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

Pathology International
M WakamatsuY Tsutsumi

Abstract

A case of mixed connective tissue disease (MCTD) in a female with positive ribonucleoprotein antibody and overlapped manifestations of systemic lupus erythematosus and dermatomyositis 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 ulceration 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.

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Citations

Oct 10, 2006·Modern Rheumatology·Yoshiko YamaguchiYoshiaki Ishigatsubo
Aug 16, 2006·The American Journal of the Medical Sciences·Manabu HamadaMasutaka Furue
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