ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 1479

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. Hammenfors9,10, Malin V. Jonsson11, Kathrine Skarstein12,13, Johan G. Brun9,10, Lars Rönnblom14, Helena Forsblad D’Elia15, Sara Magnusson Bucher16, Elke Theander17, Roald Omdal8, Roland Jonsson9,10, 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: , ,

Session Information

Date: Monday, November 6, 2017

Session 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 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).

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 January 20, 2019.

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