title: Rapidly progressive respiratory failure in mixed connective tissue disease: report of an autopsy case.
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.
authors.affiliation: Division of Pulmonary Medicine, Jichi Medical University, Saitama Medical Center, Japan. yasuyasu@omiya.jichi.ac.jp
authors.forename: Yasutaka
authors.initials: Y
authors.lastname: Watanabe
authors.forename: Shinichiro
authors.initials: S
authors.lastname: Koyama
authors.forename: Masato
authors.initials: M
authors.lastname: Moriguchi
authors.forename: Chihiro
authors.initials: C
authors.lastname: Miwa
authors.forename: Mamoru
authors.initials: M
authors.lastname: Shiraishi
authors.forename: Motoko
authors.initials: M
authors.lastname: Nomura
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Nokubi
authors.forename: Chihiro
authors.initials: C
authors.lastname: Terai
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Kawabata
mesh_terms: D001344:Autopsy; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008875:Middle Aged; D008947:Mixed Connective Tissue Disease / Q000150:complications*; D012131:Respiratory Insufficiency / Q000209:etiology*; D013997:Time Factors