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

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%.

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Anti-gbm Rapidly Progressive Glomerulonephritis (Syndroma Goodpasture): A Case Report

Serbian Journal of Experimental and Clinical Research ◽

10.2478/sjecr-2014-0021 ◽

2014 ◽

Vol 15 (3) ◽

pp. 157-159

Author(s):

Andreja Figurek ◽

Vlastimir Vlatkovic ◽

Dragan Vojvodic ◽

Milorad Grujicic

Keyword(s):

Renal Function ◽

Rapidly Progressive Glomerulonephritis ◽

Current Therapy ◽

Severe Illness ◽

Term Survival ◽

End Stage Renal Failure ◽

Goodpasture Syndrome ◽

Preserve Renal Function ◽

End Stage

Abstract 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%. Before, the mortality was higher than 90%. In our patient, the disease began as dysuria, continued as anaemic syndrome, and ended with the development of end-stage renal failure. Immunosuppressive therapy with pulse doses of methylprednisolone and cyclophosphamide has put the disease into remission, but the permanent impairment of renal function remained. Early diagnosis of Goodpasture syndrome helps preserve renal function and improves patients’ survival. In patients who achieve remission, a kidney transplant can be considered. Currently, our patient is awaiting transplantation

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Diffuse alveolar haemorrhage

Oxford Textbook of Medicine ◽

10.1093/med/9780198746690.003.0421 ◽

2020 ◽

pp. 4235-4238

Author(s):

S. J. Bourke ◽

G.P. Spickett

Keyword(s):

Renal Involvement ◽

Connective Tissue Diseases ◽

Autoimmune Disorder ◽

Alveolar Haemorrhage ◽

Diffuse Alveolar Haemorrhage ◽

Coagulation Disorders ◽

Goodpasture's Syndrome ◽

Goodpasture’S Syndrome ◽

Immune Mediated ◽

Dominant Feature

Diffuse alveolar haemorrhage is characterized by acute respiratory failure, diffuse air space shadowing on the chest radiograph, haemoptysis, and anaemia. There are many different causes including immune-mediated diseases (notably pulmonary vasculitis, connective tissue diseases, and Goodpasture’s syndrome) and non-immune-mediated disease (cardiac failure, infection, coagulation disorders, thrombolytic therapy, toxins, and barotrauma). Prompt identification of the underlying cause is important in directing specific treatments. Goodpasture’s syndrome is an autoimmune disorder characterized by alveolar haemorrhage and glomerulonephritis due to antibasement membrane antibodies. Renal failure is usually the dominant feature, but alveolar haemorrhage can precede renal involvement. Idiopathic pulmonary haemosiderosis is a rare disorder of unknown cause with recurrent alveolar bleeding, which may provoke pulmonary fibrosis, and anaemia.

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