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Year : 2018  |  Volume : 1  |  Issue : 1  |  Page : 29-32

Reversal of delayed pulmonary hemorrhage with plasmapheresis in antiglomerular basement membrane disease

Department of Pediatric Nephrology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan

Correspondence Address:
Vina Tresa
Department of Pediatric Nephrology, Sindh Institute of Urology and Transplantation, Dewan Farooq Medical Complex, Deen Mohammed Wafai Road, Karachi 74800
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/AJPN.AJPN_3_18

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Antiglomerular basement membrane (GBM) disease, caused by IgG antibodies directed against type IV collagen in the GBM, may present with rapidly progressive glomerulonephritis and/or diffuse alveolar hemorrhage. While rare in childhood, pediatric patients usually have both renal and pulmonary involvement. We report a child who presented with crescentic glomerulonephritis and severe renal-limited anti-GBM disease. The diagnosis was aided by findings of characteristic linear IgG deposits on GBM on immunofluorescence on kidney biopsy and high titers of anti-GBM antibody. As patients with renal-limited disease and severe renal failure usually do not recover renal function despite aggressive immunosuppression and plasmapheresis, intensive therapy was withdrawn while continuing maintenance hemodialysis. However, the patient later developed severe alveolar hemorrhage that responded completely to plasmapheresis and intensification of immunosuppression. Sustained serological and pulmonary response was documented, even though renal dysfunction persisted. Our case emphasizes the utility of kidney biopsy and serology in enabling rare diagnosis in patients with rapidly progressive glomerulonephritis and the role of aggressive plasmapheresis in enabling recovery from delayed catastrophic alveolar hemorrhage.

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