Chronic auto-inflammatory disease of unknown origin
Aetiology
- Women in 20-30s most commonly affected
- Higher incidence in African Americans and Hispanics
- Affects skin, joints, kidneys, lungs, nervous system, serous membranes
Pathophysiology
Lupus nephritis
- Up to 50% of lupus patients will have renal involvement at presentation, and up to 60% during the course of their disease
- Most frequently observed abnormality is proteinuria
- A number of types of renal disease can occur and are differentiated by renal biopsy - ISN classification I-VI
Clinical presentation
- Arthralgia, synovitis, arthritis
- 'Butterfly' facial rash, photodermatosis, alopecia
- Anaemia, leukopenia, thrombocytopenia, thromboses
Investigations
- Bloods - raised inflammatory markers, low complement
- Immunology - ANA positive, anti-dsDNA present in ~70%
- Urinalysis - proteinuria +/- microscopic haematuria
Management
- Immunosuppresion - level depends on presentation and severity