MIXED CONNECTIVE-TISSUE DISEASE COMPLICATED BY PNEUMATOSIS-CYSTOIDES-INTESTINALIS AND MALABSORPTION-SYNDROME - CASE-REPORT AND LITERATURE-REVIEW

A case of mixed connective tissue disease (MCTD) in a female with posi tive ribonucleoprotein antibody and overlapped manifestations of syste mic lupus erythematosus and dermatomyositis is presented. During the l ast 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 co mpression from marked abdominal distention, Autopsy revealed esophagea l fibrosis with ulceration and the typical appearance of pneumatosis c ystoides intestinalis (PCI), in association with atrophy and fibrosis of the inner circular muscle layer of the small bower. The association of PCI with MCTD is reviewed briefly.