慢性リウマチ様関節炎のような症状を伴った原発性amyloidosis

A Rare Case of Primary Amyloidosis with Rheumatoid Arthritis and Various Symtoms

　Amyloidosisは1855年Virchow，1856年Wilks，1857年Buddらにより初めて記載された，全身性にAmyloidの沈着をきたす比較的稀な疾患である．近年本邦でも診断技術の進歩と相まってかなりの報告例をみるようになった．

　本症には原発性のものと他の疾患に附随して二次的に起こってくるものとが考えられるが，われわれは消化管にAmyloidが沈着した慢性リウマチ様関節炎に類似した関節症状を呈した原発性Amyloidosisの1症例を経験したので，文献的考察を加え報告する．

　A case of primary amyloidosis is described in this paper in a patient, treated for a long time as rheumatoid arthritis, who presented intestinal symptoms about one year and a half after the appearance of joint disorders.

　X-ray examination ― The stomach : No abnormality was seen. The small bowel: In an area extending from the duodenum to the jejunum the patterns of the Kerckring's folds were lost, replaced by very fine granular or reticular patterns. This finding was more distinct in the jejunum. The small bowel as a whole was moderately distended or hypotonic. These abnormal fine patterns were considered the results of atrophy of the intestinal mucosa and of deposition of amyloid in the mucosa and submucosa as was proven by biopsy. Delicate unevenness of the margins of the small bowel was regarded as tiny protrusions caused by amyloid deposits. The colon : Barium enema examination revealed loss of haustration from the rectum up to the Cannon-Bohm's ring. The colon was hypotonic as a whole and the descending colon was distended. Atrophy of the mucosa was noticed with loss of normal patterns. The over-distended segments were spotted with tiny flecks of contrast medium. In properly distended segments were seen very fine reticular patterns the same as in the small bowel. These changes of the small intestine did not differ qualitatively from those of the colon.

　Rectoscopic findings : The mucosa of the rectum 22 cm long as far up as from the anus was generally pale, anemic and easy to bleed.

　Biopsy of the rectum : Amyloid deposits, positive for Congo red stain, wereclearly seen in the interstitium of the lamina propria of the mucosa. It was also slightly recognized in the perivascular space in the muscularis mucosae and in the the submucosa.

　Biopsy of the jejunum : Biopsy of a segment 10 cm distal from the Treitz's ligament revealed deposition of amyloid in the lamina propria mucosae, murcularis mucosae and around small vessels in the submucosal layer.