Systemic Lupus Erythema (SLE) Flashcards
what is it?
-Chronic AI disease
pathogenesis (proposed)
-Defect in apoptosis causes increased cell death
-Defective clearance of apoptotic cell debris causes persistence of antigen and immune complex production
-Circulating immune complexes with antigens in various tissues
- in small blood vessels - complement activation and inflammation - basement membranes of skin and kidneys
who gets it?
black»white though low prevalence in Africa
90% cases in women normally of child bearing age
Onset after puberty - 20s-30s
what is the prognosis?
currently 10yr survival exceeds 90%
Previously mortality was due to the disease itself now mortality is a result of side effects of medication - infections or CV events
constitutional presentation
- Fever
- Fatigue
- Weight loss
- Pulmonary embolism
- Pulmonary hypertension
- Interstitial lung disease
musculoskeletal presentation
- Arthralgia
-Myalgia
-Inflammatory arthritis
-Arthopathy rarely erosive/ derforming
mucocutaneous presentation
-Malar rash
-Photosensitivity
-Discoid lupus/ Subacute cutaneous lupus: oral/nasal ulceration + Raynaud’s
renal presentation
lupus nephritis
respiratory presentation
- pleurisy
- pleural effusion
- pneumonitis
haematological presentation
- leukopenia
- lymphopenia
- anaemia
- thrombocytopenia
neuropsychiatric presentation
seizures
- psychosis
- headache
- aseptic meningitis
cardiac presentation
- pericarditis
- pericardial effusion
- pulmonary hypertension
- sterile endocarditis
- accelerated ischaemic heart disease
Gastrointestinal presentation
- autoimmune hepatitis
- pancreatitis
- mesenteric vasculitis
what are the investigations?
no one diagnostic test
- FBC may show - anaemia - leucopenia - thromobocytopenia
-Urinalysis - look for glomerulonephritis
-Imaging - organ involvement (CT, MRI, echo)
antibodies to test
- ANA - high sensitivity (>95% of patients)
- Anti-dsDNA
- Anti-SM - low sensitivity -
- specific c3/4 - low sensitivity when disease is active