Sarcoidosis is a multisystemic granulomatous disease of unknown origin, that is generally presumed to be a T-helper and macrophage-driven disease. Follicular helper CD4+ T (Tfh) cells had a crucial role in the formation of germinal centers. We aimed to demonstrate a link between an imbalance of Tfh subsets in three anatomical compartments. Peripheral blood (PB), bronchoalveolar lavage (BAL) and lymph node (LLN) were collected from sarcoidosis patients at the same time at the moment of diagnosis before starting any pharmacological treatment. Flow-cytometry analysis was performed to detect Tfh (especially CCR4- and CXCR3-expressing Tfh subsets). We enrolled twenty-four patients (mean age 56; 60% females). High Tfh17 and Tfh2 cell percentages were found. The percentages of Tfh17 were higher in PB than BAL and LLN (p= 0.0242 and p= 0.0342, respectively) as well as Tfh2 percentages were higher in PB than BAL (p= 0.0035). Tfh1 cell percentages were not statistical significant different in the three compartments. Our findings revealed a peripheral predominance of the pro-inflammatory Tfh17 and Tfh2 subsets over that of Tfh1 cells that is typical of different systemic autoimmune diseases and of sarcoidosis. Thus, our results supported an association between the altered balance of Tfh subsets and pathogenesis of sarcoidosis. Tfh cells could be considered a potential therapeutic strategy for sarcoidosis patients.