Rheumatology Flashcards
What are the main ANCA associated vasculitis conditions?
- granulomatosis with polyangiitis
- eosinophilic granulomatosis with polyangiitis
- microscopic polyangiitis
Common features of ANCA associated vasculitis:
- renal impairment: immune complex GN (haematuria, proteinuria, increased creatinine)
- respiratory: dyspnoea, haemoptysis
- systemic: lethargy, weight loss, fatigue
- vasculitic rash
- sinusitis
cANCA (target, association, monitoring)
- seine proteinase 3
- mostly granulomatosis with polyangiitis
- some microscopic polyangiitis
- some correlation with disease activity
pANCA (target, association, monitoring)
- myeloperoxidase
- mostly eosinophilic granulomatosis with polyangiitis
- some microscopic polyangiitis
- also in UC, primary sclerosis cholangitis, anti GBM, Crohn’s
- no correlation with disease activity
Typical presentation of ankylosing spondylitis:
- HLA-B27 associated spondyloarthropathy
- more common in males 3:1 20-30yo
- present with lower back pain and stiffness, worse in morning, at night, improves with exercise
- reduced lateral flexion, forward flexion and chest expansion
What are the 8 A’s of ankylosing spondylitis:
- apical fibrosis
- anterior uveitis
- achilles tendonitis
- aortic regurgitation
- amyloidosis
- peripheral arthritis
- cauda equina
- AV node block
Investigations and signs of ankylosing spondylitis:
-increased CRP and ESR
-HLA B27 positive
x-ray sacroiliac joints:
-sacroiliitis: subchondral erosion, sclerosis
-squaring of lumbar vertebrae
-bamboo spine (late, rare)
-syndesmophytes
-apical fibrosis on CXR
spirometry: restriction
Management of ankylosing spondylitis:
- exercise
- physio
- NSAIDs
- DMARD if peripheral joint involvement
- anti-TNF for persistent disease (etanercept, adalimumab)
What is antiphospholipid syndrome and what are the typical features?
- acquired disorder of arterial and venous thromboses, recurrent foetal loss and thrombocytopaenia
- paradoxical increase in APTT
- other symptoms: livedo reticularis, pulmonary hypertension, pre-eclampsia
What are the associations of antiphospholipid syndrome and the management?
- associated: SLE, autoimmune, lymphoproliferative disorders, phenothiazines
- primary prophylaxis: aspirin
- secondary prophylaxis: lifelong warfarin (target INR 2-3 for initial and arterial, 3-4 if occurred whilst taking aspirin and warfarin)
What is antisynthetase and the features:
- autoantibodies against aminoacyl-tRNA synthetase e.g. Jo-1
- myositis
- interstitial lung disease
- dry and cracked skin of hands
- Raynaud’s
What is Behcet’s, epidemiology and features?
-multisystem disorder of autoimmune mediated inflammation of arteries and veins
-typically eastern mediterranean, men, young, HLA B51 positive, 30% FHx
Features:
-triad: genital ulcers, oral ulcers, anterior uveitis
-thrombophlebitis and deep vein thrombosis
-aseptic meningitis
-arthritis
-GI: colitis, abdominal pain, diarrhoea
-erythema nodosum
Calcium, phosphate, ALP and PTH levels in osteoporosis, osteopetrosis, Paget’s, CKD and osteomalacia:
- everything normal in osteoporosis and osteopetrosis
- Paget’s: everything normal except ALP increased
- CKD: calcium decreased, everything else increased
- Osteomalacia: calcium and phosphate decreased, everything else increased
What qualifies as CFS?
4 months of disabling fatigue affecting mental and physical function more than 50% of the time
more common in females
What can dactylitis be caused by?
- spondyloarthopathies e.g. psoriatic and reactive arthritis
- sickle cell
- rare: TB, sarcoidosis, syphilis
What is dermatomyositis?
- inflammatory disorder causing symmetrical, proximal muscle weakness and skin lesions
- idiopathic or associated with CTD or underlying malignancy
- polymyositis is variant with less skin manifestation
What are the features of dermatomyositis?
skin:
- photosensitivity
- macular rash over back and shoulder
- heliotrope rash in periorbital region
- mechanic’s hands
- Gottron’s papules
- nailfold capillary dilatation
- proximal muscle weakness and tenderness
- Raynaud’s
- respiratory muscle weakness
- interstitial lung disease
- dysphagia, dysphonia
What are the investigations of dermatomyositis?
- 80% ANA +ve
- 30% Ab to aminoacyl-tRNA synthetase (Jo-1, SRD, anti-Mi-2)
What are the features of drug induced lupus?
- arthralgia
- myalgia
- malar rash
- pleurisy
- ANA +ve
- dsDNA -ve
- anti-histone Ab +ve
- some anti-Ro, anti-Smith
Causes of drug induced lupus?
common: procainamide, hydralazine
rare: isoniazid, minocycline, phenytoin
What is EDS and what are the typical features?
- autosomal dominant CTD affecting type III collagen
- joint hypermobility
- elastic, fragile skin
- easy bruising
- aortic regurgitation, aortic dissection, mitral valve prolapse
- SAH
- angioid retinal streaks
How can you treat fibromyalgia?
- pregabalin
- duloxetine
- amitriptyline
Presentation of gout:
- form of inflammatory arthritis
- microcrystal synovitis - deposition of monosodium urate monohydrate in synovium
- chronic hyperuricaemia (uric acid >0.45mmol/L)
- episodes last several days with no symptoms in between
- pain, swelling, erythema
- most present 1st MTP joint
Radiological features of gout:
- joint effusion early sign
- well defined erosion with sclerotic margins in juxta-articular distribution
- relative joint space preservation until late
- eccentric erosions
- no periarticular osteopaenia unlike RA
- soft tissue tophi