Fatal multisystem disease with immune enteropathy heralded by juvenile rheumatoid arthritis.
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.
Pearson
R D
RD
Department of Medicine, University of Virginia School of Medicine, Charlottesville, VA 22908.
Swenson
I
I
Schenk
E A
EA
Klish
W J
WJ
Brown
M R
MR
D001171:Arthritis, Juvenile / Q000150:complications*; D001327:Autoimmune Diseases / Q000209:etiology*; D004386:Duodenum / Q000473:pathology; D004751:Enteritis / Q000209:etiology*; D005260:Female; D006801:Humans; D007223:Infant; D008107:Liver Diseases / Q000209:etiology; D012871:Skin Diseases / Q000209:etiology