Pre-existing chronic interstitial pneumonia is a poor prognostic factor of Goodpasture's syndrome: a case report and review of the literature

Abstract

Background: Goodpasture's syndrome is a rare disease that is characterized by rapidly progressive glomerulonephritis and diffuse alveolar hemorrhage.

Case presentation: A 71-year-old Japanese man who had chronic interstitial pneumonia was diagnosed as having Goodpasture's syndrome. Both anti-glomerular basement membrane antibody and myeloperoxidase anti-neutrophil cytoplasmic antibody were increased. Despite intensive treatments, including mechanical ventilation, he died from respiratory failure. Pathological findings at autopsy showed rapidly progressive glomerulonephritis in his kidneys, diffuse alveolar hemorrhage, hyaline membranes, and fibroblastic foci in his lungs. The cause of death was diagnosed as respiratory failure as a result of diffuse alveolar damage induced by a combination of diffuse alveolar hemorrhage and exacerbation of interstitial pneumonia.

Conclusions: We report a case of Goodpasture's syndrome complicated with pre-existing chronic interstitial pneumonia and positive myeloperoxidase anti-neutrophil cytoplasmic antibody. We reviewed six similar cases reported in the literature and concluded that Goodpasture's syndrome with pre-existing interstitial pneumonia and myeloperoxidase anti-neutrophil cytoplasmic antibody is related to a poor prognosis.

Keywords: Anti-glomerular basement membrane antibody; Goodpasture’s syndrome; Interstitial pneumonia; Myeloperoxidase anti-neutrophil cytoplasmic antibody.

Fig. 1

Findings of chest radiograph and computed tomography at admission. a Arrows show chest radiograph findings of pulmonary infiltrates and ground-glass shadows in both lung fields. b Arrows show chest computed tomography findings of pulmonary infiltrates with ground-glass shadows. Arrowheads show honeycomb shadows in both lung bases

Fig. 2

Pathological findings at autopsy. a Arrows show pathological findings in the kidney of crescentic glomerulonephritis. Arrowheads show fibrinous necrosis and infiltration of inflammatory cells (hematoxylin and eosin, ×400). b Arrow shows pathological findings of the lung showing diffuse alveolar hemorrhage. c Arrows shows hyaline membrane in the alveolar wall. d Arrow shows fibrotic hyperplasia of the alveolar walls and fibroblastic foci (hematoxylin and eosin, ×400)