Circulating monoclonal IgG1-kappa antibodies causing anti-glomerular basement membrane nephritis

Anti-glomerular basement membrane (GBM) antibody disease is a rare but well-characterized cause of glomerulonephritis. Patients present with rapidly progressive renal failure with hemoptysis. Early diagnosis is crucial in salvaging the renal damage and life-threatening pulmonary hemorrhage. Plasmapheresis and immunosuppression is the mode of therapy. Anti-GBM antibodies are polyclonal in nature. However, rare monoclonal antibodies can cause similar destruction of glomerular capillary walls. We describe distinct combination of circulating monoclonal and anti-GBM nephritis

Dense deposit disease involving C3 and C4d Deposits

Dense deposit disease (DDD), earlier called Type II membranoproliferative glomerulonephritis is distinct disease having frequent relapses reaching end-stage kidney disease by 10-year in up to 50%–60% of cases and high recurrence rate in the allograft. The term DDD is derived from its distinctive ribbon-like osmiophilic deposits in the lamina densa of glomerular basement membrane by electron microscopy. Pathogenetically, alternate pathway dysfunction leads to this disease, which is diagnosed by ultrastructure. Herein, we describe our observation of C4d positivity in an adolescent boy with DDD