Case Report: PDF OnlyFatal Multisystem Disease with Immune Enteropathy Heralded by Juvenile Rheumatoid ArthritisPearson, Richard D.; Swenson, Ida; Schenk, Eric A.*; Klish, William J.†; Brown, Marilyn R.† Author Information Departments of Medicine and Pathology, University of Virginia School of Medicine, Charlottesville, Virginia; and *Departments of Pathology and † Pediatrics, University of Rochester School of Medicine, Rochester, New York, U.S.A. Journal of Pediatric Gastroenterology and Nutrition 8(2):p 259-265, February 1989. Free Abstract 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 patient's 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. © Lippincott-Raven Publishers.