What are common clinical features of SLE?
General symptoms:
- Fatigue.
- Weight loss.
- Fever.
- Musculoskeletal:
- Arthralgia (joint pain).
- Non-erosive arthritis.
- Myalgia (muscle pain).
Dermatological:
- Photosensitive malar rash ("butterfly" rash across the nose and cheeks).
- Hair loss.
- Mouth ulcers.
Hematological:
- Lymphadenopathy.
- Splenomegaly.
Cardiopulmonary:
- Shortness of breath.
- Pleuritic chest pain.
Renal:
- Edema (indicative of nephritis).
Vascular:
- Raynaud’s phenomenon.
116
What investigations are essential in diagnosing SLE?
Autoantibodies:
- Anti-nuclear antibodies (ANA).
- Anti-double stranded DNA (anti-dsDNA) antibodies.
- Extractable nuclear antigen panel (e.g., Anti-Sm, Anti-Ro, Anti-La).
Blood tests:
- Full blood count (may reveal anemia, leukopenia, thrombocytopenia).
- Elevated ESR and CRP during active inflammation.
- Decreased C3 and C4 complement levels in active disease.
Urinalysis:
- Detects proteinuria, suggesting lupus nephritis.
- Renal biopsy:
- Assesses the extent of renal involvement in lupus nephritis.
117
What is the significance of anti-dsDNA antibodies in SLE?
Highly specific for SLE, aiding in diagnosis.
- Present in approximately 50% of SLE patients.
- Levels correlate with disease activity, making them useful for monitoring disease progression and treatment response.
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What are the primary goals in managing SLE?
- Control symptoms and induce remission.
- Prevent disease flares.
- Minimize organ damage and complications.
- Enhance quality of life.
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What general lifestyle modifications are recommended for SLE patients?
- Sun protection:
- Use of broad-spectrum sunscreens.
- Wearing protective clothing.
- Avoiding excessive sun exposure to prevent photosensitive rashes.
Smoking cessation: - Smoking can exacerbate symptoms and increase cardiovascular risk.
Regular exercise: - Maintains joint function and reduces fatigue.
Balanced diet: - Supports overall health and immune function.
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What pharmacological treatments are commonly used in SLE?
Non-steroidal anti-inflammatory drugs (NSAIDs):
- Alleviate joint and muscle pain.
Hydroxychloroquine:
- An antimalarial drug that reduces disease activity and flares.
- Regular ophthalmologic exams are necessary due to potential retinal toxicity.
- Corticosteroids: - Used for acute flares to control inflammation. - Dose and duration are minimized to reduce side effects.
- Immunosuppressive agents: Examples include methotrexate, azathioprine, and mycophenolate mofetil.
- Employed in cases with significant organ involvement or when disease is refractory to other treatments.
- Biologic agents: - Belimumab is approved for SLE and targets B-lymphocyte stimulator (BLyS). - Considered in patients with active, autoantibody-positive SLE despite standard therapy.
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What are potential complications associated with SLE?
- Renal involvement: - Lupus nephritis can progress to renal failure if untreated.
- Cardiovascular disease: - Increased risk due to chronic inflammation and potential side effects of medications.
- Infections: - Immunosuppressive therapy elevates the risk of opportunistic infections.
- Neuropsychiatric manifestations: - Cognitive dysfunction, seizures, psychosis.
- Hematological abnormalities: - Anemia, thrombocytopenia, leukopenia.
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How is lupus nephritis monitored and managed?
- Monitoring: - Regular urinalysis to detect proteinuria.
- Periodic assessment of renal function (e.g., serum creatinine, glomerular filtration rate).
- Management: - Immunosuppressive therapy tailored to the severity of renal involvement.
- Agents include corticosteroids, mycophenolate mofetil, cyclophosphamide, and azathioprine.
- Blood pressure control is crucial, often requiring antihypertensive medications.
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What is the relationship between SLE and antiphospholipid syndrome (APS)?
- APS can occur secondary to SLE.
- Characterized by antiphospholipid antibodies leading to increased risk of venous and arterial thrombosis.
- Associated with complications such as recurrent miscarriages and thrombocytopenia.
- Management includes anticoagulation therapy to prevent thrombotic events.
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Why is regular follow-up essential for SLE patients?
- To monitor disease activity and detect flares early.
- Assess and manage medication side effects.
- Screen for complications, especially cardiovascular and renal.
- Adjust treatment plans based on disease progression and patient response.
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What role do vaccinations play in the management of SLE?
- Immunosuppressive therapy increases infection risk.
- Vaccinations, such as annual influenza and pneumococcal vaccines, are recommended.
- Live vaccines are generally avoided in immunocompromised patients.
- Timing of vaccinations should be coordinated to ensure optimal efficacy and safety.
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How does SLE affect pregnancy, and what precautions are necessary?
- SLE increases the risk of complications like pre-eclampsia, preterm birth, and fetal growth restriction.
- Disease should be well-controlled for at least six months before conception.
- Certain medications (e.g., methotrexate, cyclophosphamide) are teratogenic and must be discontinued prior to pregnancy
127
What is Discoid Lupus Erythematosus (DLE)?
- A chronic autoimmune skin condition characterized by inflamed, scaly plaques.
- Most common form of cutaneous lupus erythematosus.
- Primarily affects the face, scalp, and ears.
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Which populations are more commonly affected by DLE?
- More prevalent in women, especially between ages 20-50.
- Higher incidence in individuals with darker skin tones.
- Increased occurrence in smokers.
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What are the typical clinical features of DLE?
- Lesion characteristics: - Erythematous (red) plaques.
- Dry and scaly surface.
- Well-defined borders. -
Distribution: Predominantly on sun-exposed areas: face, scalp, and ears.
- Associated changes:
- Scarring alopecia (permanent hair loss in affected areas).
- Pigmentary alterations: hyperpigmentation or hypopigmentation.
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What factors can exacerbate DLE lesions?
- Sun exposure: - Lesions are photosensitive and worsen with ultraviolet (UV) light.
- Environmental triggers: - Trauma to the skin.
- Stress.
- Infections.
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How is DLE diagnosed?
- Clinical evaluation: - Assessment of characteristic skin lesions and distribution.
- Skin biopsy: - Histopathological examination confirms diagnosis.
- Laboratory tests: - Antinuclear antibody (ANA) testing to evaluate for systemic involvement.
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What is the relationship between DLE and systemic lupus erythematosus (SLE)?
- DLE is primarily a cutaneous condition.
- Less than 5% of DLE patients develop SLE.
- Regular monitoring is essential to detect any systemic involvement early.
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What are the primary goals in managing DLE?
- Symptom control: - Reduce lesion activity and prevent progression.
- Prevent complications: Minimize scarring and pigmentary changes.
- Enhance quality of life: Address cosmetic concerns and associated psychosocial impacts.
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What general measures are recommended for DLE patients?
- Sun protection: - Use broad-spectrum sunscreens (SPF 50+).
- Wear protective clothing and wide-brimmed hats.
- Avoid peak sun exposure hours (10 a.m. to 4 p.m.).
- Smoking cessation: - Smoking is a known risk factor and can exacerbate DLE.
135
What topical treatments are commonly used in DLE?
- Topical corticosteroids: - Reduce inflammation and lesion activity.
- Potency and duration tailored to lesion severity and location.
- Topical calcineurin inhibitors (e.g., tacrolimus, pimecrolimus): - Alternative to steroids, especially for facial lesions.
- Lower risk of skin atrophy with prolonged use.
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When are intralesional steroid injections indicated in DLE?
- For thickened or hypertrophic lesions unresponsive to topical therapies.
- Triamcinolone acetonide is commonly used.
- Administered with caution to minimize skin atrophy and other side effects.
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What systemic treatments are considered for DLE?
- Antimalarial agents: - **Hydroxychloroquine** is the first-line systemic treatment.
- Regular ophthalmologic monitoring is essential due to potential retinal toxicity.
- Immunosuppressive agents: - Considered in refractory cases or when antimalarials are contraindicated.
- Options include methotrexate, azathioprine, or mycophenolate mofetil.
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What are potential complications associated with DLE?
- Scarring and disfigurement: - Permanent cosmetic changes, especially on the face and scalp.
- **Squamous cell carcinoma (SCC): -** Chronic lesions, particularly on the scalp and ears, have a rare potential to develop into SCC.
- Psychosocial impact: - Emotional distress due to visible skin changes.
139
Why is regular follow-up essential for DLE patients?
- Monitor treatment efficacy and adjust therapies as needed. - Early detection of potential progression to systemic involvement. - Surveillance for complications, including skin malignancies.
140
What lifestyle modifications can aid in managing DLE?
- Stress management: - Engage in relaxation techniques and counseling if needed.
- Healthy diet and exercise: - Support overall well-being and immune function.
- Avoidance of known triggers: - Identify and minimize exposure to factors that exacerbate lesions.
141
How does smoking affect DLE, and what advice should be given?
- Smoking is associated with increased lesion activity and reduced treatment efficacy.
- Strongly advise cessation and provide resources to support quitting.
142
What role does patient education play in DLE management?
- Empower patients with knowledge about their condition.
- Emphasize the importance of adherence to sun protection and treatment regimens.
- Provide guidance on recognizing early signs of flare-ups or complications.
143
What is Systemic Sclerosis (SSc)?
- A rare autoimmune connective tissue disorder characterized by fibrosis of the skin and internal organs.
- Involves vascular abnormalities and immune system activation.
- Also known as scleroderma, which specifically refers to skin hardening.
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What are the main subtypes of SSc?
- Limited Cutaneous Systemic Sclerosis: - Skin involvement distal to elbows and knees; face and neck may be affected.
- Associated with CREST syndrome
- Diffuse Cutaneous Systemic Sclerosis: - Widespread skin involvement, including the trunk and proximal limbs.
- Higher risk of internal organ involvement, such as interstitial lung disease and renal crisis.
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What are common clinical features of SSc?
Skin manifestations:
- Thickening and hardening (scleroderma).
- Sclerodactyly (tightening of skin on fingers).
- Digital ulcers.
Vascular manifestations:
- Raynaud's phenomenon.
- Telangiectasia.
- Gastrointestinal involvement:
Esophageal dysmotility leading to dysphagia and reflux. - Malabsorption due to intestinal involvement.
- Pulmonary involvement:
- Interstitial lung disease causing dyspnea and cough.
- Pulmonary arterial hypertension.
- Renal involvement: - Scleroderma renal crisis characterized by acute renal failure and severe hypertension.
- Musculoskeletal involvement:
- Joint contractures. - Myopathy.
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What are the primary goals in managing SSc?
Symptom control: - Alleviate manifestations such as Raynaud's phenomenon and skin tightness.
- Prevent disease progression: - Monitor and address internal organ involvement early.
- Improve quality of life: - Provide supportive care and address psychosocial aspects.
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What general measures are recommended for SSc patients?
- Sun protection: - Use broad-spectrum sunscreens to prevent skin damage.
- Skin care: - Regular moisturizing to prevent dryness and ulceration.
- Smoking cessation: - Smoking can exacerbate vascular symptoms.
- Physical therapy: - Maintain joint mobility and prevent contractures.
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How is Raynaud's phenomenon managed in SSc?
- Lifestyle modifications: - Keep extremities warm. - Avoid cold exposure and stress.
- Pharmacological treatments: - Calcium channel blockers (e.g., nifedipine) to dilate blood vessels. - Phosphodiesterase-5 inhibitors (e.g., sildenafil) for severe cases.
149
What treatments are available for skin manifestations in SSc?
- Topical therapies: - Emollients to maintain skin hydration.
- Systemic therapies: - Immunosuppressants (e.g., methotrexate) to reduce skin thickening.
- Antifibrotic agents under investigation.
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How is gastrointestinal involvement managed in SSc?
- Esophageal dysmotility: - Proton pump inhibitors for reflux.
- Prokinetic agents to enhance motility.
- Malabsorption: - Nutritional support and supplementation.
- Antibiotics for bacterial overgrowth.
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What is the approach to managing pulmonary complications in SSc?
Interstitial lung disease (ILD): - Regular pulmonary function tests for monitoring.
- Immunosuppressive therapy (e.g., mycophenolate mofetil) to slow progression.
- Pulmonary arterial hypertension (PAH): - Vasodilator therapies (e.g., endothelin receptor antagonists, phosphodiesterase-5 inhibitors).
- Referral to specialized centers for advanced therapies.
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How is scleroderma renal crisis managed?
Early recognition: - Monitor blood pressure regularly. - Watch for signs of renal impairment.
- Pharmacological intervention: - Immediate initiation of ACE inhibitors (e.g., captopril) to control blood pressure.
- Avoid high-dose corticosteroids, which may precipitate renal crisis.
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What role do immunosuppressive agents play in SSc management?
- Indications: - Significant skin involvement.
- Internal organ involvement, particularly lungs.
- Common agents: - Methotrexate for skin disease.
- Mycophenolate mofetil for interstitial lung disease.
- Cyclophosphamide for severe, progressive cases.
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Why is regular monitoring essential in SSc?
Early detection of complications:
- Regular assessments of lung, heart, and kidney function.
- Guiding treatment adjustments:
- Tailoring therapies based on disease progression and response.
Improving outcomes: - Proactive management of emerging issues to prevent irreversible damage.
155
What lifestyle modifications can aid in managing SSc?
- Dietary adjustments: - Small, frequent meals to manage gastrointestinal symptoms.
- Avoidance of caffeine and alcohol, which can exacerbate reflux.
- Exercise: - Regular, gentle exercises to maintain mobility and reduce stiffness.
- Stress management: - Techniques such as meditation and counseling to cope with chronic illness.
156
What are potential complications associated with SSc?
- Cardiac involvement:
- Arrhythmias.
- Heart failure due to myocardial fibrosis.
- Dental issues: - Reduced oral aperture leading to difficulties in dental care.
- Psychosocial impact: - Depression and anxiety related to chronic disease and cosmetic changes.
157
How does SSc affect pregnancy, and what precautions are necessary?
- Risks: - Increased likelihood of preterm birth and hypertensive disorders.
- Management: - Pre-pregnancy counseling, Close monitoring by a multidisciplinary team.
- Adjustment of medications to those safe in pregnancy.
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What is the importance of patient education in SSc?
- Empowerment: - Understanding the disease enables active participation in care.
- Adherence: - Knowledge of treatment benefits encourages compliance.
- Early intervention: - Recognizing early signs of complications leads to prompt medical attention.
159
What is Polymyalgia Rheumatica (PMR)?
- An inflammatory disorder causing muscle pain and stiffness, primarily in the shoulders and pelvic girdle.
- Frequently associated with giant cell arteritis (GCA).
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Who is most commonly affected by PMR?
- Affects people over 50, with a higher prevalence in those aged 70-80.
- More common in women and those of Northern European descent.
161
What are the classic symptoms of PMR?
- Pain and stiffness in the shoulders, hips, neck, and pelvis.
- Morning stiffness lasting more than 30 minutes.
- Fatigue, weight loss, and mild fever.
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How does the pain in PMR typically present?
- Symmetrical pain in the shoulders and hips.
- Pain worsens after rest or in the morning.
- Relieves with movement, especially after warm-up.
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What other systemic symptoms are associated with PMR?
- Low-grade fever.
- Anorexia and weight loss.
- Mild depression and fatigue.
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What is the main risk factor for developing PMR?
- Age: Most common in individuals over 50 years old. - Gender: More common in women.
- Ethnicity: Higher prevalence in those of Northern European descent.
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What are the key diagnostic features of PMR?
- Clinical presentation with typical symptoms of pain and stiffness.
- Elevated ESR and CRP (inflammatory markers).
- No other underlying conditions explaining the symptoms.
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What is the gold standard for diagnosing PMR?
- There is no definitive test, but diagnosis is mainly clinical based on symptoms and exclusion of other conditions.
- Response to steroids is considered a diagnostic criterion (improvement within 1-2 days).
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What is the first-line treatment for PMR?
- Prednisolone 15mg daily.
- Significant improvement is typically seen within 1 week.
- Tapering of the dose is required after symptom control.
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How is the dose of Prednisolone adjusted in PMR treatment?
- Start with 15mg daily, gradually reduce by 2.5mg every 2-4 weeks.
- Aim to reduce the dose to the lowest effective level (typically 5-10mg daily).
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What are the possible side effects of long-term steroid use in PMR?
- Osteoporosis (risk of fractures).
- Increased risk of infection.
- Hyperglycemia, leading to potential steroid-induced diabetes.
- Weight gain, hypertension, and mood changes.
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How can the side effects of steroids be minimized in PMR treatment?
- Calcium and vitamin D supplementation for bone health.
- Bisphosphonates may be considered for osteoporosis prevention.
- Regular monitoring of blood pressure, glucose levels, and weight.
171
What other treatments are used in PMR if steroids are not effective?
- Methotrexate: Sometimes used as a steroid-sparing agent, particularly in severe or relapsing cases. - TNF-alpha inhibitors (e.g., etanercept) for refractory cases.
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What are the complications associated with PMR?
- Giant cell arteritis (GCA): Can cause blindness if untreated. - Relapse of symptoms: Often occurs when tapering steroids too quickly.
173
What is the relationship between PMR and Giant Cell Arteritis (GCA)?
- Up to 40% of PMR patients develop GCA. - GCA is characterized by inflammation of large vessels (e.g., temporal arteries) and can cause blindness if untreated.
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How should PMR be monitored during treatment?
- Regular clinical reviews to assess symptom control and adjust the steroid dose.
- Monitoring inflammatory markers (ESR, CRP) every 2-4 weeks.
- Regular bone health checks (e.g., DEXA scan).
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When should a patient with PMR be referred to a specialist?
- If there is no response to initial steroid treatment. - If there is concern for giant cell arteritis (e.g., temporal headaches, vision problems). - In cases of complicated management or severe side effects from treatment.
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What other conditions should be considered in the differential diagnosis of PMR?
- Rheumatoid arthritis: Joint involvement but not as prominent in the shoulders or hips.
- Osteoarthritis: Joint pain and stiffness but no significant elevation of inflammatory markers.
- Fibromyalgia: Widespread muscle pain without inflammatory markers. - Infections: May cause systemic symptoms similar to PMR.
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What is the role of imaging in diagnosing PMR?
- Imaging is typically not required for diagnosis but may help rule out other conditions. - Ultrasound of the shoulder or hip may show bursitis or synovitis.
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What are the possible long-term outcomes for patients with PMR?
- Most patients have good prognosis with appropriate treatment. - Relapses are common, requiring further steroid treatment. - Chronic low-dose steroids may be needed in some cases to prevent relapse.
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Can PMR resolve on its own?
- PMR typically requires treatment with steroids. - Without treatment, symptoms may persist or worsen over time.
180
What advice should be given to PMR patients regarding exercise?
- Encourage gentle physical activity to maintain mobility and reduce stiffness. - Avoid strenuous activities that could worsen symptoms.
181
What is the importance of patient education in PMR management?
- Educate patients on the importance of steroid adherence and tapering to avoid relapses. - Stress the need for sun protection and bone health maintenance. - Provide guidance on recognizing symptoms of giant cell arteritis (e.g., headache, vision loss).
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What is Giant Cell Arteritis (GCA)?
A systemic vasculitis affecting medium and large arteries, commonly the temporal artery. Strongly linked with polymyalgia rheumatica. More prevalent in older white patients.
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What is the primary complication of GCA?
Vision loss, which is often irreversible.
184
What are the common symptoms of GCA?
- Unilateral severe headache, often around the temple and forehead. - Scalp tenderness. - Jaw claudication. - Blurred or double vision. - Vision loss if untreated.
185
What signs may be present upon physical examination of GCA?
- Tender and thickened temporal artery with reduced or absent pulsation.
- Symptoms of polymyalgia rheumatica (e.g., shoulder and pelvic girdle pain and stiffness).
- Systemic symptoms like weight loss, fatigue, and low-grade fever.
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How is GCA diagnosed?
- Clinical presentation. - Elevated inflammatory markers, particularly ESR (usually more than 50 mm/hour).
- Temporal artery biopsy showing multinucleated giant cells.
- Duplex ultrasound revealing the hypoechoic “halo” sign and stenosis of the temporal artery.
187
What is the initial treatment for GCA?
- Without visual symptoms or jaw claudication: 40-60mg prednisolone daily. - With visual symptoms or jaw claudication: 500-1000mg methylprednisolone daily. - Initiate treatment immediately, even before confirming the diagnosis, to reduce the risk of vision loss.
188
What additional medications are recommended in GCA management?
- Aspirin 75mg daily: Decreases vision loss and stroke risk.
- Proton pump inhibitor (e.g., omeprazole): Provides gastroprotection during steroid therapy.
- Bisphosphonates and calcium with vitamin D: For bone protection during prolonged steroid use.
189
What are the potential complications of GCA?
- Steroid-related complications (e.g., weight gain, diabetes, osteoporosis). - Vision loss. - Cerebrovascular accident (stroke).
190
What is Antiphospholipid Syndrome (APS)?
An autoimmune disorder where the immune system produces antibodies against phospholipids, increasing the risk of thrombosis and pregnancy complications.
191
What are the key complications of APS?
- Venous thromboembolism: Deep vein thrombosis and pulmonary embolism.
- Arterial thrombosis: Stroke, myocardial infarction, and renal thrombosis.
- Pregnancy-related complications: Recurrent miscarriage, stillbirth, and pre-eclampsia.
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What are the specific antiphospholipid antibodies associated with APS?
- Lupus anticoagulant
- Anticardiolipin antibodies
- Anti-beta-2 glycoprotein I antibodies
193
What is the first-line treatment for APS?
Long-term warfarin therapy with a target INR of 2-3 to prevent thrombosis.
194
What is the management approach for pregnant women with APS?
- Low molecular weight heparin (e.g., enoxaparin) and aspirin are used during pregnancy to reduce risks. - Warfarin is contraindicated in pregnancy.
195
What is Catastrophic Antiphospholipid Syndrome (CAPS)?
A rare complication characterized by rapid thrombosis in multiple organs within a few days, leading to multiorgan failure and high mortality.
196
What is the association between APS and Livedo Reticularis?
Livedo Reticularis is a purple, lace-like rash that gives a mottled appearance to the skin and is associated with APS.
197
What is the association between APS and Libman-Sacks Endocarditis?
Libman-Sacks Endocarditis is a non-bacterial endocarditis with growths (vegetations) on the heart valves, most often the mitral and aortic valves, and is associated with APS.
198
What is the association between APS and Thrombocytopenia?
Thrombocytopenia (low platelets) is common in APS.
199
What is Sjögren’s Syndrome?
An autoimmune condition affecting exocrine glands, notably the lacrimal and salivary glands, causing dry mouth, eyes, and vagina.
200
What are the common symptoms of Sjögren’s Syndrome?
- Dry mouth (xerostomia) - Dry eyes (keratoconjunctivitis sicca) - Dry vagina - Dry skin - Joint pain and stiffness - Fatigue
201
What is the difference between primary and secondary Sjögren’s Syndrome?
- Primary: Occurs in isolation. - Secondary: Occurs alongside other autoimmune diseases like systemic lupus erythematosus or rheumatoid arthritis.
202
Which antibodies are associated with Sjögren’s Syndrome?
- Anti-SS-A (anti-Ro) - Anti-SS-B (anti-La)
203
What is the Schirmer Test?
A diagnostic test measuring tear production by placing filter paper under the lower eyelid; less than 10mm of moisture after 5 minutes suggests Sjögren’s Syndrome.
204
What are the first-line treatments for Sjögren’s Syndrome?
- Artificial tears (e.g., polyvinyl alcohol eye drops during the day and carbomer gel at night) - Artificial saliva - Vaginal lubricants
205
What medication can stimulate tear and saliva production in Sjögren’s Syndrome?
Pilocarpine (oral)
206
What is the role of Hydroxychloroquine in managing Sjögren’s Syndrome?
May be considered, mainly in patients with associated joint pain.
207
What are potential complications of Sjögren’s Syndrome?
- Eye problems: keratoconjunctivitis sicca, corneal ulcers - Oral problems: dental cavities, candida infections - Vaginal problems: candida infection, sexual dysfunction - Rare complications: pneumonia, bronchiectasis, non-Hodgkin's lymphoma, peripheral neuropathy, vasculitis, renal impairment
208
What laboratory finding is associated with elevated CRP and ESR in vasculitis?
Elevated C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) are nonspecific markers of inflammation, commonly seen in vasculitis.
209
What is p-ANCA and its association with vasculitis?
p-ANCA (perinuclear anti-neutrophil cytoplasmic antibodies) are associated with small-vessel vasculitis, especially microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis.
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What is c-ANCA and its association with vasculitis?
c-ANCA (cytoplasmic anti-neutrophil cytoplasmic antibodies) are associated with granulomatosis with polyangiitis (GPA), a small-vessel vasculitis.
211
What laboratory finding is commonly elevated in eosinophilic granulomatosis with polyangiitis (EGPA)?
Elevated eosinophil count is commonly seen in eosinophilic granulomatosis with polyangiitis (EGPA).
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What is vasculitis?
Vasculitis refers to inflammation of the blood vessels, which can affect small, medium, or large vessels.
213
What are common symptoms of vasculitis?
- Joint and muscle pain - Peripheral neuropathy - Renal impairment - Purpura (non-blanching rash) - Necrotic skin ulcers - Gastrointestinal symptoms (e.g., diarrhea, abdominal pain, bleeding) - Systemic symptoms: fatigue, fever, night sweats, weight loss, anorexia, anemia
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What laboratory findings are associated with vasculitis?
- Elevated inflammatory markers (CRP and ESR) - p-ANCA (MPO antibodies) associated with microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis - c-ANCA (PR3 antibodies) associated with granulomatosis with polyangiitis
215
What is Henoch-Schönlein Purpura (HSP)?
An IgA-mediated small-vessel vasculitis presenting with a purpuric rash on the lower limbs and buttocks, often following an upper respiratory infection.
216
What is Microscopic Polyangiitis?
A small-vessel vasculitis characterized by p-ANCA positivity, affecting the lungs and kidneys, leading to glomerulonephritis and diffuse alveolar hemorrhage.
217
What is Granulomatosis with Polyangiitis (GPA)?
A small-vessel vasculitis associated with c-ANCA, presenting with nasal symptoms, respiratory issues, and glomerulonephritis.
218
What is Eosinophilic Granulomatosis with Polyangiitis (EGPA)?
A small-vessel vasculitis characterized by p-ANCA positivity, elevated eosinophil count, late-onset asthma, and sinusitis.
219
What is Polyarteritis Nodosa (PAN)?
A medium-vessel vasculitis presenting with renal impairment, hypertension, cardiovascular events, and tender skin nodules.
220
What is Kawasaki Disease?
A medium-vessel vasculitis affecting children under 5 years, characterized by high fever, widespread rash, bilateral conjunctivitis, strawberry tongue, and risk of coronary artery aneurysms.
221
What is Giant Cell Arteritis (GCA)?
A large-vessel vasculitis affecting older adults, presenting with unilateral headache, scalp tenderness, and risk of vision loss.
222
What is Takayasu’s Arteritis?
A large-vessel vasculitis affecting the aortic arch, leading to "pulseless" disease due to arterial occlusion.
223
What is Behçet’s Disease?
A complex inflammatory condition affecting blood vessels and tissues, characterized by recurrent oral and genital ulcers.
224
What are the main features of Behçet’s Disease?
- Recurrent oral ulcers - Recurrent genital ulcers - Uveitis - Skin lesions (e.g., erythema nodosum) - Vascular involvement (e.g., deep vein thrombosis) - Neurological manifestations (e.g., aseptic meningitis)
225
What is the pathergy test?
A diagnostic test where a sterile needle is used to make multiple pricks on the forearm; a positive result indicates non-specific skin hypersensitivity, which can suggest Behçet’s Disease.
226
What is the association between HLA-B51 and Behçet’s Disease?
There is a link between the HLA-B51 gene and an increased risk of developing Behçet’s Disease.
227
What are common differential diagnoses for mouth ulcers?
- Simple aphthous ulcers - Squamous cell carcinoma - Herpes simplex virus - Inflammatory bowel disease (e.g., Crohn’s disease) - Coeliac disease - Connective tissue diseases (e.g., rheumatoid arthritis) - Vitamin deficiencies (e.g., iron, B12, folate) - HIV
228
What is the first-line treatment for oral ulcers in Behçet’s Disease?
Topical steroids, such as soluble betamethasone tablets.
229
What systemic treatments are used for Behçet’s Disease?
- Systemic steroids (e.g., oral prednisolone) - Colchicine - Immunosuppressants (e.g., azathioprine) - Biologic therapy (e.g., infliximab)
230
What is the prognosis for individuals with Behçet’s Disease?
The disease often follows a relapsing-remitting course. Most patients have a normal life expectancy, but major complications can include vision loss, ruptured pulmonary aneurysms, bowel perforation, and neuro-Behçet’s disease.
231
What is Ehlers-Danlos Syndrome (EDS)?
A group of genetic conditions involving defects in collagen.
232
What is the result of collagen defects in Ehlers-Danlos Syndrome?
It leads to hypermobility in joints and abnormalities in connective tissues of the skin, bones, blood vessels, and organs.
233
What are the types of Ehlers-Danlos Syndrome?
- Hypermobile EDS: Most common, with joint hypermobility and soft skin.
234
What is Classical Ehlers-Danlos Syndrome?
- Classical EDS: Characterized by very stretchy skin, joint hypermobility, joint pain, and abnormal wound healing.
235
What is Vascular Ehlers-Danlos Syndrome?
- Vascular EDS: Most severe; fragile blood vessels, thin skin, and high risk of ruptures.
236
What is Kyphoscoliotic Ehlers-Danlos Syndrome?
- Kyphoscoliotic EDS: Features poor muscle tone, kyphoscoliosis, and significant joint hypermobility.
237
What are common symptoms of Ehlers-Danlos Syndrome?
- Joint dislocations, especially in shoulders or hips.
238
What other skin-related symptoms are common in Ehlers-Danlos Syndrome?
- Soft, stretchy skin.
239
What are common skin issues in Ehlers-Danlos Syndrome?
- Stretch marks (striae).
240
What vascular-related symptoms occur in Ehlers-Danlos Syndrome?
- Easy bruising and bleeding.
241
What are some musculoskeletal symptoms of Ehlers-Danlos Syndrome?
- Chronic pain, often widespread.
242
What neurological symptoms are seen in Ehlers-Danlos Syndrome?
- Headaches and chronic fatigue.
243
What gastrointestinal symptoms are common in Ehlers-Danlos Syndrome?
- Gastro-oesophageal reflux, abdominal pain, and irritable bowel syndrome.
244
What reproductive symptoms can be seen in Ehlers-Danlos Syndrome?
- Menorrhagia (heavy periods) and dysmenorrhea (painful periods).
245
What urinary symptoms are seen in Ehlers-Danlos Syndrome?
- Urinary incontinence and pelvic organ prolapse.
246
What musculoskeletal symptom related to the jaw is seen in Ehlers-Danlos Syndrome?
- Temporomandibular joint dysfunction.
247
What is the inheritance pattern of Hypermobile Ehlers-Danlos Syndrome?
- Hypermobile EDS: Autosomal dominant.
248
What is the inheritance pattern of Classical Ehlers-Danlos Syndrome?
- Classical EDS: Autosomal dominant.
249
What is the inheritance pattern of Vascular Ehlers-Danlos Syndrome?
- Vascular EDS: Autosomal dominant.
250
What is the inheritance pattern of Kyphoscoliotic Ehlers-Danlos Syndrome?
- Kyphoscoliotic EDS: Autosomal recessive.
251
What is a key differential diagnosis for hypermobility in Ehlers-Danlos Syndrome?
- Marfan syndrome: Features a high-arched palate, arachnodactyly (long fingers), and increased arm span to height ratio.
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What is Gout?
- A type of inflammatory arthritis caused by the deposition of uric acid crystals in the joints.
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What is the primary cause of Gout?
- High levels of uric acid in the blood (hyperuricemia), which leads to the formation of urate crystals.
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What are the common symptoms of Gout?
- Acute, severe pain in a single joint (often the big toe), redness, swelling, and warmth.
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What joints are most commonly affected by Gout?
- The big toe (metatarsophalangeal joint), followed by the ankle, knee, and wrists.
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What are the risk factors for developing Gout?
- Male gender, obesity, high purine diet (e.g., red meat, seafood), alcohol consumption, family history, and certain medications (e.g., diuretics).
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What is the pathophysiology of Gout?
- Uric acid crystallizes and deposits in the joints, triggering inflammation and acute pain.
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What is the first-line treatment for acute Gout?
- Nonsteroidal anti-inflammatory drugs (NSAIDs) like ibuprofen or naproxen.
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What is the role of Colchicine in Gout treatment?
- Used as a second-line treatment for acute attacks, particularly in patients who cannot tolerate NSAIDs.
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What is the role of steroids in Gout management?
- Oral or intra-articular steroids are used for acute flares when NSAIDs or colchicine are contraindicated.
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What medications are used for long-term management of Gout?
- Uric acid-lowering drugs such as Allopurinol or Febuxostat.
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When is long-term treatment for Gout started?
- After the acute attack has resolved, typically weeks later.
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What are Gout tophi?
- Deposits of urate crystals that form under the skin, typically on the fingers, elbows, and ears.
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How is Gout diagnosed?
- Joint aspiration to detect monosodium urate crystals under a microscope and elevated serum uric acid levels.
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What is the appearance of urate crystals in Gout?
- Needle-shaped and negatively birefringent under polarized light microscopy.
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What is the typical presentation of a Gout attack?
- Sudden, severe pain, redness, and swelling in a single joint, most commonly the big toe.
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What lifestyle changes can help manage Gout?
- Avoiding alcohol, staying hydrated, eating a low-purine diet, and losing weight.
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What foods should be avoided in Gout?
- Foods high in purines, such as red meat, organ meats, seafood, and alcohol (especially beer).
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What is the long-term goal of Gout management?
- To prevent future attacks by lowering serum uric acid levels below the saturation point of urate crystals.
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What are common complications of untreated Gout?
- Chronic joint damage, tophi formation, and kidney stones due to urate crystal deposits.
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What is the role of uric acid-lowering therapy in Gout prevention?
- To lower uric acid levels and prevent the formation of urate crystals and future Gout attacks.
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What is the target serum uric acid level in Gout management?
- Below 6 mg/dL to prevent crystal formation and recurrent attacks.
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What is the recommended fluid intake for Gout patients?
- At least 2-3 liters of water daily to help prevent urate crystal formation and promote kidney function.
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How does alcohol affect Gout?
- Alcohol, particularly beer and spirits, can increase uric acid levels and trigger acute attacks.
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What is the role of X-ray in diagnosing Gout?
- X-rays can show joint damage, tophi deposits, and characteristic "punched-out" erosions.
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What is the role of ultrasound in Gout diagnosis?
- Ultrasound can detect urate crystal deposits in the joints and is increasingly used for diagnosis.
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What is osteoporosis?
- A condition involving a significant reduction in bone density, making bones weaker and prone to fractures.
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What is osteopenia?
- A less severe decrease in bone density compared to osteoporosis.
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How is osteoporosis diagnosed?
- Diagnosis is made using a T-score from a DEXA scan, with a T-score of less than -2.5 indicating osteoporosis.
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What are the main risk factors for osteoporosis?
- Age, post-menopausal women, low BMI, low calcium and vitamin D intake, smoking, alcohol, and chronic diseases like rheumatoid arthritis.
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What is the T-score for osteoporosis on a DEXA scan?
- A T-score of less than -2.5 is diagnostic for osteoporosis.
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What role does hormone replacement therapy (HRT) play in osteoporosis?
- HRT is protective against osteoporosis, especially in post-menopausal women.
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What is the first-line treatment for osteoporosis?
- Bisphosphonates, such as alendronate, risedronate, or zoledronic acid.
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What is the recommended calcium intake for osteoporosis management?
- At least 1000mg of calcium daily.
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What is the recommended vitamin D intake for osteoporosis management?
- 400-800 IU of vitamin D daily.
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What is the role of bisphosphonates in osteoporosis treatment?
- They reduce bone resorption by inhibiting osteoclast activity, helping to strengthen bones.
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What are common side effects of bisphosphonates?
- Reflux, oesophageal erosions, atypical fractures, osteonecrosis of the jaw.
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What is the management for patients on long-term corticosteroids?
- Bisphosphonates are recommended for patients on long-term steroids, and calcium and vitamin D supplementation.
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How often should bisphosphonate treatment be reassessed?
- After 3-5 years, with a repeat DEXA scan to guide further treatment.
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What is the FRAX tool used for?
- It is used to assess the 10-year fracture risk and guide decisions on DEXA scanning and treatment.
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What are the key lifestyle modifications for managing osteoporosis?
- Increase physical activity, maintain a healthy weight, stop smoking, reduce alcohol consumption.
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What age groups should be assessed for osteoporosis?
- All women over 65, all men over 75, and anyone over 50 with risk factors or previous fractures.
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What is the role of a DEXA scan in osteoporosis diagnosis?
- DEXA scans measure bone mineral density and provide the T-score for diagnosis.
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What is chondrocalcinosis?
- Calcification in cartilage, often seen on X-ray in conditions like Pseudogout, but not specifically in osteoporosis.
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What is osteomalacia?
- A condition with defective bone mineralisation leading to soft bones. It results from insufficient vitamin D.
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What is the primary cause of osteomalacia?
- Vitamin D deficiency.
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What is the role of vitamin D in bone health?
- Vitamin D regulates calcium and phosphate absorption, promoting bone mineralisation and turnover.
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What are the common symptoms of osteomalacia?
- Fatigue, bone pain, muscle weakness, muscle aches, and pathological fractures.
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What are Looser zones?
- Fragility fractures that partially go through the bone, typically seen in osteomalacia.
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What are some risk factors for vitamin D deficiency leading to osteomalacia?
- Darker skin, low sun exposure, living in colder climates, malabsorption disorders, and chronic kidney disease.
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How does osteomalacia differ from osteoporosis?
- Osteomalacia involves defective bone mineralisation, while osteoporosis is characterised by bone density loss.
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How is osteomalacia diagnosed?
- Serum 25-hydroxyvitamin D levels, low calcium and phosphate, high alkaline phosphatase, and high parathyroid hormone (PTH).
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What imaging findings are associated with osteomalacia?
- X-rays may show osteopenia; a DEXA scan shows low bone mineral density.
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What are the key laboratory findings in osteomalacia?
- Low serum calcium, low phosphate, high alkaline phosphatase, and high PTH (secondary hyperparathyroidism).
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What is the typical treatment for osteomalacia?
- Vitamin D supplementation, typically colecalciferol (vitamin D₃), with a loading dose followed by a maintenance dose.
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What is the initial treatment regime for vitamin D deficiency in osteomalacia?
- 50,000 IU once weekly for 6 weeks or 4000 IU daily for 10 weeks.
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What is the maintenance dose of vitamin D for osteomalacia?
- 800-2000 IU per day following the loading regime.
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Why is calcium monitored during osteomalacia treatment?
- Calcium levels may be low due to deficiency or high in cases of hyperparathyroidism or conditions like sarcoidosis.
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What is the role of parathyroid hormone (PTH) in osteomalacia?
- Low calcium levels due to vitamin D deficiency cause increased PTH secretion, leading to bone resorption.
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What are common clinical features of osteomalacia?
- Bone pain, muscle weakness, fatigue, and possible fractures due to weak bones.
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How should serum calcium be monitored in osteomalacia treatment?
- It should be checked within a month of starting treatment to ensure proper balance.
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IPF in how many dermatomyositis and myositis?
30%
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What is Felty's syndrome?
rare complication of rheumatoid, characterised by triad of RA splenomegaly and neurtropenia
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Methotrexate monitoring?
LFT, U+E, FBC every 3 months
315
Polymyositis vs polymyalgia rheumatica?
Polymyositis - proximal muscle weakness, raised CK and no rash
PR - not true weakness one xamination
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What eye problems does temporal arteritis lead to?
Anterior ischemic optic neuropathy - presents as swollen pale disc with blurred margins
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What abx do you not give pts on methotrexate?
trimethoprim
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What do we need to check before prescribing Azathioprine?
Pre-treatment checks:
TPMT activity → Low/absent activity increases risk of myelosuppression.
FBC, LFTs, U&Es → Baseline assessment.
Hepatitis B, C & TB screening → Risk of reactivation.
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Pt with RA on methotrexate presents and you don't know if it is with pulmonary fibrosis or pneumonitis - how do you differentiate?
Methotrexate-Induced Pneumonitis 🏥
- Onset: Acute (weeks to months)
- Symptoms: Dry cough, fever, dyspnoea, fatigue
- Exam Findings: Bilateral crackles, hypoxia (low O2 sats)
- CXR/CT Findings: Diffuse interstitial infiltrates, alveolar shadowing
- Risk Factors: RA, smoking, methotrexate use
- Management: STOP methotrexate immediately, consider corticosteroids
Pulmonary Fibrosis (RA-ILD) 🫁
- Onset: Chronic (years)
- Symptoms: Dry cough, gradual worsening dyspnoea
- Exam Findings: Bilateral crackles, clubbing (late stage)
- CXR/CT Findings: Reticular opacities, honeycombing (late)
- Risk Factors: RA, male sex, smoking
- Management: Immunosuppression (steroids, DMARDs), antifibrotics
Key Learning Point: Methotrexate pneumonitis is acute and potentially reversible, whereas RA-associated pulmonary fibrosis is chronic and progressive.
