十二指腸非乳頭部びまん性病変─消化管アミロイドーシスの十二指腸病変

Clinicopathological and Endoscopic Findings of Duodenal Lesions in Patients with Amyloidosis

要旨●アミロイドーシスの十二指腸病変の特徴を明らかにする目的で，アミロイド別の臨床像，内視鏡所見，病理組織学的所見を自験例23例（AL型14例，AA型9例）で遡及的に検討した．十二指腸内視鏡所見は，AA型の全例で粘膜粗糙や微細顆粒状粘膜/びらんを呈する一方，AL型の60%は皺襞肥厚や多発する粘膜下腫瘍様隆起を呈していた．粘膜下腫瘍様隆起はAL型のみで認められた．生検病理組織学的所見はアミロイド沈着の程度と沈着範囲で評価したが，明らかな関連性は認めなかった．アミロイド別の特徴的な内視鏡像や臨床像を把握することがアミロイドーシスの診断に重要であり，その基礎疾患を理解することが早期診断につながると考えられた．

　We retrospectively analyzed the relationship of types of amyloidosis with endoscopic findings in the duodenum and pathologic findings of biopsies in 23 patients with amyloidosis（14 with AL-type and 9 with AA-type）. For patients with AA-type amyloidosis, duodenal endoscopy revealed fine and granular-appearing tissue, mucosal friability, and erosions. Among the patients with AL-type amyloidosis, 60% exhibited multiple submucosal protrusions and fold thickening. Multiple submucosal protrusions were observed only in patients with AL-type disease. Pathologically, we assessed the degree and deposition of amyloid proteins in biopsy specimens. We found no relationship between pathological findings and endoscopic findings. It is important to understand the characteristic findings of duodenal endoscopy, stratified by the various types of amyloidosis and underlying disease processes.