Session Type: ACR Poster Session B
Session Time: 9:00AM-11:00AM
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 identify differences in clinical presentation between the sexes at the time of diagnosis and during long-term follow-up of primary Sjögren’s syndrome (pSS), and to establish whether male sex is associated with a more severe form of pSS.
Incident, treatment naïve patients (n=199, 186 females and 13 males) from Stockholm, Sweden were prospectively included during a 5-year period and examined for items of classification criteria for pSS as well as extraglandular manifestations (EGM). Serum was sampled at the time of diagnosis and anti-Ro52/SSA levels measured by ELISA. Replication of significant findings was confirmed in an independent cohort of incident pSS patients from Pisa, Italy (n=377, 368 females and 9 males), and meta-analysis performed.
We further studied a cohort of 967 patients with prevalent 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, glandular, EGM and comorbidities were compared between men and women.
An increased frequency of EGM in men at diagnosis was observed and replicated (p=0.05, p=0.0003, and pmeta=0.002, respectively). This related to pulmonary involvement, vasculitis and lymphadenopathy being more common in men, for whom a lower age at diagnosis was observed in the exploratory cohort. Additionally, SSA positive male patients had significantly higher levels of anti-Ro52 levels than their female counterparts (p=0.02).
After long-term follow-up, male patient serology was characterized by more frequent positivity for anti-SSA and anti-SSB (p=0.02), and ANA (p=0.02). Also, 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).
Our analysis of two independent cohorts of incident pSS and a large cohort of prevalent pSS demonstrates significant differences between women and men with pSS. Notably, men present with more EGM, enhanced serological profile and a higher frequency of lymphoma development.
To cite this abstract in AMA style:Ramírez J, Kvarnstrom M, Brauner S, Baldini C, Eriksson P, Mandl T, Brække Norheim K, Johnsen SJ, Hammenfors DS, Jonsson MV, Skarstein K, Brun JG, Rönnblom L, Forsblad D’Elia H, Magnusson Bucher S, Theander E, Omdal R, Jonsson R, Nordmark G, Wahren-Herlenius M. Difference in Clinical Presentation between Female and Male Patients with Primary Sjogren’s Syndrome at Diagnosis and in Long-Term Follow-up [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/difference-in-clinical-presentation-between-female-and-male-patients-with-primary-sjogrens-syndrome-at-diagnosis-and-in-long-term-follow-up/. Accessed April 13, 2021.
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