分類不能大腸炎に合併した続発生アミロイドーシス（全身性AAアミロイドーシス）の1例

Systemic AA Amyloidosis Associated with Undetermind Inflammatory Bowel Disease, Report of an Autopsy Case

要旨　患者は32歳，女性．27歳から発熱，腹痛，29歳に頑固な腹痛，下痢，血便，蛋白尿を訴え，大腸X線検査，内視鏡検査にてHaustraの消失，内腔の狭小化，生検で陰窩膿瘍とアミロイド沈着を認めた．腎生検にてもアミロイド沈着は認められた．salazopyrin投与後，症状は緩解し更に生検で大腸粘膜の炎症およびアミロイド沈着は減少した．salazopyrin減量3か月後再び血性下痢，蛋白尿が出現した．初発後，約6年目にsubileusに肺炎，腎不全を併発し死亡した．剖検所見より本症は続発性アミロイドーシス（全身性AAアミロイドーシス）と判明し，アミロイド沈着は大腸から胃まで連続性に拡がり，粘膜固有層にのみ高度に沈着している点が特徴的であった．

　The case reported here was a 32-year-old female who first noticed fever and abdominal pain at the age of 27 years. Bloody diarrhea occurred at the age of 29 years, followed by proteinuria. X-ray study revealed loss of haustra and diffuse luminal narrowing of the left colon. Rectal biopsies showed mucosal inflammation with crypt abscesses and scattered amyloid globules. Mild amyloidosis was also demonstrated on renal biopsy. Salazopyrin, administered under the diagnosis of ulcerative colitis, induced disappearance of mucosal inflammation and amyloid globules, relieving intestinal symptoms and proteinuria. She was free of intestinal symptoms for the subsequent two years. Three months after Salazopyrin was reduced in dose, intractable bloody diarrhea and marked proteinuria developed. Sequential GI tract biopsies revealed further advanced mucosal amyloidosis. She died of subileus complicated by pneumonia and renal insufficiency at the age of 32 years, 6 years after the initial symptom had appeared.

　Systemic AA amyloidosis involving in particular, GI tract mucosa and shortening of the entire intestine was demonstrated by autopsy. Similar clinicopathologic features in a 34-year-old female was reported by Targgart et al in 1963.