Vnitr Lek 2023, 69(5):316-321 | DOI: 10.36290/vnl.2023.062

Atypical form of Goodpasture's disease

Michaela Habánová^{1, 4}, Petra Divácká^{2, 4}, Jitka Řehořová^{2, 4}, Iva Svobodová^{3, 4}

¹ Interní hematologická a onkologická klinika, Fakultní nemocnice Brno

² Interní gastroenterologická klinika, Fakultní nemocnice Brno

³ I. ústav patologie, Fakultní nemocnice u sv. Anny, Brno

⁴ Lékařská fakulta, Masarykova univerzita, Brno

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: anti­‑GBM nephritis, Goodpasture's disease, immunosuppression, nephritic syndrome, plasmapheresis, rapidly progressive glomerulonephritis.

Accepted: August 24, 2023; Published: September 12, 2023  Show citation