Rapidly Progressive Respiratory Failure in Mixed Connective Tissue Disease: Report of an Autopsy Case
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- Watanabe Yasutaka
- Division of Pulmonary Medicine, Jichi Medical University, Saitama Medical Center, Japan
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- Koyama Shinichiro
- Division of Pulmonary Medicine, Jichi Medical University, Saitama Medical Center, Japan
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- Moriguchi Masato
- Division of Reumatology, Jichi Medical University, Saitama Medical Center, Japan
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- Miwa Chihiro
- Division of Pulmonary Medicine, Jichi Medical University, Saitama Medical Center, Japan
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- Shiraishi Mamoru
- Division of Pulmonary Medicine, Jichi Medical University, Saitama Medical Center, Japan
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- Nomura Motoko
- Division of Pulmonary Medicine, Jichi Medical University, Saitama Medical Center, Japan
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- Nokubi Mitsuhiro
- Division of Pathology, Jichi Medical University, Saitama Medical Center, Japan
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- Terai Chihiro
- Division of Reumatology, Jichi Medical University, Saitama Medical Center, Japan
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- Kawabata Yoshinori
- Division of Pathology, Saitama Cardiovascular and Respiratory Center, Japan
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抄録
A 64-year-old woman presented with exertional dyspnea. The case was diagnosed as mixed connective tissue disease (MCTD) due to presence of swollen fingers, Raynaud's phenomenon, muscle weakness, positive anti-U1RNP antibody, pericarditis and interstitial pneumonia. Although the histology from a transbronchial lung biopsy (TBLB) indicated organizing pneumonia, corticosteroid therapy was postponed for two months at the patient's request. She died 8 weeks later from acute progressive interstitial pneumonia in spite of the administration of intravenous cyclophosphamide combined with prednisolone. The autopsy revealed exudative and organizing diffuse alveolar damage (DAD). Previous reports have shown that DAD is an extremely rare pulmonary complication in MCTD. This report presents a case of MCTD with acute respiratory failure. This case thus suggests that this therapy should be administered as soon as possible.<br>
収録刊行物
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- Internal Medicine
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Internal Medicine 51 (24), 3415-3419, 2012
一般社団法人 日本内科学会