A case of
glomerulonephritis in a 35-year-old man with
Crohn's disease is described here. The patient presented with severe
diarrhea, nephrotic range
proteinuria,
hematuria,
microangiopathic hemolytic anemia,
thrombocytopenia, hypocomplementemia,
acute renal failure requiring
hemodialysis,
cryoglobulinemia, and extensive thrombotic
gangrene of the distal upper and lower limbs. The patient did not respond to
plasmapheresis and
steroid therapy and died of upper gastrointestinal
bleeding. Renal tissue obtained at autopsy showed
IgA-mediated antiglomerular basement membrane crescentic
glomerulonephritis. Linear staining of the glomerular basement membrane by non-
IgG antibodies is quite unusual with only 11 cases previously reported in the worldwide literature, 8 caused by
IgA.
Glomerulonephritis is a rarely reported extraintestinal manifestation of
inflammatory bowel disease, and there are only 24 previously described cases that are reviewed and summarized in this report.
Glomerulonephritis occurred in the setting of active bowel
inflammation in all cases, circulating
immune complexes were found in nearly half the cases, and serum complements usually were normal.
Renal insufficiency and nephrotic range
proteinuria were typically present at the time of diagnosis of
glomerulonephritis and most often improved in parallel with treatment of the
gastrointestinal disorder. The histologic findings were varied and included
membranoproliferative glomerulonephritis, mesangioproliferative
glomerulonephritis, membranous nephropathy, IgA nephropathy, and
IgM nephropathy. Thus, the authors present the first case of
glomerulonephritis caused by antiglomerular basement membrane disease in association with
inflammatory bowel disease.