Fatal Multisystem Disease with Immune Enteropathy Heralded by Juvenile Rheumatoid Arthritis

• Published in: Journal of pediatric gastroenterology and nutrition Vol. 8; no. 2; pp. 259 - 265
• Main Authors: Pearson, Richard D, Swenson, Ida, Schenk, Eric A, Klish, William J, Brown, Marilyn R
• Format: Journal Article
• Language: English
• Published: Hagerstown, MD Lippincott-Raven Publishers 01.02.1989; Lippincott
• Subjects: Arthritis, Juvenile - complications; Autoimmune Diseases - etiology; Biological and medical sciences; Diseases of the osteoarticular system; Duodenum - pathology; Enteritis - etiology; Female; Humans; Infant; Inflammatory joint diseases; Liver Diseases - etiology; Medical sciences; Skin Diseases - etiology; Human; Immunoglobulins; Mortality; Gut; Diseases of the osteoarticular system; Autoimmune disease; Complement; Inflammatory joint disease; Case study; Pathology; Chronic; Polyarthritis; Digestive diseases; Enteritis; Child
• ISSN: 0277-2116; 1536-4801
• DOI: 10.1097/00005176-198902000-00022

The prognosis of juvenile rheumatoid arthritis (JRA) is generally good, although premature death occurs in a subset of children. Secondary infections, chronic amyloidosis, and heart disease have been reported as common causes. Our experience indicates that JRA can also herald the development of a severe immune enteropathy. In the case presented, typical JRA was followed by fulminant hepatitis; skin rashes; recurrent, severe, watery diarrhea; malabsorption; and ultimately death. Biopsies of the small bowel exposed to the patientʼs serum revealed deposition of complement and immunoglobulins in the epithelium. Although not widely appreciated, JRA can herald a multisystem syndrome characterized by severe immune enteropathy.