Orthopaedics & Rheumatology Flashcards

1
Q

Osteoarthritis features

Occurs in the synovial joints
Commonly affected Joints
Hips
Knees
Distal interphalangeal (DIP) joints in the hands
Carpometacarpal (CMC) joint at the base of the thumb
Lumbar spine
Cervical spine (cervical spondylosis)

A
  • Imbalance between cartilage damage and the chondrocyte response, leading to structural issues in the joint

Presentation

  • Joint paint and Stiffness at the end of the day and after activity
  • Reduced function of joint, Restricted range of motion
  • Crepitus on movement
  • Effusions (fluid) around the joint

Signs in Hands

  • Heberden’s nodes (in the DIP joints)
  • Bouchard’s nodes (in the PIP joints)
  • Squaring at the base of the thumb (CMC joint)
  • Weak grip

Signs in Hip
* Chronic history of groin ache following exercise and relieved by rest
* Red Flag: rest pain, night pain and morning stiffness > 2 hours

Causes: genetic factors, overuse and injury

RF
Obesity
Age
Trauma
Female
Occupation

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2
Q

Osteoarthritis on Xray

LOSS

A
  • Loss of joint space
  • Osteophytes forming at joint margins
  • Subchondral sclerosis
  • Subchondral cysts
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3
Q

Osteoarthritis Management

A
  • Weight loss
  • Aerobic + Muscle Strength Excercises
  • Topical NSAID
  • Oral NSAID (+PPI)
  • Do not offer paracetamol or weak opioids
  • intra-articular steroid injections - Short lived.
  • Joint replacement

Patients who have had a hip replacement operation should receive basic advice to minimise the risk of dislocation:
* avoiding flexing the hip > 90 degrees
* avoid low chairs
* do not cross your legs
* sleep on your back for the first 6 weeks

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4
Q

HLA DR4

Rheumatoid Arthritis features

Autoimmune inflammatory arthritis

RF
Smoking
obesity
Female

A
  • Chronic inflammation in the synovial lining of the joints, tendon sheaths and bursa - Stiff, swollen + painful joints
  • Worst in morning but relieve with activity
  • Often Symmetrical

Commonly affected joints

  • Metacarpophalangeal (MCP) joints
  • Proximal interphalangeal (PIP) joints
  • Wrist
  • Metatarsophalangeal (MTP) joints (in the foot)
  • palpation: tenderness and synovial thickening: “boggy” feeling.
  • +ve ‘squeeze test’ - discomfort on squeezing across the metacarpal or metatarsal joints

Hand Signs

  • Z-shaped deformity to the thumb
  • Swan neck deformity (hyperextended PIP and flexed DIP)
  • Boutonniere deformity (hyperextended DIP and flexed PIP)
  • Ulnar deviation of the fingers at the MCP joints

Swan neck and boutonniere deformities are late features of rheumatoid arthritis and unlikely to be present in a recently diagnosed patient.

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5
Q

Rheumatoid Arthritis Ix

A
  • 1st line: Rheumatoid factor (RF)
  • 2nd line: if RF -ve: AntiCCP - much higher specificity than RF
  • Inflammatory markers, such as C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR)

Results

  • RF is positive in 70-80% of patients with rheumatoid arthritis, high titre levels are associated with severe progressive disease (but NOT a marker of disease activity.)
  • Anti-cyclic citrullinated peptide antibody may be detectable up to 10 years before the development of rheumatoid arthritis
  • llowing early detection of patients suitable for aggressive anti-TNF therapy

Rheumatoid F : Rose-Waaler test: sheep red cell agglutination

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6
Q

Rheumatoid Arthritis Xray

A
  • Periarticular osteopenia
  • Boney erosions
  • Soft tissue swelling
  • periarticular erosions
  • subluxation (a type of dislocation)
  • Joint destruction and deformity (in more advanced disease)
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7
Q

Rheumatoid Arthritis Management

A
  • Initial: DMARD + Bridging Prednislone (short course)
  • Flare up: Corticosteroid (oral or IM)
  • TNF inhibitors: considered if 2 DMARDs didn’t work.

DMARD Choices

  • Methotrexate: Once weekly - can cause pneumonitis
  • Sulfasalazine (Orange urine and male infertility (reduces sperm count))
  • Leflunomide (Hypertension and peripheral neuropathy)
  • Hydroxychloroquine: should only be considered for initial therapy if mild or palindromic disease

TNF Inhibitors

  • Etanercept (can cause TB reactivation)
  • Infliximab (can cause TB reactivation)
  • adalimumab: monoclonal antibody, subcutaneous administration

Monitoring:

  • CRP + DAS28 disease monitoring
  • Methotrexate users: Monitoring of FBC & LFTs is essential due to the risk of myelosuppression and liver cirrhosis.
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8
Q

What is palindromic rheumatism?

A
  • self-limiting episodes of inflammatory arthritis
  • pain, stiffness and swelling typically affecting only a few joints.
  • symptoms last days, then completely resolve
  • Rheumatoid factor or anti-CCP antibodies may indicate that it will progress to rheumatoid arthritis.
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9
Q

Rheuamtoid Arthritis Complications

A
  • Respiratory: pulmonary fibrosis, pleural effusion, pulmonary nodules, bronchiolitis obliterans, methotrexate pneumonitis, pleurisy
  • Ocular: keratoconjunctivitis sicca (most common), episcleritis, scleritis, corneal ulceration, keratitis, steroid-induced cataracts, chloroquine retinopathy
  • Osteoporosis
  • ischaemic heart disease: RA carries a similar risk to type 2 diabetes mellitus
  • increased risk of infections
  • Depression
  • Felty’s syndrome (RA + splenomegaly + low white cell count)
  • Amyloidosis

Poor Prognostic features

  • rheumatoid factor positive
  • anti-CCP antibodies
  • poor functional status at presentation
  • X-ray: early erosions (e.g. after < 2 years)
  • extra articular features e.g. nodules
  • HLA DR4
  • insidious onset
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10
Q

Where does Atlantoaxial Subluxation occur

A
  • Atlantoaxial subluxation occurs in the cervical spine.
  • Synovitis and damage to the ligaments around the odontoid peg of the axis (C2) allow it to shift within the atlas (C1).
  • Subluxation can cause spinal cord compression and is an emergency.
  • This needs to be considered when a patient is having a general anaesthetic and requires intubation.
  • MRI can be used to visualise changes in these areas as part of a pre-operative assessment.
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11
Q

Psoriatic Arthritis Features

inflammatory arthritis associated with psoriasis

A
  • Psoriatic arthritis occurs in 10-20% of patients with psoriasis (arthritis can appear befor skin changes)
  • seronegative spondyloarthropathy

5 Patterns
1. Symmetrical: very similar to rheumatoid arthritis - most common
2. Asymmetrical oligoarthritis: typically affects hands and feet
3. Sacroiliitis
4. DIP joint disease (10%)
5. Arthritis mutilans (severe deformity fingers/hand, ‘telescoping fingers’)

Other Signs

  • Psoriatic skin lesions
  • Enthesitis: inflammation at the site of tendon and ligament insertion e.g. Achilles tendonitis, plantar fascitis
  • Tenosynovitis: typically of the flexor tendons of the hands
  • Dactylitis: diffuse swelling of a finger or toe
  • Nail pitting + onycholysis
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12
Q

Psoriatic Arthritis Investigations & Management

Psoriasis Epidemiological Screening Tool (PEST) as a way of screening for psoriatic arthritis in patients with psoriasis. It involves questions about joint pain, swelling, a history of arthritis and nail pitting. A high score triggers a referral to a rheumatologist.

A

Xray

  • ‘pencil-in-cup’ appearance - periarticular erosions and bone resorption.
  • periostitis
  • New born formation

Management

  • mild peripheral arthritis/mild axial disease: NSAID
  • Severe: DMARD
  • Apremilast: phosphodiesterase type-4 (PDE4) inhibitor → suppression of pro-inflammatory mediator synthesis and promotion of anti-inflammatory mediators

Better prognosis than Rheumatoid Arthritis

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13
Q

Reactive Arthritis

Reiter’s Syndrome

A
  • HLA-B27 associated seronegative spondyloarthropathies
  • Triad of urethritis, conjunctivitis and asymmetrical arthritis post STI (Chlamydia trachomatis)
  • Develops within 4wk can last 4-6m or maybe chronic (rare)

Also seen

  • dactylitis
  • circinate balanitis (painless vesicles on the coronal margin of the prepuce)
  • keratoderma blenorrhagica (waxy yellow/brown papules on palms and soles)#

Management

  • symptomatic: analgesia, NSAIDS, intra-articular steroids
  • sulfasalazine and methotrexate are sometimes used for persistent disease

sexually acquired reactive arthritis, SARA

non STI form (Post-dysenteric form)

  • Shigella flexneri
  • Salmonella typhimurium
  • Salmonella enteritidis
  • Yersinia enterocolitica
  • Campylobacter
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14
Q

Septic Arthritis

how does it differ from reactive arthritis?

Feaures, Causative Organisms, Ix & Management

A

Features

  • Acute, swollen joint (mostly knee)
  • Restricted movement in 80% of patients
  • Examination findings: warm to touch/fluctuant
  • Fever: present in the majority of patients

Causative Organsisms

  • Staphylococcus Aureus
  • Neiseria Gonorrohoeae - In sexually active young adult.
  • Commonly spread by Haematogenously from distant bacterial infections e.g. abscesses
  • Can also occur in prosthetic joint replacement.

Ix

  • Synovial fluid sampling is obligatory - radiographic guidance needed
  • Blood cultures

Management

  • flucloxacillin or clindamycin if penicillin allergic
  • 2 week IV then 2-4wk Oral
  • Needle Aspiration for joint decompression.

In Reactive Arthritis you are unable to aspirate joint

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15
Q

Common Joint, Diagnosis, Management & Prophylaxis

Gout

form of crystal arthropathy

Risk Factors

  • Male
  • Family history
  • Obesity
  • High purine diet (e.g., meat and seafood)
  • Alcohol
  • Diuretics
  • Cardiovascular disease
  • Kidney disease
A
  • Chronically high blood uric acid levels.
  • Urate crystals are deposited in the joint, causing it to become inflamed.
  • Present with single acute hot, swollen and painful joint

Commonly Affected Joint

  • The base of the big toe – the metatarsophalangeal joint (MTP joint)
  • The base of the thumb – the carpometacarpal joint (CMC joint)
  • Can affect larger joints such as knee

Diagnosis

  • Clinical + Raised Blood Urate (a uric acid level ≥ 360 umol/L)
  • Joint Aspiration: Needle-shaped monosodium urate crystals, negatively birefringent of polarised light.
  • XRays: Maintained joint space, lytic lesions in the bone, Punched out erosions, Erosions can have sclerotic borders with overhanding edges, joint effusion

Management

  • Lose weight and avoid alcohol especially during acute episode
  • 1st line: NSAID + PPI
  • 2nd line: Colcichine given if NSAID contraindicated due to Renal or Gastric Issues.

Prophylaxis
Recommended if >2 attacks in 12m
* Allopurinol or Febuxostat - lower serum uric acid
* Change NSAID to Colcichine if commencing allopurinol

IMPORTANT to rule out Septic Arthritis First

  • oral steroids may be considered if NSAIDs and colchicine are contraindicated.
  • a dose of prednisolone 15mg/day is usually used
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16
Q

Pseudo Gout

form of crystal arthropathy

RF

  • > 65y
  • haemochromatosis
  • hyperparathyroidism
  • low magnesium, low phosphate
  • acromegaly, Wilson’s disease
A
  • Arthopathy caused by deposition of calcium pyrophosphate dihydrate crystals in the synovium
  • Knee, wrist and shoulders most commonly affected
  • joint aspiration: weakly-positively birefringent rhomboid-shaped crystals
  • X-ray: chondrocalcinosis + LOSS (osteoarthritis)

Management

  • aspiration of joint fluid, to exclude septic arthritis
  • NSAIDs or intra-articular, intra-muscular or oral steroids as for gout

IMPORTANT to rule out Septic Arthritis First

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17
Q

Osteomyelitis

Inflammation in a bone and bone marrow, usually by bacterial infection.

subclassified into:

  1. Haematogenous osteomyelitis: RF: sickle cell anaemia, IVDU, immunosuppression d, infective endocarditis
  2. Non-haematogenous osteomyelitis: RF: diabetic foot ulcers/pressure sores, diabetes mellitus, peripheral arterial disease
A

Presentation

  • Fever
  • Pain and tenderness
  • Erythema
  • Swelling

Haematogenous Osteomyelitis

  • Baceteremia, monomicrobial
  • most common in children
  • vertebral osteomyelitis most common form of haematogenous osteomyelitis in adults

Non-Haematogenous Osteomyelitis

  • contiguous spread of infection from adjacent soft tissues to the bone from site of trauma (usually)
  • Polymicrobial, common in adults

Microbiology
Staph. aureus is the most common cause except in patients with sickle-cell anaemia where Salmonella species predominate

Investigations
MRI is the imaging modality of choice, with a sensitivity of 90-100%
Blood tests will show raised inflammatory markers (e.g., WBC, CRP and ESR).

Management
flucloxacillin for 6 weeks
clindamycin if penicillin-allergic
rifampicin or fusidic acid added for the first 2 weeks in acute
Chronic: Abx for 3m

Differs from septic arthritis as this within the bone/bone marrow and arthritis is in the joint

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18
Q

Ankylosing spondylitis features

HLA-B27 associated spondyloarthropathy

RF

  • Male (3:1)
  • 20s
A
  • Inflammatory condition affecting the axial skeleton (mainly the spine and sacroiliac joints), causing progressive stiffness and pain
  • Insidious onset
  • Stiffness is usually worse in the morning and improves with exercise
  • Can awaken patient from sleep

Examination

  • Reduced lateral flexion
  • Reduced forward flexion - Schober’s test +ve
  • Reduced Chest Expansion

Other features - the ‘A’s
* Apical fibrosis
* Anterior uveitis
* Aortic regurgitation
* Achilles tendonitis
* AV node block
* Amyloidosis
* and cauda equina syndrome
* peripheral arthritis (25%, more common if female)
*

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19
Q

Ankylosing spondylitis Investigation & Management

HLA-B27 is of little use in making the diagnosis as it is positive in:
* 90% of patients with ankylosing spondylitis
* 10% of normal patients

A

XRay

  • sacroiliitis: subchondral erosions, sclerosis
  • squaring of lumbar vertebrae
  • ‘bamboo spine’ (late & uncommon)
  • syndesmophytes: due to ossification of outer fibers of annulus fibrosus
  • chest x-ray: apical fibrosis

Management

  • encourage regular exercise such as swimming
  • NSAIDs are the first-line treatment
  • physiotherapy
  • Anti-TNF medications are second-line (e.g., adalimumab, etanercept or infliximab)
  • Secukinumab or ixekizumab are third-line (monoclonal antibodies against interleukin-17)
  • Upadacitinib is another third-line option (JAK inhibitor)

If the x-ray is negative for sacroiliac joint involvement in ankylosing spondylitis but suspicion for AS remains high, the next step in the evaluation should be obtaining an MRI

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20
Q

Osteoporosis Features

Risk Factors

  • Age - POST MENOPausal
  • Female
  • corticosteroid use
  • smoking
  • alcohol
  • low body mass index
  • family history
  • RA
  • sedentary lifestyle
  • premature menopause
  • Caucasians and Asians
  • endocrine disorders: hyperthyroidism, hypogonadism (e.g. Turner’s, testosterone deficiency), growth hormone deficiency, hyperparathyroidism, diabetes mellitus
  • multiple myeloma, lymphoma
  • gastrointestinal disorders: inflammatory bowel disease, malabsorption (e.g. coeliac’s), gastrectomy, liver disease
  • chronic kidney disease
  • osteogenesis imperfecta, homocystinuria
A
  • Presence of bone mineral density (BMD) of less than 2.5 standard deviations (SD) below the young adult mean density.
  • Bone mineral density (BMD) is measured using a DEXA scan (Z value and T Value, T is used in this context)
  • If BMD -1 to -2.5 = Osteopenia
  • Pathophysiology: Increased bone breakdown by osteoclasts + decreased bone formation by osteoblasts → loss of bone mass.

Medications that may worsen osteoporosis (other than glucocorticoids):

  • SSRIs
  • antiepileptics
  • proton pump inhibitors
  • glitazones
  • long term heparin therapy
  • aromatase inhibitors e.g. anastrozole
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21
Q

Osteoporosis Investigations

Who should be assessed?
* Anyone on long-term oral corticosteroids or with a previous fragility fracture
* Anyone 50 and over with risk factors
* All women 65 and over
* All men 75 and over

A

QFracture tool (preferred by NICE)

  • developed in 2009 based on UK primary care dataset
  • can be used for patients aged 30-99 years (this is stated on the QFracture website, but other sources give a figure of 30-85 years)
  • includes a larger group of risk factors e.g. cardiovascular disease, history of falls, chronic liver disease, rheumatoid arthritis, type 2 diabetes and tricyclic antidepressants
  • patients above 10% are considered for a DEXA

FRAX

  • See card below

Straight DEXA

  • before starting treatments that may have a rapid adverse effect on bone density (for example, sex hormone deprivation for treatment for breast or prostate cancer).
  • in people aged under 40 years who have a major risk factor, such as history of multiple fragility fracture, major osteoporotic fracture, or current or recent use of high-dose oral or high-dose systemic glucocorticoids (more than 7.5 mg prednisolone or equivalent per day for 3 months or longer).

Straight Treatment

  • A DEXA may be arranged without calculating the risk in patients over 50 with a fragility fracture
  • Treatment may be started without a DEXA in patients with a vertebral fracture
22
Q

What is FRAX?

FRAX Questions

Questionnaire:
1.Age (between 40 and 90 years)
Age:
2.Sex
3.Weight (kg)
4.Height (cm)
5.Previous Fracture
6.Parent Fractured Hip
7.Current Smoking
8.Glucocorticoids
9.Rheumatoid arthritis
10.Secondary osteoporosis
11.Alcohol 3 or more units/day
12.Femoral neck BMD (g/cm2)
Select BMD

A
  • estimates the 10-year risk of fragility fracture
  • valid for patients aged 40-90 years
  • based on international data so use not limited to UK patients
  • assesses the following factors: age, sex, weight, height, previous fracture, parental fracture, current smoking, glucocorticoids, rheumatoid arthritis, secondary osteoporosis, alcohol intake
  • bone mineral density (BMD) is optional, but clearly improves the accuracy of the results. NICE recommend arranging a DEXA scan if FRAX (without BMD) shows an intermediate result

If the FRAX assessment was done witha bone mineral density (BMD) measurement the results (10-year risk of a fragility fracture) will be given and categorised automatically into one of the following:

  • reassure
  • consider treatment
  • strongly recommend treatment
23
Q

Osteoporosis Management

A

1st line

  • Alendronate - once weekly to be taken empty stomach, sit up right 30 mins or
  • Zoledronic acid 5 mg once yearly (intravenous)
    + Daily Calcium (at least 1000mg) + Vitamin D (400-800 IU)

If Alendronate not tolerated then: risedronate or etidronate

2nd line

  • Raloxifene - selective oestrogen receptor modulator (SERM)

Monitoring

  • Reasses treatment with bisphosphonates after 3-5 years. They suggest a repeat DEXA scan and stopping treatment if the T-score is more than -2.5. Treatment is continued in high-risk patients.

Alendronate: Upper GI issues, Osteonecrosis of the jaw & auditory canal

24
Q

Management of patients at risk of corticosteroid-induced osteoporosis

risk of osteoporosis is thought to rise significantly once a patient is taking the equivalent of prednisolone 7.5mg a day for 3 or more months

A
  • If steroid commenced then start bone protection straight away

Prior to Commencing Treatment

  1. Patients over the age of 65 years or those who’ve previously had a fragility fracture should be offered bone protection.
  2. Patients under the age of 65 years should be offered a bone density scan, with further management dependent:

T = 0: Reassure
T =Between 0 and -1.5: Repeat BMD in 1-3Y
T = Less than -1.5: Offer bone protection

Osteoporosis T <-2.5 Osteopenia T -1.5 to -2.5

25
Q

Verterbral Fractures in Osteoporosis

A
  • Advancing age is a major risk factor osteoporotic fractures: Women ≥ 65 years old and men ≥ 75 years old should be considered for fracture risk assessment.

Symptoms

  • Asymptomatic: an osteoporotic vertebral fracture may be diagnosed through an incidental finding on X-ray
  • Acute back pain
  • Breathing difficulties: changes in the shape and length of vertebrae lead to the compression of organs such as the lungs, heart and intestine
  • Gastrointestinal problems: due to compression of abdominal organs
  • Only a minority of patients will have a history of fall/trauma
  • Kyphosis

Xray

  • X-ray of the spine: This should be the first investigation ordered and may show wedging of the vertebra due to compression of the bone. An X-ray of the spine may also show old fractures (which can have a sclerotic appearance)
26
Q

Systemic Lupus Erythematosus Theory

inflammatory autoimmune connective tissue disorder.

A
  • autoimmune disease: SLE a type 3 hypersensitivity reaction
  • associated with HLA B8, DR2, DR3
  • females (F:M = 9:1)
  • more common in Afro-Caribbeans and Asian communities
  • onset is usually 20-40 years

SLE is a chronic multisystem disorder that most commonly affects women during their reproductive years.

27
Q

Systemic lupus erythematosus: features

A
  • fatigue
  • fever
  • mouth ulcers
  • lymphadenopathy
  • Purple Butterfly macular rash over face - spares nasolabial folds
  • proteinuria
  • glomerulonephritis (diffuse proliferative glomerulonephritis is the most common type)
28
Q

Systemic lupus erythematosus Investigations & Management

A

Antibodies

  • 99% are ANA positive
    this high sensitivity makes it a useful rule out test, but it has low specificity
  • 20% are rheumatoid factor positive
  • anti-dsDNA: highly specific (> 99%), but less sensitive (70%)
  • anti-Smith: highly specific (> 99%), sensitivity (30%)
  • also: anti-U1 RNP, SS-A (anti-Ro) and SS-B (anti-La)

Monitoring
* ESR is generally used
* CRP may be normal - a raised CRP may indicate underlying infection

Management
* NSAIDs
* sun-block - especially for macular rash
* Hydroxychloroquine-the treatment of choice for SLE
* DMARD and biological therapies for more advanced disease presentation

If internal organ involvement e.g. renal, neuro, eye then consider prednisolone, cyclophosphamide

29
Q

Systemic lupus erythematosus: complications

A

Cardiovascular disease is a leading cause of death. Chronic inflammation in blood vessels leads to hypertension and coronary artery disease.

Infection is more common, both from the disease process and secondary to immunosuppressant drugs.

Anaemia in SLE can be due to anaemia of chronic disease, autoimmune haemolytic anaemia, bone marrow suppression by medications or kidney disease. It can also cause leucopenia (low white cells), neutropenia (low neutrophils) and thrombocytopenia (low platelets).

Pericarditis (inflammation of the pericardial sac surrounding the heart) causes sharp chest pain that gets worse on lying flat.

Pleuritis (inflammation of the lining of the lungs) causes sharp chest pain on inspiration.

Interstitial lung disease can be caused by inflammation in the lung tissue, leading to pulmonary fibrosis.

Lupus nephritis (inflammation in the kidney) can progress to end-stage renal failure. Investigations include a urine protein:creatinine ratio and renal biopsy. The renal biopsy is often repeated to assess the response to treatment.

Neuropsychiatric SLE is caused by inflammation in the central nervous system. It can present with optic neuritis (inflammation of the optic nerve), transverse myelitis (inflammation of the spinal cord) or psychosis.

Recurrent miscarriage is more common in SLE. There is also an increased risk of intrauterine growth restriction, pre-eclampsia and pre-term labour.

Venous thromboembolism may be associated with antiphospholipid syndrome occurring secondary to SLE.

Management Lupus Nephritis
treat hypertension
initial therapy for focal (class III) or diffuse (class IV) lupus nephritis
glucocorticoids with either mycophenolate or cyclophosphamide
subsequent therapy
mycophenolate is generally preferred to azathioprine to decrease the risk of developing end-stage renal disease

30
Q

Osteomalacia

softening of the bones secondary to low vitamin D levels that in turn lead to decreased bone mineral content. If this occurs in growing children it is referred to as rickets, with the term osteomalacia preferred for adults.

A

Causes

  • vitamin D deficiency: malabsorption, lack of sunlight, diet
  • chronic kidney disease
  • drug induced e.g. anticonvulsant

Presentation

  • bone pain
  • bone/muscle tenderness
  • fractures: especially femoral neck
  • proximal myopathy: may lead to a waddling gait

Ix

  • low vitamin D levels
  • low calcium, phosphate (in around 30%)
  • raised alkaline phosphatase (in 95-100% of patients)

Management

  • vitamin D supplmentation
  • a loading dose is often needed initially
    calcium supplementation if dietary calcium is inadequate
31
Q

Anti Phospholipid Syndrome

often occurs 2ndry to SLE

an acquired disorder characterised by a predisposition to both venous and arterial thromboses, recurrent fetal loss and thrombocytopenia.

A
  • Paradoxical rise in the APTT
  • venous/arterial thrombosis
  • recurrent miscarriages
  • livedo reticularis
  • other features: pre-eclampsia, pulmonary hypertension

Ix

antibodies

  • anticardiolipin antibodies
  • anti-beta2 glycoprotein I (anti-beta2GPI) antibodies
  • lupus anticoagulant

Blood

  • thrombocytopenia
  • prolonged APTT

Management

  • primary thromboprophylaxis: low-dose aspirin
  • secondary thromboprophylaxis: initial VTE or Arterial thrombosis: lifelong warfarin with a target INR of 2-3
  • Consider addition of aspirin if VTE occurs when taking warfarin with new target INR of 3-4

Management in Pregnancy

  • low-dose aspirin should be commenced once the pregnancy is confirmed on urine testing
  • low molecular weight heparin once a fetal heart is seen on ultrasound.

One might expect the APTT to be shortened (indicating faster clotting) due to the increased presence of clotting factors. However, in some cases of APS, there can be a paradoxical prolongation of the APTT due to the presence of lupus anticoagulant antibodies, which interfere with the clotting process in the laboratory setting, leading to a false impression of impaired clotting ability.

32
Q

Sjogren’s syndrome

common in F and increase risk of lymphoid malignancy

autoimmune disorder affecting exocrine glands resulting in dry mucosal surfaces. It may be primary (PSS) or secondary to rheumatoid arthritis or other connective tissue disorders,

A

Features

  • dry eyes: keratoconjunctivitis sicca
  • dry mouth
  • vaginal dryness
  • arthralgia
  • Raynaud’s, myalgia
  • sensory polyneuropathy
  • recurrent episodes of parotitis
  • renal tubular acidosis (usually subclinical)

Investigation

  • rheumatoid factor (RF) positive in nearly 50% of patients
  • ANA positive in 70%
  • anti-Ro (SSA) antibodies in 70% of patients with PSS
  • anti-La (SSB) antibodies in 30% of patients with PSS
  • Schirmer’s test: filter paper near conjunctival sac to measure tear formation
  • histology: focal lymphocytic infiltration
  • also: hypergammaglobulinaemia, low C4

Management

  • artificial saliva and tears
  • pilocarpine may stimulate saliva production

Remember anti RoLayyy antibodies

33
Q

Features, Investigations, Management

Polymyositis and Dermatomyositis

typically affects middle-aged, female:male 3:1

Polymyositis and dermatomyositis are autoimmune disorders causing muscle inflammation (myositis). Both present with proximal muscle weakness.

A

Features

  • Symmetrical, proximal muscle weakness (causing difficulties standing from a chair, climbing stairs or lifting overhead)
  • T-cell mediated cytotoxic process directed against muscle fibres - also associated with malignancy
  • Dermatomyositis also involves characteristic skin changes, specifically Gottron papules affecting the backs of the hands and **heliotrope rash **affecting the eyelids.
  • Raynaud’s
  • respiratory muscle weakness
  • interstitial lung disease

Ix

  • Elevated Creatinine Kinase (Important)
  • Elevated LDH
  • EMG
  • muscle biopsy
  • anti-synthetase antibodies: antiJo-1 antibodies are seen in pattern of disease associated with lung involvement, Raynaud’s and fever

Management

  • high-dose corticosteroids tapered as symptoms improve
  • azathioprine may be used as a steroid-sparing agent

anti-Jo-1 antibodies associated with polymyositis.

34
Q

Raynaud’s Phenomenon

Primary or secondary

exaggerated vasoconstrictive response of the digital arteries and cutaneous arteriole to the cold or emotional stress.

A
  • Young women (e.g. 30 years old) with bilateral symptoms.
  • 2ndry causes: Connective tissue disorder, vibrating tools, scleroderma, Leukemia, OCP.

Management

  • suspected secondary Raynaud’s refer to secondary care
  • first-line: calcium channel blockers e.g. nifedipine
  • IV prostacyclin (epoprostenol) infusions: effects may last several weeks/months
35
Q

link to temporal arteritis

Polymyalgia rheumatica

muscle stiffness and raised inflammatory markers in elderly

A

Features

  • typically patient > 60 years old
  • usually rapid onset (e.g. < 1 month)
  • aching, morning stiffness in proximal limb muscles - improves w/activity
  • weakness is not considered a symptom of polymyalgia rheumatica
  • also mild polyarthralgia, lethargy, depression, low-grade fever, anorexia, night sweats

Investigations

  • raised inflammatory markers e.g. ESR > 40 mm/hr
  • creatine kinase and EMG normal

Treatment

  • prednisolone e.g. 15mg/od
  • patients typically respond dramatically to steroids, failure to do so should prompt consideration of an alternative diagnosis
  • Follow up weekly
  • treatment with steroids typically lasts 1-2 years. must titrate down.

There is a strong association with giant cell arteritis, and the two conditions often occur together.

Common Pain Areas

  • Shoulders, potentially radiating to the upper arm and elbow
  • Pelvic girdle (around the hips), potentially radiating to the thighs
  • Neck
36
Q

Polyarteritis nodosa

A
  • Vasculitis affecting medium-sized arteries with necrotizing inflammation leading to aneurysm formation
  • Middle aged men
  • Hep B association
  • fever, malaise, arthralgia
  • weight loss
  • hypertension
  • mononeuritis multiplex, sensorimotor polyneuropathy
  • testicular pain
  • livedo reticularis

Management: Steroids?

37
Q

Paget’s Disease

increased but uncontrolled bone turnover

disorder of osteoclasts, with excessive osteoclastic resorption followed by increased osteoblastic activity

A

Most affected bones: Skull, spine/pelvis, and long bones of the lower extremities
Features: older male with bone pain and an isolated raised ALP
Raised procollagen type I N-terminal propeptide (PINP) and CTx

X ray

  • osteolysis in early disease → mixed lytic/sclerotic lesions later
  • skull x-ray: thickened vault, osteoporosis circumscripta

Management

  • IV or oral Bisphosphonates

Complications

  • deafness (cranial nerve entrapment)
  • bone sarcoma (1% if affected for > 10 years)
  • fractures
  • skull thickening
  • high-output cardiac failure
38
Q

Fibromyalgia

A
  • American College of Rheumatology
    classification criteria which lists 9 pairs of tender points on the body. If a patient is tender in at least 11 of these 18 points it makes a diagnosis of fibromyalgia more likely

Management

  • Aerobic excericise
  • CBT
  • medication: pregabalin, duloxetine, amitriptyline
39
Q

Compartment Syndrome

characterised by raised pressure within a closed anatomical space

main fractures carrying this complication include supracondylar fractures and tibial shaft injuries.

A

Features

  • Pain, especially on movement (even passive)
  • excessive use of breakthrough analgesia should raise suspicion for compartment syndrome
  • Parasthesiae
  • Pallor may be present

Diagnosis

  • Is made by measurement of intracompartmental pressure measurements. Pressures in excess of 20mmHg are abnormal and >40mmHg is diagnostic
  • Compartment syndrome will typically not show any pathology on an x-ray

Management

  • This is essentially prompt and extensive fasciotomies
  • In the lower limb the deep muscles may be inadequately decompressed by the inexperienced operator when smaller incisions are performed
  • Myoglobinuria may occur following fasciotomy and result in renal failure and for this reason these patients require aggressive IV fluids

The presence of a pulse does not rule out compartment syndrome

40
Q

Vasculitis - Temporal Arteritis

Temporal arteritis (also known as giant cell arteritis: GCA) is a vasculitis of unknown cause that affects medium and large-sized vessels arteries. It occurs in those over 50 years old, with a peak incidence in patients who are in their 70s.

  • There is an overlap between temporal arteritis and polymyalgia rheumatica (PMR) - around 50% of patients will have features of PMR.
A

features

  • typically patient > 60 years old
  • usually rapid onset (e.g. < 1 month)
  • headache (found in 85%)
  • jaw claudication (65%)
  • anterior ischemic optic neuropathy
  • Fundoscopy: swollen pale disc and blurred margins

Ix

  • ESR > 50 mm/hr (note ESR < 30 in 10% of patients)
  • CRP may also be elevated
  • temporal artery biopsy: skip lesions may be present
  • creatine kinase and EMG normal

Management

  • High dose steroids even before biopsy
  • Vision loss: IV methylprednisolone
  • No vision loss: prednislone
41
Q

Bone Tumour’s

A

Osteosarcoma

  • most common primary malignant bone tumour
  • seen mainly in children and adolescents
  • occurs most frequently in the metaphyseal region of long bones prior to epiphyseal closure, with 40% occuring in the femur, 20% in the tibia, and 10% in the humerus
  • x-ray shows Codman triangle (from periosteal elevation) and ‘sunburst’ pattern
  • mutation of the Rb gene significantly increases risk of osteosarcoma (hence association with retinoblastoma)
  • other predisposing factors include Paget’s disease of the bone and radiotherapy
42
Q

autosomal dominant connective tissue disorder.

Marfan’s

A
  • defect in the FBN1 gene on chromosome 15 that codes for the protein fibrillin-1.

Features

  • tall stature with arm span to height ratio > 1.05
  • high-arched palate
  • arachnodactyly
  • pectus excavatum
  • pes planus
  • scoliosis of > 20 degrees
  • dilation of the aortic sinuses (seen in 90%) which may lead to aortic aneurysm, aortic dissection, aortic regurgitation
  • mitral valve prolapse (75%),
  • upwards lens dislocation + blue sclera
43
Q

Ehnlers Danlos

Ehler-Danlos syndrome is an autosomal dominant connective tissue disorder that mostly affects type III collagen

A
  • tissue being more elastic than normal leading to joint hypermobility and increased elasticity of the skin.
  • easy bruising
  • fragile skin
  • aortic regurgitation, mitral valve prolapse and aortic dissection
  • subarachnoid haemorrhage
  • angioid retinal streaks
    *
44
Q

Baker’s cyst

also known as a popliteal cyst

A
  • distension of the gastrocnemius-semimembranosus bursa. They may be primary or secondary
  • They present as swellings in the popliteal fossa behind the knee.
  • common in osteoarthritis
  • Rupture may occur resulting in similar symptoms to a deep vein thrombosis, i.e. pain, redness and swelling in the calf. However, the majority of ruptures are asymptomatic.
  • Baket’s cysts in children typically resolve and do not require treatment.
  • In adults, the underlying cause should be treated where appropriate.

A Baker’s cyst is filled with synovial fluid, which is the natural lubricant found in joints.

45
Q

Conditions Affecting the Knee

A
  • Meniscus tears -
  • ACL tear -
  • MCL Sprain
  • Iliotibial Band Syndrome
  • Patellofemoral pain syndrome
  • Patella Tendonitis
46
Q

Conditions affecting the Hip

A
  • Degenerative Joint Disease
  • Greater Trochanter Bursitis
  • Sacroiliac Joint Dysfunction
  • Transient Synovitis of Hip
  • Sciatica
47
Q

Conditions affecting the Shoulder

A
  • Rotator Cuff Tear/Strain/Tendinopathy
  • Impingement/Subacromial bursitis
  • Adhesive Capsulitis
48
Q

Conditions affecting Elbow

A
  • Biceps Tendinopathy
  • Lateral Epicondylitis - Tennis Elbow
  • Medial Epicondylitis - Golfer’s Elbow
  • Olecranon Bursitis
49
Q

Conditions affecting the Wrist and hands

A
  • Cubital Tunnel Syndrome
  • Carpal Tunnel Syndrome
  • Wrist Ganglions
  • De Quervain’s Tenosynovitis
  • Trigger Finger
50
Q

Conditions affecting the foot and ankle

A
  • Compartment syndrome
  • Plantar Fasciitis
  • Achilles Tendinopathy
  • Morton’s Neuroma