Glomérulonéphrites extracapillaires

Crescentic glomerulonephritis are characterised by a crescent shaped cellular proliferation that may lead to glomerular destruction. Over 50% of at least 10 analysed glomeruli should be affected. The search for immune deposits by immunofluorescence is an important diagnostic step. Patients present with rapidly progressive glomerulonephritis (RPGN): renal failure, proteinuria and haematuria. Extra-renal symptoms may help diagnosis. Diseases are classified in three groups according to immunofluorescence studies. Group I is characterised by linear deposits along the glomerular basement membrane (GBM) with anti-GBM auto-antibodies responsible for Goodpasture's disease. Group II put together various diseases with immune complex deposits. In group III, no significant immune deposits are found. Those “pauci-immune” glomerulonephritis are secondary to anti-neutrophil cytoplasmic antibodies (ANCA) positive systemic vasculitis, mainly Wegener's granulomatosis and microscopic polyangiitis. Primary glomerulonephritis may also be associated with crescent formation. Treatment is urgently required. Diagnosis is suspected in the context of extra-renal symptoms or immunological abnormalities, and confirmed by a kidney biopsy, that also helps to define prognosis. Apart from some group II glomerulonephritis, the induction treatment is often an association of steroids and cyclophosphamide, with plasma exchange in case of Goodpasture's disease. After remission, a maintenance treatment is required for ANCA-positive vasculitis to prevent relapses. The high rate of opportunistic infections and cancer give the rational for searching less aggressive therapeutic options.