Difference in Clinical Presentation between Female and Male Patients with Primary Sjogren’s Syndrome at Diagnosis and in Long-Term Follow-up

Jorge Ramírez¹, Marika Kvarnstrom², Susanna Brauner³, Chiara Baldini⁴, Per Eriksson⁵, Thomas Mandl⁶, Katrine Brække Norheim⁷, Svein Joar Johnsen⁸, Daniel S. Hammenfors^9,10, Malin V. Jonsson¹¹, Kathrine Skarstein^12,13, Johan G. Brun^9,10, Lars Rönnblom¹⁴, Helena Forsblad D’Elia¹⁵, Sara Magnusson Bucher¹⁶, Elke Theander¹⁷, Roald Omdal⁸, Roland Jonsson^9,10, Gunnel Nordmark¹⁸ and Marie Wahren-Herlenius¹⁹, ¹Unit of Experimental Rheumatology, Department of Medicine, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden, Solna, Sweden, ²Unit of Rheumatology, Department of Medicine, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden, Stockholm, Sweden, ³Department of Clinical Neuroscience, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden, Stockholm, Sweden, ⁴Internal Medicine, Rheumatology Unit, University of Pisa, Pisa, Italy, ⁵Division of Rheumatology, Department of Clinical Experimental Medicine, Linköping University, Linköping, Sweden, Linköping, Sweden, ⁶Department of Rheumatology, Skåne University Hospital, Malmö, Sweden, Lund, Sweden, ⁷Department of Internal Medicine, Clinical Immunology Unit, Department of Internal Medicine, Stavanger University Hospital, Stavanger, Norway, Stavanger, Norway, ⁸Clinical Immunology Unit, Department of Internal Medicine, Stavanger University Hospital, Stavanger, Norway, Stavanger, Norway, ⁹Broegelmann Research Laboratory, Department of Clinical Science, University of Bergen, Bergen, Norway, Bergen, Norway, ¹⁰Department of Rheumatology, Haukeland University Hospital, Bergen, Norway, Bergen, Norway, ¹¹Section for Oral and Maxillofacial Radiology, Department of Clinical Dentistry, University of Bergen, Bergen, Norway, Bergen, Norway, ¹²Gade Laboratory for Pathology, Department of Clinical Medicine, University of Bergen, Bergen, Norway, ¹³Department of Pathology, Haukeland University Hospital, Bergen, Bergen, Norway, ¹⁴Rheumatology and Science for Life Laboratory, Department of Medical Sciences, Uppsala University, Sweden, Uppsala, Sweden, ¹⁵Department of Public Health and Clinical Medicine, Rheumatology, Umeå University, Umeå, Sweden., Umeå, Sweden, ¹⁶Department of Rheumatology, Faculty of Medicine and Health, Örebro University, Örebro, Sweden, Örebro, Sweden, ¹⁷Department of Rheumatology, Skåne University Hospital, Malmö, Sweden, Malmö, Sweden, ¹⁸Department of Medical Sciences, Section of Rheumatology, Uppsala University, Uppsala, Sweden, Uppsala, Sweden, ¹⁹Unit of Experimental Rheumatology, Department of Medicine, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden, Stockholm, Sweden

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: autoimmune diseases, severity and sex bias, Sjogren's syndrome

Session Information

Date: Monday, November 6, 2017

Title: Sjögren's Syndrome Poster II: Clinical Research

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose:

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.

Methods:

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.

Results:

An increased frequency of EGM in men at diagnosis was observed and replicated (p=0.05, p=0.0003, and p_meta=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).

Conclusion:

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.

Disclosure: J. Ramírez, None; M. Kvarnstrom, None; S. Brauner, None; C. Baldini, None; P. Eriksson, None; T. Mandl, None; K. Brække Norheim, None; S. J. Johnsen, None; D. S. Hammenfors, None; M. V. Jonsson, None; K. Skarstein, None; J. G. Brun, None; L. Rönnblom, None; H. Forsblad D’Elia, None; S. Magnusson Bucher, None; E. Theander, None; R. Omdal, None; R. Jonsson, None; G. Nordmark, None; M. Wahren-Herlenius, None.

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 .

« Back to 2017 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - 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/