b shows pathological findings of the lung showing diffuse alveolar hemorrhage. anti-neutrophil cytoplasmic antibody. We examined six similar instances reported in the literature and concluded that Goodpastures syndrome with pre-existing interstitial pneumonia and myeloperoxidase anti-neutrophil cytoplasmic antibody is related to a poor prognosis. display chest radiograph findings of pulmonary infiltrates and ground-glass shadows in both lung fields. b show chest computed tomography findings of pulmonary infiltrates with ground-glass shadows. display honeycomb shadows in both lung bases Pathological findings at autopsy showed crescentic glomerulonephritis accompanied by fibrinous necrosis and an infiltration of inflammatory cells in the kidney (Fig.?2a). Lung pathology showed hemosiderin-laden macrophages in the alveolar spaces, fibrinoid necrosis of the alveolar and capillary walls, infiltration of neutrophils in the alveolar walls, and formation of hyaline membranes (Fig.?2b, c). Moreover, fibrotic hyperplasia of the alveolar walls and fibroblastic foci were seen in the lung bases (Fig.?2d). We diagnosed the cause of death as respiratory failure due to diffuse alveolar damage induced by alveolar hemorrhage and exacerbation of chronic interstitial pneumonia. Open in a separate windows Fig. 2 Pathological findings at autopsy. a show pathological findings in the kidney of crescentic glomerulonephritis. display fibrinous necrosis and infiltration of inflammatory cells (hematoxylin and eosin, 400). b shows pathological findings of the lung showing diffuse alveolar hemorrhage. c shows hyaline membrane in the alveolar wall. d shows fibrotic hyperplasia of the alveolar walls and fibroblastic foci (hematoxylin and eosin, 400) Conversation GPS is definitely a lung-specific and kidney-specific autoimmune disease that is related to anti-GBM antibodies [8]. Clinical manifestations include acute renal failure caused by RPGN and acute respiratory failure caused by DAH [9]. GPS complicated with pre-existing chronic interstitial pneumonia, as seen in our case, is certainly rare. To the best of our knowledge, six similar instances complicated with pre-existing interstitial pneumonia have been reported [10C12]. We examined BG45 the six instances along with the present case (Table?1). Six of the seven individuals (85.7%) were men, and the duration from analysis of interstitial pneumonia to the onset of GPS ranged from 2 weeks to 9 years. All of these individuals were treated with corticosteroids and hemodialysis. In addition, four were treated with plasma exchange, one with cyclophosphamide, and three required mechanical air flow. The 1-12 months survival rate of GPS is definitely reported to be between 75% and 90% [1]. Furthermore, that of GPS with positive MPO-ANCA is definitely reported to be 76% [13]. Remarkably, the 1-12 months survival rate of our examined cases of GPS with pre-existing interstitial pneumonia and positive MPO-ANCA was BG45 29%, and the cause of death in those instances was respiratory failure. DAH has been reported like a result in that exacerbates chronic interstitial pneumonia [14, 15]. In the present case, lung pathological findings showed diffuse alveolar damage that may have been induced by a combination of DAH and exacerbation of interstitial pneumonia. These findings suggest that exacerbation of pre-existing interstitial pneumonia is definitely a poor prognostic element for GPS and is related to crucial respiratory failure in the acute phase. Table 1 Characteristics of individuals with Goodpastures syndrome complicated with pre-existing interstitial pneumonia crescentic glomerulonephritis, corticosteroid, cyclophosphamide, diffuse alveolar hemorrhage, female, Goodpastures syndrome, hemodialysis, interstitial pneumonia, male, myeloperoxidase anti-neutrophil cytoplasmic antibody, mechanical ventilation, non-specific interstitial pneumonia, plasma exchange, typical interstitial pneumonia The pathogenesis of GPS is not fully recognized. The prospective GBM antigen molecule was consequently identified as the BG45 noncollagenous-1 BG45 (NC1) website of the 3 chain of collagen IV [16]. In addition, environmental factors will also be thought Mmp28 to boost the risk of the disease, for instance, respiratory illness by influenza computer virus, exposure to hydrocarbon fumes or metallic dust, and tobacco smoking [2]. Of interest, almost all of the seven instances we reviewed were positive.