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Do you recommend initiating immunosuppression and plasmapheresis in patients with dialysis dependent AKI in the setting of anti-GBM disease who do not have pulmonary involvement?

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Do you recommend initiating immunosuppression and plasmapheresis in patients with dialysis-dependent AKI due to anti-GBM disease without pulmonary involvement?

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1 Answer
Mednet MemberInvited Expert
Nephrology · UAB Spain Rehabilitation Center
Answered on

Anti-GBM disease is a rare disorder (incidence perhaps 1:1,000,000 adults/year) that is characterized as a small vessel vasculitis mediated by anti-GBM antibodies directed against the alpha-3-chain of collagen IV in basement membranes. Perhaps half of patients have disease that involves both the kidney and lung (also termed Goodpasture syndrome). As a rare disorder, randomized trials are difficult, leaving the clinician with management decisions based largely on retrospective studies and clinical manifestations.

Unlike other forms of rapidly progressive glomerulonephritis (RPGN), pathology of anti-GBM disease usually shows global glomerular involvement with crescents, suggesting that kidney recovery with treatment is challenging especially...

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