Goodpasture Syndrome Diagnosed One Year And A Half after the Appearance of the First Symptoms (Case Report)

Authors

  • Jagoda Stojkovikj University Clinic of Pulmonology and Allergology, Medical Faculty, Ss Cyril and Methodius University of Skopje, Skopje
  • Sead Zejnel University Clinic of Pulmonology and Allergology, Medical Faculty, Ss Cyril and Methodius University of Skopje, Skopje
  • Biljana Gerasimovska University Clinic of Nephrology, Medical Faculty, Ss Cyril and Methodius University of Skopje, Skopje
  • Vesna Gerasimovska University Clinic of Nephrology, Medical Faculty, Ss Cyril and Methodius University of Skopje, Skopje
  • Dragana Stojkovic Primary Health Care “D-r Dijana Jedikovska”, Skopje
  • Martin Trajkovski Primary Health Care “St. Elena”, Skopje
  • Irina Angelovska University Clinic of Pulmonology and Allergology, Medical Faculty, Ss Cyril and Methodius University of Skopje, Skopje
  • Angela Debreslioska University Clinic of Pulmonology and Allergology, Medical Faculty, Ss Cyril and Methodius University of Skopje, Skopje
  • Smilko Jovanovski University Clinic of Pulmonology and Allergology, Medical Faculty, Ss Cyril and Methodius University of Skopje, Skopje

DOI:

https://doi.org/10.3889/oamjms.2016.127

Keywords:

alveolar haemorrhage, glomerulonephritis, immune system, anti-glomerular basement membrane (anti-GBM) antibodies, Goodpasture's syndrome

Abstract

BACKGROUND: Goodpasture's syndrome was originally described as an association of alveolar haemorrhage and glomerulonephritis. It occurs when the immune system attacks and destroys healthy body tissue.

AIM: We are presenting a patient with a clinical picture of pulmonary haemorrhage and glomerulonephritis, which is diagnosed by renal biopsy.

CASE PRESENTATION: His illness began a year and a half before being diagnosed. In that period he had occasional exacerbations. He was received at our Clinic in extremely serious condition, and after stabilisation of his medical condition, there was made a biopsy of the kidney. The p-ANCA was 8.93 U/ml (neg < 3, poz > 5 U/ml). Histopathological diagnosis of biopsy of the kidney was: Glomerulonephritis extra capillaries focalis, segmentalis et globalis. Based on this he was diagnosed with Goodpasture's syndrome. He received corticosteroid therapy and cyclophosphamide, with good response to treatment, and he is currently in a stable condition, receiving only corticosteroid therapy.

CONCLUSION: Goodpasture’s syndrome is a severe illness caused by the formation of antibodies to the glomerular basement membrane and alveolus with consequential damage to renal and pulmonary function. With current therapy, long-term survival is more than 50%.

Downloads

Download data is not yet available.

Metrics

Metrics Loading ...

Plum Analytics Artifact Widget Block

References

Castro C, Gourley M. Diagnostic Testing and Interpretation of Tests for Autoimmunity. J Allergy Clin Immunol. 2010; 125(2): 238–S247. https://doi.org/10.1016/j.jaci.2009.09.041 PMid:20061009 PMCid:PMC2832720 DOI: https://doi.org/10.1016/j.jaci.2009.09.041

Chan P, Leung M. Sequential occurrence of anti-glomerular basement membrane disease 9 years after anti-neutrophil cytoplasmic antibody-associated vasculitis. Oxf Med Case Reports. 2016;(4):91–93. https://doi.org/10.1093/omcr/omw026 PMid:27123311 PMCid:PMC4845091 DOI: https://doi.org/10.1093/omcr/omw026

Srivastava G, Rao P, Segal M, Geetha Sh. Characteristics and outcome of crescentic glomerulonephritis in patients with both antineutrophil cytoplasmic antibody and anti-glomerular basement membrane antibody. Clinical Rheumatology. 2013; 2 (9): 1317–1322. https://doi.org/10.1007/s10067-013-2268-5 PMid:23624587 DOI: https://doi.org/10.1007/s10067-013-2268-5

Olson SW, Arbogast CB, Baker TP, Owshalimpur D, Oliver DK, Abbott KC, Yuan CM. Asymptomatic autoantibodies associate with future anti-glomerular basement membrane disease. J Am Soc Nephrol. 2011;22:1946–1952. https://doi.org/10.1681/ASN.2010090928 PMid:21868497 PMCid:PMC3279953 DOI: https://doi.org/10.1681/ASN.2010090928

Parekh N, Epstein E, El-Sayegh S. Necrotizing RPGN with linear anti IgG deposits in a patient with history of granulomatosis with polyangiitis: a case report. International Journal of Nephrology and Renovascular Disease. 2014;(7): 441—446. https://doi.org/10.2147/IJNRD.S61621 PMid:25473306 PMCid:PMC4251529 DOI: https://doi.org/10.2147/IJNRD.S61621

Spyros A, Manali ED, Kalomenidis I, Kapotsis GE, Karakatsani A, Roussos C. Bench-to-bedside review: Pulmonary–renal syndromes – an update for the intensivist. Critical Care. 2007;11:213. https://doi.org/10.1186/cc5778 PMid:17493292 PMCid:PMC2206392 DOI: https://doi.org/10.1186/cc5778

Swainson C, Robson J, Urbaniak S, Keller A, Kay A. Treatment of Goodpasture's disease by plasma exchange and immunosuppression. Clin Exp Immunol.1978; 32(2): 233–242. PMid:668199 PMCid:PMC1541272

Collins RD. Dr Goodpasture: "I was not aware of such a connection between lung and kidney disease". Ann Diagn Pathol. 2010;14(3):194-8. https://doi.org/10.1016/j.anndiagpath.2010.02.003 PMid:20471565 DOI: https://doi.org/10.1016/j.anndiagpath.2010.02.003

Greenspon SA, Krakower CA. Direct evidence for the antigenicity of the glomeruli in the production of nephrotoxic serums. AMA Arch Pathol. 1950;49(3):291-7. PMid:15406262

Lerner RA, Glassock RJ, Dixon FJ. The role of anti-glomerular basement membrane antibody in the pathogenesis of human glomerulonephritis. J Exp Med. 1967;126(6):989-1004. https://doi.org/10.1084/jem.126.6.989 PMid:4964566 PMCid:PMC2138413 DOI: https://doi.org/10.1084/jem.126.6.989

Mazidi P, Bajestani S, Khan M, Khair T, Laos L. Goodpasture's Syndrome: A Case Report And Review Of Literature. The Internet Journal of Internal Medicine. 2001;7: 2.

Kalluri R, Wilson CB, Weber M, Gunwar S, Chonko AM, Neilson EG, Hudson BG. Identification of the alpha 3 chain of type IV collagen as the common autoantigen in antibasement membrane disease and Goodpasture syndrome. J Am Soc Nephrol. 1995;(4):1178-85. PMid:8589284 DOI: https://doi.org/10.1681/ASN.V641178

Shiferaw B, at all. Goodpasture's Disease: An Uncommon Disease With an Atypical Clinical Course. J Clin Med Res. 2016; 8(1): 52–55. https://doi.org/10.14740/jocmr2379w PMid:26668684 PMCid:PMC4676347 DOI: https://doi.org/10.14740/jocmr2379w

Molecular characteristics of the Goodpasture autoantigen.Hudson BG, Kalluri R, Gunwar S, Noelken ME, Mariyama M, Reeders ST. Kidney Int. 1993;43(1):135-9. PMid:7679455 DOI: https://doi.org/10.1038/ki.1993.22

Vucković B, Ilić T, Mitić I, Knezević V, Vodopivec S, Curić S. Med Pregl.[Goodpasture's syndrome--case report]. Med Pregl. 2004; 57(7-8):391-5. PMid:15626299 DOI: https://doi.org/10.2298/MPNS0408391V

Sinha V, Hibbert C. Near-lethal acute kidney injury due to Goodpasture's syndrome: A case report. Journal of the Intensive Care Society. 2015; 0(0) 1–5. https://doi.org/10.1177/1751143715593560 DOI: https://doi.org/10.1177/1751143715593560

Salam N, Rezki H, Fadili W, Hachim K, Ramdani B. Goodpasture's syndrome - Four Case Reports. Saudi J Kidney Dis Transpl (serial online). 2007;18:235-8. PMid:17496401

Rossert J. Goodpasture's disease. Orphanet encyclopedia; 2002. http://www.orpha.net/data/patho/GB/uk-goodpasture.pdf

Figurek A, Vlatkovic V, Vojvodic D, Grujic M. Anti-GBM rapidly progressive glomerulonephritis (Syndroma Goodpasture): A case report. Serbian Journal of Experimental and Clinical Research. 2014;15(3):157-159. https://doi.org/10.5937/sjecr1403157F DOI: https://doi.org/10.2478/sjecr-2014-0021

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. https://doi.org/10.7326/0003-4819-134-11-200106050-00009 PMid:11388816 DOI: https://doi.org/10.7326/0003-4819-134-11-200106050-00009

Published

2016-12-06

How to Cite

1.
Stojkovikj J, Zejnel S, Gerasimovska B, Gerasimovska V, Stojkovic D, Trajkovski M, Angelovska I, Debreslioska A, Jovanovski S. Goodpasture Syndrome Diagnosed One Year And A Half after the Appearance of the First Symptoms (Case Report). Open Access Maced J Med Sci [Internet]. 2016 Dec. 6 [cited 2024 Apr. 26];4(4):683-7. Available from: https://oamjms.eu/index.php/mjms/article/view/oamjms.2016.127

Issue

Section

C- Case Reports

Most read articles by the same author(s)