Connective Tissue Disease Flashcards
Who most commonly gets SLE?
Women (90%)
Usually presents age 20-30
More common and more severe in Afro-Caribbean, Hispanic American, Asian and Chinese
Which organs are most commonly affected by SLE?
Skin Joints Kidneys Blood cells Nervous system
What are the constitutional features of SLE?
fever
fatigue
weight loss
What are the musculoskeletal features of SLE?
Arthralgia, myalgia
Arthritis - synovitis/tenderness of at least 2 joints with early morning stiffness > 30 mins, non erosive (Jaccoud arthropathy)
What are the mucocutaneous features of SLE?
Malar/butterfly rash Photosensitivity Discoid lupus Subacute cutaneous lupus Oral/nasal ulceration Raynaud's phenomenon Alopecia
What are the renal features of SLE and how are they diagnosed?
Lupus nephritis
- proteinuria > 0.5g in 24 hours
- red cell casts on renal biopsy
Which cardiorespiratory features may be seen in SLE?
Pleural or pericardial effusion
Acute pericarditis
Which haematological features are seen in SLE?
Leukopenia
Thrombocytopenia
Haemolytic anaemia
Lymphadenopathy
Which neuropsychiatric features are seen in SLE?
Delirium Psychosis Seizures Headache Cranial nerve disorders
Which investigations should be done for suspected SLE?
FBC (anaemia, leukopenia, thrombocytopenia) Autoantibodies C3 + C4 Urinalysis Imaging for specific organ involvement
Which autoantibodies may be positive in SLE?
ANA Anti-dsDNA Anti-Sm Anti-Ro Anti-La Anti-RNP Antiphospholipid antibodies
Which autoantibody is most specific for SLE?
Anti-dsDNA (but varies with disease activity)
How is complement affected in SLE?
C3 and C4 are low when disease is active, especially in renal and haematological disease
What is the first line drug management for SLE?
Hydroxychloroquine + topical steroids + NSAIDs
Which score is used to monitor disease activity in SLE?
SLEDAI score
What monitoring should be done in a patient with SLE?
Monitor anti-dsDNA + complement as they vary with disease activity
Urinalysis for nephritis
Evaluate/manage CVD risks as this is major cause of mortality
What are the clinical features of sjogren’s syndrome?
Dry eyes and mouth (sicca symptoms) Gritty feeling in eyes Vaginal dryness Bilateral parotid enlargement Joint pain Fatigue Unexplained increase in dental caries
Risk of which cancer is increased in sjogren’s syndrome?
Lymphoma
How is Sjogren’s syndrome diagnosed?
Clinical features
Schirmer’s test –> confirm ocular dryness
+ve anti-Ro and anti-La
What is the management for Sjogren’s syndrome?
Symptomatic:
- eyedrops + saliva replacement
- pilocarpine to stimulate saliva production (causes flushing)
Dental care
Hydroxychloroquine may help with arthralgia and fatigue
Which antibodies are associated with mixed connective tissue disease?
Anti-RNP antibodies
What are the clinical features of anti-phospholipid syndrome?
Recurrent venous or arterial thrombosis +/- foetal loss Stroke/MI in young patients Libman Sacks (sterile) endocarditis Recurrent PE --> severe pulmonary HTN Migraine Livedo reticularis
What is Livedo reticularis?
Mottled, lace-like discolouration on skin
Which investigations should be done for suspected anti-phospholipid syndrome?
Bloods: thrombocytopenia + prolonged APTT Anti-phospholipid antibodies: - lupus anticoagulant - anti-cardiolipin - anti-beta 2 glycoprotein
What is the management for anti-phospholipid syndrome?
Anticoagulation (do not need to treat if +ve antibodies but no episode of thrombosis)
If recurrent miscarriage –> LMWH during pregnancy (warfarin is teratogenic)
What is another name for systemic sclerosis?
Scleroderma
What are the classic symptoms in systemic sclerosis?
Raynaud's (almost always) Skin thickening and tightening Dysphagia GORD SOB Telangiectasis Calcinosis (SC calcium deposits in fingers) Sclerodactyl
How is systemic sclerosis classified?
Diffuse cutaneous SSc:
- skin involvement on extremities above AND below elbows and knees (+ face + trunk)
Limited cutaneous SSc:
- skin involvement only below elbows + knees on extremities (+ face)
Which antibodies are associated with diffuse systemic sclerosis?
Anti-Scl-70
Which antibodies are associated with limited systemic sclerosis?
Anti-centromere
Which facial features are seen in systemic sclerosis?
Small puckered mouth
Beaked nose
Lack of wrinkles
Telangectasia
Apart from skin, which other clinical features may be seen in systemic sclerosis?
Pulmonary HTN, pulmonary fibrosis + accelerated HTN –> renal crisis
Dysphagia, malabsorption + bacterial overgrowth of small bowel
Inflammatory arthritis and myositis
How is Raynaud’s/digital ulcers managed in systemic sclerosis?
CCBs –> nifedipine first line
Others: fluoxetine, ARBs, nitrates, iloprost, bosentan
How is a renal crisis treated in systemic sclerosis?
ACE inhibitors
May need dialysis
How is pulmonary hypertension treated in systemic sclerosis?
PDE5 inhibitor e.g. Sildenafil
Bosentan
Oxygen
How is interstitial lung disease managed in systemic sclerosis?
Mycophenolate mofetil or cyclophosphamide
