Pulmonary granulomas confirmed in Blau syndrome using TBLC specimens: Case report

Y. Shimizu, Yoshitomo Kushima, Ayae Tanaka, A. Takemasa, Kazuyuki Ishida, S. Niho
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Abstract

Blau syndrome (BS), is an autoinflammatory granulomatosis disease characterized by a distinct triad of skin, joint, and eye disorders similar to those of sarcoidosis, but the lung involvement frequently observed in sarcoidosis are rare. Granulomas from patients with BS displayed a distinct morphology indicating an exuberant chronic inflammatory response. Patients with BS may have granulomatous lung lesions, which require early diagnosis. To determine whether therapeutic intervention is needed for lung lesions, examining transbronchial lung cryobiopsy specimens and accumulating cases of BS with lung involvement could be contributed to improving BS management in the future.
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利用 TBLC 标本确诊布劳综合征肺肉芽肿病例报告
布劳综合征(BS)是一种自身炎症性肉芽肿病,其特点是皮肤、关节和眼部出现明显的三联症,与肉样瘤病相似,但很少出现肉样瘤病常出现的肺部受累症状。肉芽肿病患者的肉芽肿形态独特,表明其慢性炎症反应旺盛。BS 患者的肺部可能出现肉芽肿病变,需要及早诊断。为了确定是否需要对肺部病变进行治疗干预,检查经支气管肺冷冻活检标本和积累肺部受累的 BS 病例有助于改善未来的 BS 管理。
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