Atypická forma Goodpastureovy nemoci
| Title in English | Atypical form of Goodpasture's disease |
|---|---|
| Authors | |
| Year of publication | 2023 |
| Type | Article in Periodical |
| Magazine / Source | Vnitřní lékařství |
| MU Faculty or unit | |
| Citation | |
| Web | https://casopisvnitrnilekarstvi.cz/artkey/vnl-202305-0007_atypical-form-of-goodpasture-s-disease.php?l=cz |
| Doi | http://dx.doi.org/10.36290/vnl.2023.062 |
| Keywords | anti-GBM nephritis; Goodpasture's disease; immunosuppression; nephritic syndrome; plasmapheresis; rapidly progressive glomerulonephritis |
| Description | 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. |