Rheumatology conditions Flashcards
What antibodies are involved in Rhematoid arthritis?
- IgG Rheumatoid Factor
- anti-CCP (citrullinated cyclic peptide)
What is the pathophysiology of RA?
- Citrullation of self antigens which are then recognised by T&B cells
- Macrophages are stimulated and release TNFalpha
- There is an inflammatory cascade leading to proliferation of synoviocyes
- Synoviocytes grow over cartilage and restrict nutrients
What is the typical history of a patient with RA?
- Usually female
- 30-50 years old
- Stiffness is symmetrical, progressive and peripheral
- Morning stiffness for >30mins duration
- Usually involves with MCPs/PIPs/ MTPs (but spares the DIPs) but can affect any joint
What will you find on examination of a patient with RA?
- Soft tissue swelling and tenderness
- Ulnar deviation
- Palmar subluxation of MCPs
- Swan neck deformity
- Boutonniere deformity
- Rheumatoid nodules (at the elbow)
- May have median nerve involvement
What investigations would you order to confirm RA?
- RF and anti-CCP
- FBC- may have normocytic aneamia (aneamia of chronic disease)
- Inflammatory markers
- Can request X-ray but changes only appear in more established disease
- USS/MRI is more sensitive in early disease
What X ray findings will you find on a patient with RA?
- Loss of joint space
- Erosions (periarticular)
- Soft tissue swelling
- Subluxation
How do you treat RA?
- Initially DMARD therapy - methotrexate
- Can give combination DMARDs
- Steroids for acute flares
- Symptoms control with NSAIDs (+PPI cover)
- Severe disease can consider biologics
Give some of the extra-articular manifestations of RA?
3 CAPS
3Cs
- Carpal tunnel syndrome
- Cord compression (atlanto-axial subluxation)
- elevated Cardiac risk
3As
- Anaemia (normocytic & normochromic)
- Amyloidosis (rare now)
- Arteritis (rare)
3Ps
- Pericarditis (uncommon)
- Pleural disease (common)
- Pulmonary disease (common) e.g. bronchiectasis
3Ss
- Sjögren’s
- Scleritis/ episcleritis
- Spenic englargement (if + neutropenia = Felty’s syndrome (rare))
What is Giant Cell Arteritis?
Chronic vasculitis of large and medium sized vessels
(also called temporal arteritis)
What are some of the risk factors for developing giant cell arteritis?
- Age (greatest risk factor) >90% are older than 60
- Increased prevalence in northern latitude
- More common in women
- Common in whites
- Strong link with polymyalgia rheumatica
- Genetic predisposition: HLA-DR4
What are some of the symptoms of giant cell arteritis?
- Headache
- often localised, unilateral over the temple
- Tongue or jaw claudication on mastication
- Visual symptoms are an emergency!
- Amaurosis fugax
- Blindness
- Diplopia
- Blurring
- Scalp tenderness
How do you diagnose giant cell arteritis?
Presnce of any 2 or more of the following in patients >50yrs
- Raised ESR, CRP or PV
- New onset localised headache
- Tenderness or decreased pulsation of temporal artery
- New visual symptoms
- Biopsy revealing necrotising arteritis
How do you treat Giant Cell Arteritis?
- Prednisolone 60-100mg PO for at least 2 weeks before considering slow tapering
- For acute onset visual symptoms:
- consider 1g methyprednisolone IV pulse therapy for 1-3 days
- Low dose aspirin reduces thrombotic risks
What is Polymyalgia Rheumatica?
Clinical syndrome of pain and stiffness in the shoulder, hip girdles and neck
Describe the typical history of a patient with polymyalgia rheumatica
- Elderly patients with new sudden onset proximal limb pain and stiffness (neck, shoulders, hips)
- Have difficulty rising from the chair or combin hair
- Pain is worse at night
- Systemic symptoms (25% cases): fatigue, weight loss, low grade fever)
What will you find on physical examination of a patient with polymyalgia rheumatica?
- Decreased ROM of shoulders, neck, hips
- Muscle stregnth is usually normal
- Muscle tenderness
How do you treat polymyalgia rheumatica?
- 15mg Prednisolone daily - will see rapid improvement within 5 days (confirms diagnosis too, if not seen reconsider diagnosis)
- Dose can be tapered very slowly
- Methotrexate can steroid sparing in relapsing patients
What are spondyloarthropathies?
Group of conditions that affect the spine and the peripheral joints
- Ankylosing spondylitis (most common)
- Enteropathic arthritis
- Psoriatic arthritis
- Reactive arthritis
Which gene is associated with spondyloarthropathies?
HLA-B27
What are the common features of all spondyloarthopathies?
- Sacro-iliac/ axial disease (back/buttock pain)
- Inflammatory arthropathy of peripheral joints
- Entheisitis (inflammtio at tendon insertions)
- Extra articular features (skin/gut/eye)
How does a patient with anklyosing spondylsis present?
Usually a young man (teens-mid thirties)
Presents with bilateral buttock , chest wall and thoracic pain
Examination is usually normal but will later lose lumbar lordosis and have an exaggerated kyphosis
What investigations can you do to help diagnose ankylosing spondylisis?
-
Schober’s Test - mark skin 10cm above and 5 cm below PSIS
- ask patient to bend forward with straight legs, increase of >20 cm is normal
- Will have reduced chest expansion
- CRP may be raised by often normal
- MRI of spine and SI joints
How do you treat ankylosing spondylsis?
- NSAIDs and physio
- TNF inhibitors
- IL-17 inhibitors
What typical exam findings will you find on a patient with psoriatic arthritis?
- Oligo-arthritis with dactylitis (sausage like fingers)
- Can be symmetrical or mono-arthritis
How do you treat psoriatic arthritis?
- NSAIDs
- DMARDs
- TNF inhibitors
- IL-17 inhibitors
- IL-12/23 inhibitors
What is reactive arthritis?
- Sterile synovitis developing after a distant infection
- Either post dysentry (Salmonella/ Shigella/ Campylobacter)
- or, following urethritis/ cervicitis (Chlamydia trachomatis)
How does reactive arthritis present?
- Usually presents 2 weeks post infection as acute asymmetrical lower limb arthritis
- Skin: circinate balantitis - rash on penis
- Keratoderma blennorrhagica
- Eye: conjunctivitis
- Enthesitis
How do you treat reactive arthritis?
- Treat underling infection - may not improve the arthritis
- NSAIDs and joint injections
- Most resolve in 2 years- if not may need DMARDs
What is enteropathic arthritis?
- 10-20% of patients with IBD develop arthropathy
- 2/3 develop peripheral arthritis
- 1/3 develop axial disease
What are the 2 types of peripheral disease in enteropathic arthritis?
Type 1: oligoarticular, asymmetric and has a correlation with IBD flares
Type 2: polyarticular, symmetrical and less correlation with IBD flares
How do you treat enteropathic arthritis?
- Consider DMARDS
- TNF inhibitors will treat both bowel disease and arthritis
- Remember: NSAIDs may flare IBD so best to avoid
What are some of the extra- articular manifestations of ankylosing spondylitis?
All the A’s
- Anterior uveitis
- Aortic incompetence
- AV block
- Apical lung fibrosis
- Amyloidosis
What are some of the features of inflammatory back pain?
- Insidious onset
- Pain at night (improves with waking)
- Age of onset <40 years
- Improves with exercise
- No improvement with rest
What is Systemic lupus erythematosus?
- autoimmune disease
- inadequate T cell suppressory activity with increased B cell activity
- complex multisystemic disease with variable presentations
- characterised by remissions and flares
What are some of the signs and symtpoms of SLE?
SOAP BRAIN
- Serositis - pleurisy, pericarditis
- Oral ulcers - usually painless, palate is mores specific
- Arthritis- of small joints (non erosive)
- Photosensitivity - malar or discoid rash
- Blood disorders- low WCC, lymphopenia, thrombocytopenia, haemolytic anaemia
- Renal involvement - glomerulonephritis
- Autoantibodies (ANA +)
- Immunological test - low complement
- Neurologic disorder - seizures or psychosis
What investigations can be done to investigate lupus?
- ESR / plasma viscocity (will be raised)
- 95% are ANA +
- Anti-Ro, Anti La are common
- Anti dsDNA titre rises with disease activity
- Urinalysis - vital for detecting renal disease
- Skin biopsy- can be diagnostic
- Renal biopsy- can be diagnostic
How do you treat lupus?
- Sun protection
- Healthy lifestyle to reduce CV risk
- Hydroxychoroquine is helpful for rashes and arthralgia
- Mycophenolate mofetil
- Azathioprine
- Rituximab
- Short course prednisolone for flares
What is Raynaud’s Phenomenon?
Painful condition due to vasospasm of the digits
Painful and characterised by colour changes in response to cold stimulus and stress
White> blue > red
What is Raynaud’s syndrome?
Term used when Raynaud’s phenomenon is idiopathic
Common in young women, most improve with age
Advise to avoid smoking and keep warm
What diseases are associated with raynaud’s phenomenon?
- Scleroderma
- SLE
- Dermatomyositis and Polymyositis
- Sjogren’s syndrome
What are some physical causes of Raynaud’s?
- Smoking
- Use of heavy vibrating tools
- Sticky blood e.g. cryoglbulinaemia
- Drug induced: beta blockers
How do you treat Raynaud’s Phenomenon?
- Keep warm
- stop smoking
- CCB can be used first line
- Phosphodiesterase-5-inhibitors usually effective
What are some of the complications of Raynaud’s phenomenon?
- Digital ulcers
- Severe digital ischemia
- Infection
What is vasculitis?
An inflammatory blood vessel disorder
How do you take a history to diagnose vasculitis?
- Consider age, gender, ethnicity
- Comprehensive drug history
- Family history of vasculitis
- Consitutional symptoms : fever, weight loss, malaise, fatgue, diminished appetite, sweats
- Ask about Raynaud’s
- MSK: arthralgia, myalgia, proximal muscle weakness?
- CNS/PNS: headaches, visual loss, tinnitus, stroke, seizure, encephalopathy
- Nose bleeds, crusts, ulcers
- Heart lung: pericarditis, cough, chest pain, haemoptysis, dyspnoea
- GI: abdo pain
- Renal: haematuria
- Limbs: neuropathy, digital ulcers/ ischemia
What should you look for on examination of a patient with vasculitis?
- Vital signs: hypertension, pulse regularity and rate
- Skin: palpable purpura, livedo reticularis nodules (mottled skin), digiatal ulcersm gangrene, nail bed capillary changes
- Neurologic: CN exam, sensorimotor exam
- Ocular exam: visual fields, scleritis, uveritis, episcleritis
- Cardiopulmonary exam: crackles, pleural rubs, murmurs, arrhythmias
- Abdo exam: tenderness, organomegaly
What are the primary vasculitides (small, medium and large vessels)?
Small vessel vasculitis
- Microscopic polyangiitis (MPA)
- Granulomatosis with polyangiitis (GPA)
- Eosinophilic granulomatosis with polyangiitis (formerly Churg Strauss)
- IgA vasculitis
Medium vessels
- Polyarteritis nodosa
- Kawasaki disease
Large vessel vasculitis
- GCA
- Takatasu arteritis
What tests should you do if suspecting vasculitis?
- Urine dip (renal involvement is usually silent)
- FBC, U&E, LFTs, CRP, PV, ESR
- Complement levels
- Hepatitis screen for B, C, HIV
How do you treat vasculitis?
- Rule out infection
- Stop any offending drugs
- 1st line: Corticosteroids
- 2nd line: Cytotoxic medications, immunomodulatory or biologics
What are Dermatomyositis and polymyositis?
Rare idiopathic muscle diseases that are characterised by inflammation of striated muscles
What is the diagnostic criteria for dermatomyositis and polymyositis?
- Symmetrical proximal muscle weakness
- Raised serum muscle enzyme markers
- Electromyographic changes
- Biopsy evidence of myositis
- Typical rask of dermatomyositis
- PM if > 3 of first 4 above
- DM if rash and _>_2 of the first 2
How do you treat dermatomyositis and polymyositis?
- High dose corticosteroids main stay for first few weeks
- Monitor CK and inflammatory markers
- Long term control with MTX and AZA or Rituximab
- Use sun protection in DM to reduce the rash
What is the link between dermatomyositis and malignancy?
- Patients with DM have increased risk of malignancy in 2-3 years before and after DM diagnosis
- Usually followed for 5 years
Why are patients with dermatomyositis and polymyositis at risk of aspiration pneumonia or respiratory failure?
The upper oesophagus has striated muscle therefore swallowing may be affecting increasing risk of aspiration pneumonia
The diaphragm involvement may lead to respiratory failure
What visual features do you see in dermatomyositis
- Gottron’s papules (linear plaques on the dorsal aspect of hands)
- Dilated nail fold capillaries
- Periorbital oedema - violet rask to eye lids (heliotrope rash)
What medications should you consider stopping in patients with recurent flares of gout?
Thiazides
e.g. indapamide
bendroflumothiazide
What are the crystal differences between gout and pseudogout?
Gout: monosodium urate cystals
- negatively birefringent
- needle shaped
Pseudogout: Calcium pyrophosphate crystals
- positively birefringent
- rhomboid shaped
How do you diagnose polymyalgia rheumatica?
- Typically based off history and exam supported by raised inflammatory markers
- Check ESR, PV and CRP
- Consider temporal artery biopsy if there are symptoms of GCA
What is hypermobility spectrum disorder?
A pain syndrome in people with joints that move beyond normal limits
May affect a number of joints
Due to the laxity of ligaments, capsules and tendons. though the origin of pain is from microtrauma
What are some of the signs and symptoms of hypermobility spectrum disorder?
- Pain around the joints worse after activity
- General pain and fatigue
- May have
- soft tissue rheumatism e.g. epicondylitis
- abnormal skin: scars, hyperextensible, thin, straie
- marfanoid habitus
- arachnodactyly
- drooping eyelids/ myopia
- hernia
- uterine/ rectal prolapse
What treatment is offered for hypermobility spectrium disorder?
Mainstay is non-drug treatment
- strengthening exercises to reduce joint subluxation
- work on posture and balance
- splinting or surgical intervnetions
- specialist pain management
Pharmacologically: paracetamol is the main stay
Give some heritable connective tissue disorders
- Marfan’s syndrome
- Ehler’s Danlos syndrome
What is defective in marfan’s sydrome?
- Fibrillin
What is osteoarthritis?
A degenerative joint disorder with progessive loss of articular cartilage accompanied by new bone formation and capsular fibrosis
What happens to cartilage in OA?
- Loss of elasticity with reduced tensile stregnth
- Cellularity and proteoglycan content are reduced
What are some of the risk factors for developing OA?
- Age main factors - present in 80% of people age 65+
- Women > men
- Obesity
- Trauma and joint malalignment
Which joints are most commonly affected by OA?
- Hip
- Knee
- Spine
What are the signs and symptoms of OA?
- Pain provoked by movement and weight bearing
- starts intermittently but can become constant
- Feeling joint may give way (knee)
- Joints feel ‘stuck’ after inactivity
What are the X-ray findings of OA?
- Loss of joint space
- Osteophytes
- Subchondral cysts
- Subarticular sclerosis
What non drug therapy can be offered for OA?
Should be offered to all
- Strengthening exercises
- Weight loss
- Use of laterally wedged insoles reduces joint loading
- Walking sticks
What is the pharmacological management of OA?
- Regular paracetamol (1st line)
- NSAIDs should only be used short term
- Topical NSAIDs
- Intraarticular corticosteroid injections can be offered
When are surgical options to OA offered?
- If conservative management fails
- Young patient have higher change of revision surgery so best to wait as long as possible
What is fibromyalgia?
Chronic widespread pain in all 4 quadrants of the body
What are some of the triggers for fibromyalgia?
-
Sleep disturbance is main cause
- causes hyper-activation in response to noxious stimulation
Give some of the signs and symptoms of fibromyalgia
- Pain- heightened to innocuous stimuli
- joint/muscle stiffness
- profound fatigue
- unrefreshed sleep
- numbness
- headaches
- irritable bowel/bladder
- depression and anxiety
- poor concentration and memory
What will you find on examination in fibromyalgia?
There should be no physical abnormalities or neurological signs
Examine for tender points on palpation of muscles
(no specific diagnostic test)
Give some risk factors of fibromyalgia
- Female: male 9:1
- Peak onset 40-50yrs
- May have ovious trigger (emotional or physical)
How is fibromyalgia treated?
Tailor to pain intensity, function and associted features (sleep distubance, depression, fatigue)
Simple meausures to improve sleep and physical activity levels.
Most patients improve with explanation of symtpoms
Drugs: low dose amitryptyline (opiates are not reccommended)
CBT is effective
What is osteoperosis?
Skeletal condition of low bone mass, deterioration of bone tissue and disruption of bone architecture
Leads to compromised bone stregnth and increased risk of fracture
What are some of the non modifiable risk factors for OA?
- Age >65 yrs
- Female
- Caucasian or south asian
- FHx of osteoporosis
- History of low trauma fracture
What are some of the modifiable risk factors of osteoporosis?
- Low BMI <21
- Premature menopause (<45)
- Calcium/ vitamin D deficiency
- Inadequate physical activity
- Cigarette smoking
- Excessive alcohol intake (>3 drinks/day)
- Iatrogenic e.g. corticosteroids, aromatase inhibitors
How do you diagnose osteoporosis?
- DEXA scan (dual energy x-ray absorptiometry) of lumbar spine and hip is gold standard
- T score <-2.5 = osteoperosis
- normal T score is > -1
- osteopenia if t score between -1 and -2.5
- Plain radiographs lack sensitivty to diagnose OP but can show rib fractures of vertebral compression fractures without history of trauma which should warrant investigation
How is osteopenia managed?
- weight bearing exercises
- Vitamin D3 supplements
- limit alcohol
- smoking cesation
- dietary advice (calcium intake)
How is osteoperosis managed?
Vitamin D3 + calcium supplements plus:
- First line: oral bisphosphonates (or IV if oral not tolerated)
- Second line : Denosumab or terparatide
What adive should you give someone taking alendronic acid?
- Take once a week
- Take first thing in the morning before having anything to E+D to avoid GI upset
- Sit upright for at least 30 minutes after taking
What are some of the secondary causes of osteoporosis?
- Coeliac disease
- Eating disorders
- Hyperparathyroidism
- Hyperthyroidism
- Multiple myeloma
- Hypogonadism in men
What is one of the main risks of taking bisphosphonates e.g. alendronic acid?
Osteonecrosis of the jaw
The Z score on DEXA scans is adjusted for which parameters?
- Age
- Gender
- Ethnic factors
