症例は68歳,女性. 1992年6月より慢性C型肝炎にて当科を受診中であった.
同年8月右上眼瞼腫脹のため眼科でブドウ膜炎と診断され,また,胸部X線写真にて両側肺門部腫脹(BHL)を認め,呼吸器科にて肺サルコイドーシスと診断,さらに両頬部に皮疹を認め,皮膚科にて皮膚サルコイドーシスと診断された.同年10月よりステロイド治療(プレドニゾロン40mg)を開始し症状が軽快, BHLは消失した.
2001年7月,口渇,眼の渇きが出現し,眼科,耳鼻科的検索にてシェーグレン症候群(SjS)と診断された. HCVとの関連を含めサルコイドーシスおよびSjSの病態を考察する上で貴重な1例と考えられた.

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症例は, 49歳女性.主訴は,上下肢の皮疹. 1983年頃より多関節痛,皮疹があり, 1985年当院を受診した.円板状皮疹,中枢神経障害,関節炎を呈し,血液検査では,白血球減少,血小板減少,抗核抗体陽性,抗DNA抗体陽性, LEテスト陽性,低補体血症を認め,全身性エリテマトーデス(以下SLE)と診断した. 1990年および1994年にループス腹膜炎を合併し,いずれもステロイドパルス療法にて改善した.その後,プレドニゾロン10mg/日でSLEの活動性はコントロールされている. 1993年から凍瘡様皮疹, 1996年にはレイノー現象, 1999年には背部,前胸部,及び両上肢に皮下硬結が出現,皮膚生検の結果, Lupus panniculitisと診断した. 2002年,右手,左膝に鱗屑を伴う紅斑様皮疹を認め皮膚生検にて尋常性乾癬と診断した.尋常性乾癬はSLEの皮膚病変としては比較的稀であり,また乾癬においてSLEと同様の自己免疫機序が推測されている点も両者の病因を考える上で興味深い. SLEの皮膚病変を考える上で,示唆に富む症例と考え報告する.

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To assess the frequency and the possibility of local production of autoantibodies against SS-A/Ro and SS-B/La in patients with primary Sjögren's syndrome (SS), serum and saliva samples were obtained from 42 patients with SS, 10 with rheumatoid arthritis without sicca syndrome, and 12 healthy volunteers. Autoantibodies were detected using enzyme-linked immunosorbent assay and immunoblotting. The frequencies of IgA anti-SS-A antibody, IgA anti-SS-B antibody, IgG anti-SS-A antibody and IgG anti-SS-B antibody in serum from SS patients were 45%, 50%, 43% and 21%, respectively. The frequencies of IgA anti-SS-A antibody, IgA anti-SS-B antibody, IgG anti-SS-A antibody and IgG anti-SS-B antibody in saliva from SS patients were 31%, 33%, 40%, and 19%, respectively. We also found secretory IgA anti-SS-A and anti-SS-B antibodies accompanying secretory components in saliva and sera in representative SS patients. Significant correlations were found between serum and salivary levels of IgA anti-SS-A antibodies, and between serum and salivary levels of IgA anti-SS-B antibodies in SS patients. Significant correlations were also found between serum and salivary levels of IgG anti-SS-A antibodies, and between serum and salivary levels of IgG anti-SS-B antibodies in SS patients. Immunoblot analysis confirmed the presence of IgA-class autoantibodies against SS-A and SS-B in saliva and serum from representative patients. The presence of IgA-and IgG-class autoantibodies against SS-A and SS-B and those accompanying secretory components in saliva from SS patients suggests the local production of these antibodies and the relationship between local and systemic antibody responses.

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