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

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 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 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%.

Clinic morphological features of Goodpasture’s syndrome manifested with respiratory disorders

“…According to the data of different studies, the use of pathogenetic treatment in the absence of signs of respiratory and renal failure leads to the survival of 86.9 % of patients within one year; otherwise, the vast majority of patients die within several weeks or months after diagnosis [12,13]. Patient survival rates improved significantly after 2007, which are associated with the timely use of immunosuppressive therapy and plasmapheresis, however, dependence on hemodialysis, a decrease in the number of normal functioning nephrons and widened infiltrating changes in interstitial tissue considerably wor sen the prognosis of patients [14].…”

“…The current treatment of this syndrome includes the immunosuppressive therapy using pulse therapy with systemic corticosteroids and cyclophosphamide in combination with plasmapheresis [10][11][12].…”

“…Interaction between them is provided by molecular mediators, cytokines. Important role in the development of immune inflammation is played by metabolites of arachidonic acid, free oxygen radicals, proteolytic enzymes, adhesion molecules [1,7,11,12].…”

“…The timely recognition of anti-GBM antibodiesdisease, when the function of the kidneys and lungs is still preserved, is the key to the effectiveness of modern pathogenetic treatment, which allows recovery in the vast majority of patients. Otherwise, the fulminant development of respiratory and renal failure leads to a lethal outcome [7,11,12].…”

Goodpasture's syndrome in early pregnancy: A case report

“…Combination therapy is recommended for the initial management of the disease, including corticosteroid therapy, cytostatic therapy and plasmapheresis. However, patients have poor prognosis with current treatments and the mortality rate has been estimated as 11% (4). The etiology of Goodpasture's Syndrome remains unknown; however, the development of the disease following environmental exposures including hydrocarbon fumes, metallic dust or tobacco smoke has been reported (5).…”