Autoimmune connective tissue disorders Flashcards
Clinical criteria of SLE
Acute cutaneous lupus
Chronic cutaneous lupus
Oral/nasal ulcers (unexplained, roof of mouth)
Synovitis
Serositis
Proteinuria/red cell casts
Neuro features
Haemolytic anaemia
Leuco or lymphopenia
Thrombocytopenia
Acute cutaneous features of lupus
Malar rash, sparing nasolabial folds
Photosensitive rash
Bullous lupus (like TEN)
Chronic cutaneous features of lupus
Discoid rash (90% won’t have systemic involvement)
Raised erythematous patch > pigmented hyperkeratotic oedematous papules > atrophic, depressed, lesions
Profundis: dusky, deep fat inflammation
Serositis features in SLE
Lung: pleurisy, pleural effusion, pleural rub
Heart: Pericarditis, pericardial pain, pericardial effusion, pericardial rub
Lab investigations for lupus
ANA – NOT SPECIFIC
Anti-dsDNA/Anti-Smith – MOST SPECIFIC
Antiphospholpid
Low complement
+ve Coombs test
Pathophysiology of SLE
Autoimmune reaction to nuclear components > formation (and failure of clearance) of immune complexes
What is Libman-Sacks syndrome?
Non-infective endocarditis assoc w/ SLE
Monitoring SLE disease activity
Disease activity scores
FBC, U+e
CRP/ESR (CRP nomal)
Complement
Anti-dsDNA titres
Features of antiphospholipid syndrome
Coagulopathy
Livedo reticularis
Obstetric (recurrent miscarriage)
Thrombocytopenia
Anti-cardiolipin or lupus anticoagulant (prolongation of coagulation studies not corrected w/ serum c.f. haemophilia)
Treatment of mild SLE
NSAIDs, sunblock, HCQ
Treatment of SLE flares
Immunosuppression (dose + choice depends on severity)
SLE and pregnancy
Antiphospholipid/anti-ro/anti-la: Obstetric complications
Cyclophosphamide: Infertility
Mycophenolate: Craniofacial abnormalities
Use HCQ as safe!
Feature of mixed connective tissue disease
High anti-U1 RNP antibodies
SLE, systemicsclerosis, polymyositis mixed features
Prognosis in SLE
80% survival at 15 years
Features of limited sytemic sclerosis (aka CREST)
Scleroderma (face, hands, feet, spares trunk)
Telengiectasia
Esophogeal dysmotility
Raynaud’s
Subclinical pulmonary hypertension > may become life-threatening
Anti-centromere antibodies
Management of pulmonary hypertension in limited systemic sclerosis
Sildenafil, bosentan
Diffuse systemic sclerosis antibody tests
Anti-topoisomerase-1 (SCL-70) and anti-RNA polymeraase
Management of systemic sclerosis
BP, echocardiogram, spirometry monitoring
Manage symptoms
Steroids for ILD
Antihistone antibodies
Drug-induced lupus
Anti-Ro antibodies
Sjogrens
Systemic sclerosis
SLE