Hua Jeremy T, Cool Carlyne D, Klein Einat Fireman, Adir Yochai, Lee Lukas J, Zell-Baran Lauren M, Cohen Robert A, Kraus Richard C, Brigitte Gottschall E, Krefft Silpa D, Van Hook Charles, Rose Cecile S
Division of Environmental and Occupational Health Sciences, National Jewish Health, Denver, Colorado, USA.
Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado School of Medicine, Aurora, Colorado, USA.
Am J Ind Med. 2025 Jun;68(6):491-507. doi: 10.1002/ajim.23724. Epub 2025 Apr 25.
Sarcoidosis is a multisystem inflammatory disease of unknown etiology. Growing evidence indicates that occupational exposure to respirable crystalline silica (RCS) is associated with an increased incidence of sarcoidosis. Yet a diagnosis of sarcoidosis rarely prompts investigation to identify preventable exposures. We sought to elucidate features that identify this important clinical syndrome of silicosarcoidosis. We assembled a multinational case series of workers with sarcoidosis who also reported occupational RCS exposure. We characterized clinical and histopathologic findings using a standardized instrument. We also assessed lung specimens using a novel quantitative microscopy technique to measure birefringent dust density in silicosarcoidosis cases and compared them to control groups. We identified 35 silicosarcoidosis cases (97% male, mean age 48 years) from the United States, Israel, and Taiwan who reported 21 ± 9 years of RCS exposure. On histology scoring, 25/29 (86%) had granulomas and 17/18 (94%) with evaluable lung tissue had lymphocytic inflammation and/or lymphoid aggregates. Common lung interstitial findings included silicotic nodules (39%), mixed-dust macules/nodules (44%), and birefringent dust (50%). Quantitative birefringent dust density was significantly greater (p < 0.001) in silicosarcoidosis cases compared with healthy controls (147 ± 179 vs. 12 ± 9 particles/mm) but lower than in coal miners with silica-related progressive massive fibrosis (623 ± 777). We found significant differences in the frequency of histologic abnormalities in large versus small biopsy specimens, with fewer findings of RCS exposure in smaller tissue samples. The use of the term silicosarcoidosis should enhance recognition of this significant exposure-related granulomatous lung disease and will help guide clinical management that addresses exposure prevention in combination with appropriate pharmacologic treatment.
结节病是一种病因不明的多系统炎症性疾病。越来越多的证据表明，职业性接触可吸入结晶二氧化硅（RCS）与结节病发病率增加有关。然而，结节病的诊断很少促使人们进行调查以确定可预防的接触因素。我们试图阐明识别这种重要的硅结节病临床综合征的特征。我们收集了一组来自多个国家的结节病工人病例系列，这些工人也报告有职业性RCS接触。我们使用标准化工具对临床和组织病理学发现进行了特征描述。我们还使用一种新型定量显微镜技术评估肺标本，以测量硅结节病病例中的双折射粉尘密度，并将其与对照组进行比较。我们从美国、以色列和台湾地区识别出35例硅结节病病例（97%为男性，平均年龄48岁），这些病例报告有21±9年的RCS接触史。在组织学评分中，25/29（86%）有肉芽肿，17/18（94%）有可评估肺组织的病例有淋巴细胞炎症和/或淋巴样聚集。常见的肺间质表现包括硅结节（39%）、混合粉尘斑/结节（44%）和双折射粉尘（50%）。与健康对照组相比，硅结节病病例中的定量双折射粉尘密度显著更高（p<0.001）（147±179对12±9颗粒/mm），但低于患有与二氧化硅相关的进行性大块纤维化的煤矿工人（623±777）。我们发现大活检标本与小活检标本中组织学异常频率存在显著差异，较小组织样本中RCS接触的发现较少。使用“硅结节病”一词应能增强对这种与接触相关的重要肉芽肿性肺病的认识，并有助于指导结合适当药物治疗和接触预防的临床管理。
