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Background  Despite men being less prone to develop autoimmune diseases, male sex has been associated with a more severe disease course in several systemic autoimmune diseases. In the present study, we aimed to investigate differences in the clinical presentation of primary Sjögren’s syndrome (pSS) between the sexes and establish whether male sex is associated with a more severe form of long-term pSS.    Methods  Our study population included 967 patients with pSS (899 females and 68 males) from Scandinavian clinical centers. The mean follow-up time (years) was 8.8 ± 7.6 for women and 8.5 ± 6.2 for men (ns). Clinical data including serological and hematological parameters and glandular and extraglandular manifestations were compared between men and women.    Results  Male patient serology was characterized by more frequent positivity for anti-Ro/SSA and anti-La/SSB ( p  = 0.02), and ANA ( p  = 0.02). Further, men with pSS were more frequently diagnosed with interstitial lung disease ( p  = 0.008), lymphadenopathy ( p  = 0.04) and lymphoma ( p  = 0.007). Conversely, concomitant hypothyroidism was more common among female patients ( p  = 0.009).    Conclusions  We observe enhanced serological responses and higher frequencies of lymphoma-related extraglandular manifestations in men with pSS. Notably, lymphoma itself was also significantly more common in men. These observations may reflect an aggravated immune activation and a more severe pathophysiological state in male patients with pSS and indicate a personalized managing of the disease due to the influence of the sex of patients with pSS.

Long-term follow-up in primary Sjögren’s syndrome reveals differences in clinical presentation between female and male patients