ANTI – GBM RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS (SYNDROMA GOODPASTURE) – CASE REPORT

  • Andreja M Figurek Clinical Centre Banja Luka, Clinic for internal diseases, Banja Luka, Republic of Srpska

Sažetak


ABSTRACT

Introduction. Goodpasture's syndrome is a severe illness caused by the formation of antibodies to the glomerular basement membrane and alveolus with consequential damages of renal and pulmonary function. With current therapy, long term survival is greater than 50%, and before it the mortality due to this disease was higher than 90%. Case report. In our patient, disease has began as dysuria, then continued as anemic syndrome, and at the end the end stage renal failure developed. Immunosuppressive therapy with pulse doses Methylprednisolone and Cyclophosphamide, has introduced this patient in the disease remission, but the permanent impairment of renal function still remained. Conclusion. Early diagnosis of Goodpasture's syndrom allows to preserve renal function and has a positive effect on the survival of patients. In patients who achieve remission of the disease, a kidney transplant can be considered. According to this, our patient is in processing for transplantation.

Key words: Syndroma Goodpasture; collagen α3 (IV) chain; anti-GBM antibodies; crescentic forms.

Reference

Rossert J. Goodpasture's disease. Orphanet encyclopedia; 2002.

Fisher EG, Lager DJ. Anti-glomerular basement membrane glomerulonephritis: A morphologic study of 80 cases. Am J Pathol 2006; 125: 445-50.

Kalluri R, Gattone II VH, Nollken ME et al. The α3 chain of type IV collagen induces autoimmune Goodpasture sy. Proc. Natl.Acad. Sci USA. 1994;91:6201-5.

Persson U, Hertz JM, Carlsson M et al. Patients with Goodpasture's disease have two normal COL4A3 alleles encoding the NC1 domain of the type IV collagen α3 chain. Nephrol Dial Transplant 2004; 19: 2030-5.

Panjwani AH, Deoskar RB, Falleiro JJJ et al. Goodpasture Syndrom. MJAFI 2003; 59: 77-9.

Rowe PA, Mansfield DC, Dutton GN. Ophthalmic features of fourteen cases of Goodpasture sy. Nephron 1994; 68: 52-6.

Segelmark M, Hellmark T, Wieslander J. The Prognostic Significance in Goodpasture’s Disease of Specificity, Titre and Affinity of Anti-Glomerular-Basement-Membrane Antibodies. Nephron Clin Pract 2003; 94: 59-68.

Briggs WA, Johnson JP, Teichman S, et al. Antiglomerular basement membrane antibody-mediated glomerulonephritis and Goodpasture's syndrome. Medicine (Baltimore) 1979 Sep; 58 (5): 348-61.

Lockwood CM, Pearson TA, Rees AJ, et al. Immunosuppression and plasma-exchange in the treatment of Goodpasture's syndrome. The Lancet 1976; 307(7942):711-5.

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

Vučković B, Ilić T, Mitić I, et al. Goodpastureov sindrom – prikaz slučaja. Medicinski pregled 2004; 57(7-8):391–5.

Objavljeno
2014/09/26
Rubrika
Prikaz slučaja