Goodpasture Syndrome Diagnosed One Year And A Half after the Appearance of the First Symptoms (Case Report)
Journal
Open Access Macedonian Journal of Medical Sciences
ISSN
ISSN 1857-9655 (Print)
Date Issued
2016-12-06
Author(s)
Stojkovikj, Jagoda
Stojkovic, Dragana
Trajkovski, Martin
Jovanovski, Smilko
DOI
10.3889/oamjms.2016.127
Abstract
BACKGROUND:
Goodpasture 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 illnessbegan 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 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 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%.
Keywords: alveolar haemorrhage, glomerulonephritis, immune system, anti-glomerular basement membrane (anti-GBM) antibodies, Goodpasture syndrome
Goodpasture 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 illnessbegan 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 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 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%.
Keywords: alveolar haemorrhage, glomerulonephritis, immune system, anti-glomerular basement membrane (anti-GBM) antibodies, Goodpasture syndrome
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