Shimizu Yasuo, Kushima Yoshitomo, Tanaka Ayae, Takemasa Akihiro, Ishida Kazuyuki, Niho Seiji
Department of Pulmonary Medicine and Clinical Immunology, Dokkyo Medical University, Mibu, Tochigi, Japan.
Respiratory Endoscopy Center, Dokkyo Medical University Hospital, Mibu, Japan.
Front Med (Lausanne). 2024 Jun 12;11:1380236. doi: 10.3389/fmed.2024.1380236. eCollection 2024.
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.
布劳综合征(BS)是一种自身炎症性肉芽肿病,其特征是具有与结节病相似的皮肤、关节和眼部疾病的独特三联征,但结节病中常见的肺部受累在布劳综合征中很少见。布劳综合征患者的肉芽肿表现出独特的形态,表明存在旺盛的慢性炎症反应。布劳综合征患者可能有肉芽肿性肺部病变,需要早期诊断。为了确定肺部病变是否需要进行治疗干预,检查经支气管肺冷冻活检标本并积累布劳综合征肺部受累病例,可能有助于未来改善布劳综合征的管理。