Mixed connective tissue disease complicated by pneumatosis cystoides intestinalis and malabsorption syndrome: Case report and literature review

Manami Wakamatsu, Ken‐ichi ‐i Inada, Yutaka Tsutsumi

Research output: Contribution to journalArticlepeer-review

17 Citations (Scopus)

Abstract

A case of mixed connective tissue disease (MCTD) in a female with positive ribonucleoprotein antibody and overlapped manifestations of systemic lupus erythematosus and der‐matomyositis is presented. During the last 9 years of her 13 year clinical course, she repeatedly manifested abdominal distension, pneumoperitoneum and malabsorption syndrome. She died, aged 54 years, of respiratory failure secondary to pulmonary compression from marked abdominal distention. Autopsy revealed esophageal fibrosis with ulcer‐ation and the typical appearance of pneumatosis cystoides intestinalis (PCI), in association with atrophy and fibrosis of the inner circular muscle layer of the small bowel. The association of PCI with MCTD is reviewed briefly.

Original languageEnglish
Pages (from-to)875-878
Number of pages4
JournalPathology International
Volume45
Issue number11
DOIs
Publication statusPublished - 11-1995
Externally publishedYes

All Science Journal Classification (ASJC) codes

  • Pathology and Forensic Medicine

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