Division of Pulmonary, Allergy, Critical Care and Sleep Medicine, Department of Medicine, Univesity of Minnesota Medical School, Minneapolis, USA.
Cardivascular Division, Department of Medicine, Univesity of Minnesota Medical School, Minneapolis, USA.
BMC Pulm Med. 2020 Jun 1;20(1):155. doi: 10.1186/s12890-020-01191-x.
Sarcoidosis is a systemic granulomatous disease of unknown etiology. Clinical cohort studies of different populations are important to understand the high variability in clinical presentation and disease course of sarcoidosis. The aim of the study is to evaluate clinical characteristics, including organ involvement, pulmonary function tests, and laboratory parameters, in a sarcoidosis cohort at the University of Minnesota. We compare the organ system involvement of this cohort with other available cohorts.
We conducted a retrospective data collection and analysis of 187 subjects with biopsy-proven sarcoidosis seen at a tertiary center. Organ system involvement was determined using the WASOG sarcoidosis organ assessment instrument. Clinical phenotype groups were classified using the Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis criteria.
Mean subject age at diagnosis was 45.8 ± 12.4, with a higher proportion of males (55.1%), and a higher proportion of blacks (17.1%) compared to the racial distribution of Minnesota residents (5.95%). The majority (71.1%) of subjects required anti-inflammatory therapy for at least 1 month. Compared to the A Case Control Etiologic Study of Sarcoidosis cohort, there was a higher frequency of extra-thoracic lymph node (34.2% vs. 15.2%), eye (20.9% vs. 11.8%), liver (17.6% vs. 11.5%), spleen (20.9% vs. 6.7%), musculoskeletal (9.6% vs. 0.5%), and cardiac (10.7% vs. 2.3%) involvement in our cohort. A multisystem disease with at least five different organs involved was identified in 13.4% of subjects. A restrictive physiological pattern was observed in 21.6% of subjects, followed by an obstructive pattern in 17.3% and mixed obstructive and restrictive pattern in 2.2%. Almost half (49.2%) were Scadding stages II/III. Commonly employed disease activity markers, including soluble interleukin-2 receptor and angiotensin-converting enzyme, did not differ between treated and untreated groups.
This cohort features a relatively high frequency of high-risk sarcoidosis phenotypes including cardiac and multiorgan disease. Commonly-utilized serum biomarkers do not identify subpopulations that require or do better with treatment. Findings from this study further highlight the high-variability nature of sarcoidosis and the need for a more reliable biomarker to predict and measure disease severity and outcomes for better clinical management of sarcoidosis patients.
结节病是一种病因不明的系统性肉芽肿性疾病。了解结节病临床表现和疾病过程的高度变异性，需要对不同人群的临床队列研究。本研究的目的是评估明尼苏达大学结节病队列的临床特征，包括器官受累、肺功能检查和实验室参数。我们将本队列的器官系统受累与其他可用队列进行比较。
我们对在三级中心就诊的 187 例经活检证实的结节病患者进行了回顾性数据收集和分析。使用 WASOG 结节病器官评估工具确定器官系统受累。使用 Alpha-1 抗胰蛋白酶缺乏症和结节病的基因组研究（Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis）标准将临床表型组分类。
诊断时患者的平均年龄为 45.8±12.4 岁，男性（55.1%）和黑人（17.1%）的比例高于明尼苏达州居民的种族分布（5.95%）。大多数（71.1%）患者需要至少 1 个月的抗炎治疗。与 A Case Control Etiologic Study of Sarcoidosis 队列相比，本队列中胸外淋巴结（34.2% vs. 15.2%）、眼（20.9% vs. 11.8%）、肝（17.6% vs. 11.5%）、脾（20.9% vs. 6.7%）、肌肉骨骼（9.6% vs. 0.5%）和心脏（10.7% vs. 2.3%）受累的频率更高。在 13.4%的患者中，发现了至少有五个不同器官受累的多系统疾病。21.6%的患者存在限制性生理模式，其次是阻塞性模式（17.3%）和混合阻塞性和限制性模式（2.2%）。近一半（49.2%）为 Scadding 分期 II/III。常用的疾病活动标志物，包括可溶性白细胞介素 2 受体和血管紧张素转换酶，在治疗组和未治疗组之间没有差异。
本队列的特征是高危结节病表型的频率相对较高，包括心脏和多器官疾病。常用的血清生物标志物不能识别需要治疗或治疗效果更好的亚群。本研究的结果进一步强调了结节病高度变异性的性质，需要更可靠的生物标志物来预测和衡量疾病的严重程度和结果，以更好地管理结节病患者。
