[A case of systemic AA amyloidosis complicating Crohn's disease]

Although systemic AA amyloidosis complicating Crohn's disease has been found in 0.5 to 6% in America and Europe, it is relatively rare in Japan. We report a case of systemic AA amyloidosis complicating Crohn's disease. In 1979, a 26-year-old Japanese man presented with diarrhea, melena and perianal abscesses, and was diagnosed as having Crohn's disease. He was treated with oral prednisolone, salazosulfapyridine and diet therapy. However, the gastrointestinal symptoms recurred and he was hospitalized several times. In 1991, his thyroid gland was found to be swollen, but with normal thyroid function, and his thyroid gland became larger subsequently. In October 1995, he showed renal dysfunction (blood urea nitrogen 33.2 mg/dl; serum creatinine 1.5 mg/dl) with proteinuria. His renal function had been deteriorating rapidly. On September 13, 1996, he was admitted to the Tsukuba University Hospital. At the time of admission, his renal function showed a blood urea nitrogen of 129.5 mg/dl with a creatinine of 5.4 mg/dl. The urine contained 0.8 g of protein per 24 hours. He presented with diarrhea for several days before admission and was treated with central venous hyperalimentation. Despite supportive care, he developed end-stage renal failure, then hemodialysis was initiated on October 7. His condition was complicated by a complete auriculoventricular block on October 18. He died of hemoperitoneum on October 25. On postmortem examination, extensive amyloid deposits were found in multiple organs including kidneys, intestine, heart, thyroid gland, lungs, liver, spleen, pancreas, gall bladder, adrenal glands, testis, prostate, bone marrow and parathyroid glands. Analysis of amyloid protein in the autopsy specimens showed type AA.