Rapidly Progressive Respiratory Failure in Mixed Connective Tissue Disease: Report of an Autopsy Case

Yasutaka Watanabe; Shinichiro Koyama; Masato Moriguchi; Chihiro Miwa; Mamoru Shiraishi; Motoko Nomura; Mitsuhiro Nokubi; Chihiro Terai; Yoshinori Kawabata

doi:10.2169/internalmedicine.51.8728

CASE REPORTS

Rapidly Progressive Respiratory Failure in Mixed Connective Tissue Disease: Report of an Autopsy Case

Yasutaka Watanabe, Shinichiro Koyama, Masato Moriguchi, Chihiro Miwa, Mamoru Shiraishi, Motoko Nomura, Mitsuhiro Nokubi, Chihiro Terai, Yoshinori Kawabata

Author information

Yasutaka Watanabe
Division of Pulmonary Medicine, Jichi Medical University, Saitama Medical Center, Japan
Shinichiro Koyama
Division of Pulmonary Medicine, Jichi Medical University, Saitama Medical Center, Japan
Masato Moriguchi
Division of Reumatology, Jichi Medical University, Saitama Medical Center, Japan
Chihiro Miwa
Division of Pulmonary Medicine, Jichi Medical University, Saitama Medical Center, Japan
Mamoru Shiraishi
Division of Pulmonary Medicine, Jichi Medical University, Saitama Medical Center, Japan
Motoko Nomura
Division of Pulmonary Medicine, Jichi Medical University, Saitama Medical Center, Japan
Mitsuhiro Nokubi
Division of Pathology, Jichi Medical University, Saitama Medical Center, Japan
Chihiro Terai
Division of Reumatology, Jichi Medical University, Saitama Medical Center, Japan
Yoshinori Kawabata
Division of Pathology, Saitama Cardiovascular and Respiratory Center, Japan

Keywords: mixed connective tissue disease, diffuse alveolar damage, interstitial pneumonia, cytomegalovirus

JOURNAL OPEN ACCESS

2012 Volume 51 Issue 24 Pages 3415-3419

DOI https://doi.org/10.2169/internalmedicine.51.8728

Details

Abstract

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.

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