Clinical characteristics and organ system involvement in sarcoidosis: comparison of the University of Minnesota Cohort with other cohorts

Abstract

Background: 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. Methods: 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. Results: 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.

Clinical characteristics and outcomes of Korean patients with sarcoidosis

“…In a population-based cohort study conducted in the United States (US), the Scadding radiographical stage of pulmonary sarcoidosis at diagnosis was a strong predictor for chronic respiratory impairment defined as forced vital cavity (FVC) or DL co of < 50% and < 40% of the predicted values, respectively, or as the requirement for oxygen supplementation (hazard ratio, 8.36 for stages III and IV versus stage I) 29 . In addition, in a retrospective study using a sarcoidosis cohort in the US, abnormal pulmonary function were observed in 41.1% of the subjects (a restrictive pattern in 21.6%, an obstructive pattern in 17.3%, and a mixed obstructive and restrictive pattern in 2.2%) 30 ; however, in our study, 29.4% of the subjects showed abnormal lung function (a restrictive pattern in 19.8%, an obstructive patterns in 7.3%, and a mixed pattern in 2.3%). Moreover, mean lung function was better in our study group (FVC; 89.8% vs. 85.2%, FEV1; 87.7% vs 85.2%) 30 .…”

Sarcoidosis: Review of Diagnosis and Treatment

“…Spectrum of Disease: The lungs are initially involved or subsequently involved in 91-98 % of patients with the most common presentation being a dry cough with mild to moderate dyspnea on exertion, often accompanied by atypical chest pain, fatigue and malaise [24][25][26][27][28]. Fifty to sixty percent of patients have more than one organ involved [25].…”

“…Spectrum of Disease: The lungs are initially involved or subsequently involved in 91-98 % of patients with the most common presentation being a dry cough with mild to moderate dyspnea on exertion, often accompanied by atypical chest pain, fatigue and malaise [24][25][26][27][28]. Fifty to sixty percent of patients have more than one organ involved [25]. The most common sites of extrapulmonary [29], eye-uveitis (12.4%) [30], liver (11.5%) [31], neurologic (8%) [33], ENT/parotid/salivary (6.7%) bone/joint (4.5%) [34][35][36] cardiac (3.7%) [32] hypercalcemia (3.5%), bone marrow (3.4%), renal or muscle (<2%) [25].…”

“…Fifty to sixty percent of patients have more than one organ involved [25]. The most common sites of extrapulmonary [29], eye-uveitis (12.4%) [30], liver (11.5%) [31], neurologic (8%) [33], ENT/parotid/salivary (6.7%) bone/joint (4.5%) [34][35][36] cardiac (3.7%) [32] hypercalcemia (3.5%), bone marrow (3.4%), renal or muscle (<2%) [25].…”

“…Follow-up intervals for sarcoid are not standardized. However, one should keep in mind that as many as 50% of patients will have a remission in 3 years, and 66% in 10 years, and yet, approximately 1/3 rd of patients will have complications of vital organs (lungs, heart, CNS, hepatic, renal) with progressive disease [25,27,50,67,69]. One should also remember that the status of pulmonary, ocular, cutaneous and hepatic sarcoid sarcoid are easier to monitor than cardiac or neurologic disease and that involvement of three of more organs, stage 3 or 4 chest radiograph, posterior uvieits, age >40, African American heritage, hypercalcemia, the extent of cardiac or neurologic disease, lupus pernio and >6 months are risk factors for disease recurrence [25][26][27]37,39,50].…”