Background
- Chronic inflammatory disease of connective tissue with multi-organ involvement
- More severe and more frequent in non-caucasions
- F > M
- ? Oestrogen as those on OCP more susceptible
- Multiple anigens and auto-antibodies
- 4 or more manifestations (below)
Clinical Manifestations
- Serositis (pleuritis, pericarditis)
- Oral ulcers
- Arthritis (nonerosive)
- Photosensitivity (exposure to UV radiation causes skin manifestations)
- Blood dyscrasias (hemolytic anemia, leukopenia, thrombocytopenia)
- Renal manifestations (proteinuria, casts)
- Positive ANA finding
- Immunologic disorders (anti-Smith antibodies positive, antiphospholipid antibody positive, false positive test for syphilis, presence of anti–double-stranded DNA antibodies)
- Neurologic manifestations (seizures, focal signs, psychosis)
- Malar (butterfly) rash (photosensitive)
- Discoid rash
Complications
Renal disease (30%-60%)
- Proteinuria
- Active urinary sediment (> 5 RBC per high powered field)
- Pyuria
- Casts
- Urinary monitoring in inactive disease
- Renal biopsy in all with signs of kidney involvement
- (glomerulonephritis clasification and grading)
- May need repated Bx with flare ups
Respiratory
- Pleural inflammation is common
- Chest pain, SOB, cough
- Pleural effusions typical in ANA-positive exudates with low complement
- Occasional pulmonary hypertension or interstitial disease
Labs
- Anemia (normochromic normocytic or Fe++ deficient
- Leukopenia (lymphopenia - not granulocytopenia), thrombocytopenia (disease or drug effects)
- Antiphospholipid disorder (thrombotic disorder)
- Anticardiolipin antibodies give a false positive test for syphilis
Treatment
- Suppress flares
- Maintenance prevent organ damage
Mild disease
( arthritis, dermatitis, and constitutional symptoms)
NSAIDs, hydroxychloroquine, and low-dose steroids
Severe disease
High dose steroids with cyclophosphamide to induce remission, followed by longer-term, immunosuppressive therapy