P0335PULMONARY HEMORRAHGE IN IG A NEPHROPATHY: A RARE MANIFESTATION

Abstract

Abstract Background and Aims Ig A nephropathy is the most frequent cause of glomerulonephritis worldwide. Renal-limited disease is the habitual clinical form of the disease. Pulmonary involvement may also be seen in rare cases. Capillaritis with pulmonary hemorrhage is the most frequent pulmonary involvement and is lifethreatening. We reported the case of patient presenting a rapidly progressive glomerulonephritis with pulmonary hemorrhage revealing Ig A nephropathy. Method A case report Results A 23 year-old man admitted for a rapidly progressesive glomerulonephritis with sever hypertension. He presented a respiratory distress with hemoptysis. At biology serum creatinine was at 2200µmol/l (eGFR = 2.4 ml/min) and Hemoglobin was at 5g/dl. Immunoassays of anti-glomerular basement antibodies and antineutrophil cytoplasmic autoantibodies were negatives. Computed tomography scan showed diffuse intra alveolar hemorrhage associated with pleural effusion. Bronchoalveolar lavage confirms this diagnosis. Renal histology showed Ig A nephropathy. Treatment consisted of daily depleted haemodialysis with blood transfusion and corticosteroids associated with monthly pulses of cyclophosphamide. His hemoptysis gradually resolved with a stable hemoglobin at 11 g/dL. However, renal failure persisted requiring maintenance hemodialysis regularly. Conclusion Pulmonary hemorrhage is a rare but serious complication of Ig A Nephropathy which is associated with significant morbidity and mortality. The exact cause for this “extra renal bleed” in Ig A Nephropathy is not known but it possibly represents IgA capillaritis. Though no evidence-based treatment protocol exists, therapeutic options includes steroids, plasmapheresis, cyclophosphamide and rituximab.