Abstract
BackgroundSarcoidosis is a poorly understood chronic inflammatory condition. Infiltration of affected organs by lymphocytes is characteristic of sarcoidosis, however previous reports suggest that circulating lymphocyte counts are low in some patients with the disease. The goal of this study was to evaluate lymphocyte subsets in peripheral blood in a cohort of sarcoidosis patients to determine the prevalence, severity, and clinical features associated with lymphopenia in major lymphocyte subsets.Methodology/Principal FindingsLymphocyte subsets in 28 sarcoid patients were analyzed using flow cytometry to determine the percentage of CD4, CD8, and CD19 positive cells. Greater than 50% of patients had abnormally low CD4, CD8, or CD19 counts (p<4×10−10). Lymphopenia was profound in some cases, and five of the patients had absolute CD4 counts below 200. CD4, CD8, and CD19 lymphocyte subset counts were significantly correlated (Spearman's rho 0.57, p = 0.0017), and 10 patients had low counts in all three subsets. Patients with severe organ system involvement including neurologic, cardiac, ocular, and advanced pulmonary disease had lower lymphocyte subset counts as a group than those patients with less severe manifestations (CD4 p = 0.0043, CD8 p = 0.026, CD19 p = 0.033). No significant relationships were observed between various medical therapies and lymphocyte counts, and lymphopenia was present in patients who were not receiving any medical therapy.Conclusions/SignificanceSignificant lymphopenia involving CD4, CD8, and CD19 positive cells was common in sarcoidosis patients and correlated with disease severity. Our findings suggest that lymphopenia relates more to disease pathology than medical treatment.
Highlights
Sarcoidosis is a systemic inflammatory disease characterized by noncaseating granulomas which consist of CD4+ T-cells and macrophages surrounded by CD8+ T-cells [1]
The goal of this study was to evaluate lymphocyte subsets in peripheral blood in a cohort of sarcoidosis patients followed at the University of Chicago Rheumatology Clinic to determine the prevalence, severity, and clinical features associated with lymphopenia in major lymphocyte subsets
This study provides the first report of frequent and severe CD4, CD8, and CD19 lymphopenia in sarcoidosis patients to our knowledge
Summary
Sarcoidosis is a systemic inflammatory disease characterized by noncaseating granulomas which consist of CD4+ T-cells and macrophages surrounded by CD8+ T-cells [1]. It was demonstrated that CD4+CD25brightFoxP3+ cells accumulated in the periphery of sarcoid granulomas, in BAL washings, and in peripheral blood of patients with active disease These cells effectively suppressed the proliferation of effector cells but not their production of TNF. The goal of this study was to evaluate lymphocyte subsets in peripheral blood in a cohort of sarcoidosis patients followed at the University of Chicago Rheumatology Clinic to determine the prevalence, severity, and clinical features associated with lymphopenia in major lymphocyte subsets. The goal of this study was to evaluate lymphocyte subsets in peripheral blood in a cohort of sarcoidosis patients to determine the prevalence, severity, and clinical features associated with lymphopenia in major lymphocyte subsets
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