A multi-system autoimmune disease predominantly affecting young women.  Typically presents with joint pains and malaise, quite often with fever, and a characteristic erythematous facial ‘butterfly’ rash. Pleurisy is another common early symptom.  However the range of possible manifestations is very large.  Antinuclear antibodies and antibodies to double-stranded DNA are typical.  Serum complement levels may be low.

Renal involvement may occur in 50% but is often minor.  Major renal disease indicates serious disease. Patients typically present with the inflammatory signs of haematuria, proteinuria, high blood pressure. Nephrotic syndrome is also a very common presentation.  SLE is an important cause of serious renal disease in young women.  It is more common in black and particularly some East Asian races.

The most common histological pattern is an inflammatory, diffusely proliferative glomerulonephritis.  Appearances are highly variable but prognostically important.

Corticosteroids are required for most types of lupus affecting the kidneys, though some regimens are now minimising this.  For aggressive disease, treatment with cyclophosphamide (usually in regular pulses) reduces the risk of progression to ESRF, but less toxic alternatives are increasingly the first choice.  Mycophenolate mofetil (MMF) is effective for many.  Cyclophosphamide is usually stepped down to azathioprine or other cytotoxic agent after a variable period. Anti-B cell antibodies (Rituzimab) may also be effective.

If ESRF is reached despite treatment, SLE tends to become relatively quiescent. Dialysis and transplantation are successful.

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