Atypical form of Goodpasture’s disease

Česká verzia

Authors: Michaela Habánová ^1,4; Petra Divácká ^2,4; Jitka Řehořová ^2,4; Iva Svobodová ^3,4
Authors place of work: Interní hematologická a onkologická klinika, Fakultní nemocnice Brno ¹; Interní gastroenterologická klinika, Fakultní nemocnice Brno ²; I. ústav patologie, Fakultní nemocnice u sv. Anny, Brno ³; Lékařská fakulta, Masarykova univerzita, Brno ⁴
Published in the journal: Vnitř Lék 2023; 69(5): 316-321
Category: Case Reports
doi: https://doi.org/10.36290/vnl.2023.062

Summary

Goodpasture’s disease and anti-glomerular basement membrane nephritis (anti-GBM nephritis) are rare autoimmune small vessel vasculitis predominantly affecting young men. Goodpasture’s disease plays an important part in differential diagnosis of pulmonary – renal syndrome. The evidence of circulating autoantibodies, a typical histological appearance of the kidney biopsy with finding of the crescent glomerulonephritis and clinical presentation of nephritic syndrome play an important role in diagnosis. Our case report describes a case of a young male with anti-GBM nephritis that presents as rapidly progressive glomerulonephritis (RPGN) with progression to dialysis-dependent renal failure. The atypical sign of the case was development of nephrotic syndrome with volume-dependent hypertension. The case was complicated by heparin-induced thrombocytopenia. During combined immunosuppressive therapy with plasmapheresis, the condition of the patient has stabilized. However, renal function did not recover. This previously fatal disease has nowadays a very good survival rate because of immunosuppresion therapy, plasmapheresis and hemodialysis.

Keywords:

plasmapheresis – immunosuppression – anti-GBM nephritis – Goodpasture’s disease – nephritic syndrome – rapidly progressive glomerulonephritis

Zdroje

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