特異な食道所見を呈したSjögren症候群の1例

Sjögren's Syndrome with Peculiar Esophageal Findings, a Case Report

　膠原病における食道病変は，強皮症（PSS：progressive systemic sclerosis）において，最も報告が多くみられ，全身性エリテマトーデス（SLE：systemic lupus erythematosus）においても，それに近い病変のあることが知られている．その多くは，平滑筋の機能異常または結合織増殖に一部基因する嚥下障害を主訴とし，X線的に食道の拡張，緊張低下，蠕動の減少あるいは消失，造影剤の通過時間の遅延および停滞が証明されている．

　一方，Sjögren症候群は，眼球乾燥（keratoconjunctivitis sicca），口腔内乾燥（xerostomia）のsicca syndromeに，膠原病の合併する疾患で，唾液分泌量減少による嚥下障害を訴えることが多いが，その食道病変については，国の内外を問わず，報告は皆無に等しい．

　A 26-year-old female had been diagnosed as SLE for 3 years before she complained of dysphagia, with dryness of eyes and mouth. A diagnosis of Sjögren's syndrome was made by sialogram and histological feature of parotid gland. Barium meal radiographs showed peculiar granulation of the lower half of the esophagus, with slight dilation and decreased peristalsis. By endoscopy, mucous membrane was found heavily covered with granular white coat, which was easily removed to expose bleeding or erosive surface. Histologically, it was found to show degeneration or necrosis of mucous membrane with marked cell infiltration. A lot of fungi were observed at the ulcereted area.

　These findings, completely different from those usually seen in PSS or related diseases, strongly suggested the possibility that the changes of esophagus were mainly due to the sicca syndrome characteristic of Sjögren's syndrome. Esophagitis followed by stasis of meals or regurgitation of stomach juice, and fungal proliferation from long-term administration of steroid, might also have participated in producing those changes. Although the presence of “progressive systemic sclerosis sine scleroderma” could not be excluded, we would like to postulate that the esophageal changes seen in this case were specific to Sjögren's syndrome and different from PSS.