[Case of HLA-DR8 positive sarcoidosis following polymyositis and Sjögren's syndrome].

A 69-year-old woman had been found to have idiopathic interstitial pneumonia (fibrotic NSIP) in 1997. Proximal muscle weakness appeared in April 2005. Chest CT revealed hilar and mediastinal lymphadenopathy. Polymyositis and Sjögren's syndrome were subsequently diagnosed. We assumed that the interstitial pneumonia had preceded polymyositis and Sjögren's syndrome. A muscle biopsy and transbronchial needle aspiration biopsy demonstrated noncaseating epithelioid cell granulomas. A diagnosis of sarcoidosis complicated with polymyositis and Sjögren's syndrome was made from these findings. Moreover, her HLA genotype contained DR8. HLA-DR8 is considered to be associated with polymyositis, Sjögren's syndrome, and sarcoidosis in Japanese patients. This case suggests the possibility that there are common immunological and genetical pathogenetic mechanisms in autoimmune diseases and sarcoidosis.

[Case of acute interstitial pneumonia that responded to therapy but relapsed six months later].

A 66-year-old man was admitted because of general fatigue. A chest computed tomography showed bilateral alveolar consolidation and ground glass opacities. Although we treated him with broad-spectrum antibiotics, his symptoms and chest image findings did not improve. Thoracoscopic lung biopsy (rS2, S9) was performed. The specimens showed obstructive type intraluminar organization and interstitial inflammatory thickening. Membranous organization was seen in a limited area. The etiology of the illness could not be identified. We diagnosed acute interstitial pneumonia (AIP) because the specimens showed diffuse alveolar damage pattern (DAD/P) and because of unknown etiology. The symptoms and chest image findings were improved on treatment with corticosteroid and cyclophosphamide. However, he was readmitted because of dyspnea 6 months later after the thoracoscopic lung biopsy. Chest computed tomography showed bilateral diffuse ground glass opacities and reticular opacities in both lower lobes. We employed mechanical ventilation, antibiotics, sivelestat sodium hydrate and steroid pulse therapy, but he died without any response to treatment. The findings of autopsy revealed DAD/P accompanied by a new lesion mainly composed of membranous organization and hyaline membrane. We believe this case is valuable when considering the variety of responses to treatment of AIP and prognosis.

[A case of Wegener's granulomatosis associated with the syndrome of inappropriate secretion of ADH].

A 68 year-old woman was admitted with fever, productive cough and sore throat. A chest radiograph and a chest computed tomography showed multiple nodules in both lungs. Thoracoscopic lung biopsy was performed. The specimens showed vasculitis and geographic basophilic necrosis with palisading histiocytes, giant cells, and neutrophils. Wegener's granulomatosis was diagnosed. On the 5th hospital day, the serum sodium level was 128 mEq/l. Since secretion of antidiuretic hormone had continued despite a low plasma osmolarity, we diagnosed the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) and initiated oral prednisolone and cyclophosphamide. As a result, the symptoms and image findings were improved, and serum sodium level became normal. This case was considered to be SIADH secondary to Wegener's granulomatosis.