膠原病に合併する十二指腸びまん性病変―血管炎症候群を中心に

Endoscopic Findings in Duodenum of Systemic Vasculitis Patients

要旨　膠原病に合併する十二指腸びまん性病変症例（結節性多発動脈炎など血管炎症候群）についての内視鏡所見の検討を行った．十二指腸での病変は血管炎に伴う循環障害が十二指腸に起こって発症すると考えられ，内視鏡上，十二指腸球部より下行脚にかけての広範な粘膜びらん，難治性の浅い潰瘍の形成と動脈性出血を呈した．生検上非特異的な炎症所見を認めるのみで特徴的な所見に乏しく，診断は難しい．治療に対する反応に乏しく潰瘍，出血を繰り返す症例では腎不全，急性膵炎，脳血管障害などを合併して重篤となることが多く予後不良である．病初期での診断と全身疾患としての早期治療開始が予後改善のためには重要である．

　We investigated endoscopic findings of the duodenum in patients with systemic vasculitis. Out of 37 patients with systemic vasculitis, duodenal lesions were found to be affected in 9 patients. Nine patients, comprised of 2 patients with polyarteritis nodosa, 2 with microscopic polyarteritis and one each with Henoch-Schönlein purpura, Rheumatoid arthritis, Wegener, Dermatomyositis and SLE respectioely. The duodenal vasculitic lesions demonstrated clinically by gastroduodenoscopy were confirmed histologically. Endoscopy revealed multiple early lesions, separated by mildly edematous or red inflamed mucosa in the superior duodenum in all patients. These lesions developed into erosions, dome-shaped shallow ulcers and bled profusely. Histological assessment of the gut biopsies revealed non-specific inflammation or ulceration and intramucosal hemorrhage. Areas of necrosis and ulceration in endoscopic biopsies were highly suggestive of vasculitis whereas arteritis was seen in surgical specimens only.

　In conclusion, the duodenal vasculitic lesions presenting as an acute abdominal emergency are an uncommon, but often fatal presentation of systemic vasculitis. Endoscopic findings and early aggressive medical therapy may contribute significantly towards an overall successful outcome.