Abstract
BackgroundSarcoidosis 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.MethodsWe 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.ResultsMean 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.ConclusionsThis 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.
Highlights
Sarcoidosis is a systemic granulomatous disease of unknown etiology
Sarcoidosis is a multi-system disease of unknown etiology characterized by the presence of non-caseating granulomas in involved organs [1]
Japanese cohorts have a higher incidence of cardiac disease, and cardiac involvement is the most frequent case of death due to sarcoidosis in Japan [13]
Summary
Clinical cohort studies of different populations are important to understand the high variability in clinical presentation and disease course of sarcoidosis. Mediastinal and hilar lymph nodes are most commonly involved, sarcoidosis is associated with heterogeneous manifestations, disease severity, and outcomes [1,2,3,4]. Four high-risk sarcoidosis manifestations were recognized as highly associated with greater morbidity and mortality [12]. These include treatment-resistant pulmonary disease, neuro-sarcoidosis, cardiac sarcoidosis, and multisystem organ involvement. The heterogeneity in sarcoidosis presents challenges in predicting disease course, optimizing treatments, and establishing a uniform standard for organ involvement assessment
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