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Nephrology


Answer 1
  1. Plasma exchange, prednisone, and cyclophosphamide. This patient has Goodpasture’s syndrome, also known as anti-GBM antibody disease, a syndrome of glomerulonephritis, pulmonary hemorrhage, and circulating anti-GBM antibodies. Goodpasture’s syndrome is 1 of the 3 major forms of rapidly progressive (crescentic) glomerulonephritis. Patients usually present with acute kidney injury, and urinalysis reveals non-nephrotic range proteinuria with nephritic sediment. Pulmonary involvement is present in 70% of cases.1 The presence of anti-GBM antibodies and linear IgG staining of the basement membrane, as seen in this patient, define the disorder. The treatment of choice for Goodpasture’s syndrome is plasmapheresis with concurrent prednisone and cyclophosphamide. Plasmapheresis removes circulating anti-GBM antibodies and other mediators of inflammation, while immunosuppressive therapy reduces new antibody production. Duration of treatment is determined by attainment of clinical remission and disappearance of anti-GBM antibodies.1 IV prednisolone alone would not sufficiently treat this patient, and he has no indication for dialysis. Observation without treatment will lead to rapid progression to end-stage renal disease and death.

    REFERENCE
    1.
     Levy JB, Turner AN, Rees AJ, Pusey CD. Long-term outcome of anti-glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression. Ann Intern Med 2001;134:1033–42.

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