University of Cincinnati Medical Center, Cincinnati, OH, USA
University of Utah Medical Center, Salt Lake City, UT, USA.
Eur Respir Rev. 2020 Mar 20;29(155). doi: 10.1183/16000617.0145-2019. Print 2020 Mar 31.
A variety of phenotypic categorisations have been developed for sarcoidosis. Phenotyping has been used for genetics studies and to guide treatment selection. The authors participated in a Delphi expert consensus panel to develop a proposed phenotype categorisation and treatment recommendations for pulmonary sarcoidosis patients. Panellists reached consensus that asymptomatic patients with normal pulmonary function and adenopathy alone or normal chest imaging do not require therapy, while symptomatic patients with impaired pulmonary function or infiltrates should be treated. The panel did not reach consensus on asymptomatic patients with abnormal chest imaging or reduced pulmonary function, or symptomatic patients with normal chest imaging and pulmonary function. The proposed phenotype categories and associated treatment recommendations are asymptomatic (no therapy), acute (disease duration <1-2 years, apparently self-limited, corticosteroids), chronic (antimetabolites and other second-line therapies) and advanced (biologics). Some clinical settings, such as dyspnoea/hypoxaemia at rest, severely impaired or rapidly decreasing pulmonary function tests, and severe cardiac, neurologic, ocular or renal involvement warrant immediate therapy.
已针对结节病开发出多种表型分类。表型分析已用于遗传学研究和指导治疗选择。作者参加了一项 Delphi 专家共识小组,以制定一种拟议的表型分类和治疗建议,用于治疗肺结节病患者。小组成员达成共识,无症状、肺功能正常且仅伴有淋巴结病或正常胸部影像学的患者不需要治疗,而有症状、肺功能受损或浸润的患者需要治疗。专家组对于无症状、胸部影像学异常或肺功能下降的患者,或有症状、胸部影像学和肺功能正常的患者,未达成共识。提出的表型分类和相关治疗建议为无症状(无需治疗)、急性(病程<1-2 年,表现为自限性,使用皮质类固醇)、慢性(抗代谢药物和其他二线治疗)和晚期(生物制剂)。一些临床情况,如静息时呼吸困难/低氧血症、严重受损或迅速下降的肺功能检查以及严重的心脏、神经、眼部或肾脏受累,需要立即治疗。