Comparison of Clinical Phenotype, Serological Characteristics and Histologic Features Between Males and Females Patients with Primary Sjögren’s Syndrome (pSS)

Loukas Chatzis¹, Saviana Gandolfo ², Francesco Ferro ³, Marco Binutti ², Valentina Donati ⁴, Sara Zandonella Callegher ⁵, Vasilis Pezoulas ⁶, Aliki Venetsanopoulou ⁷, Argyropoulou Ourania ⁷, Georgios Michalopoulos ⁷, maria pappa ⁷, Clio Mavragani ⁷, Dimitris fotiadis ⁸, Chiara Baldini ³, Salvatore De Vita ², Athanasios Tzioufas ¹ and Andreas Goules ⁷, ¹Department of Pathophysiology, Athens School of Medicine, National and Kapodistrian University of Athens, Athens, Greece, Athens, Greece, ²Rheumatology Clinic, Udine University Hospital, Department of Medical Area, University of Udine, Udine, Italy, Udine, Italy, ³Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy, Pisa, Italy, ⁴University of Pisa, Pisa, ⁵Rheumatology Clinic, Udine University Hospital, Udine, Italy, Udine, Italy, ⁶Unit of Medical Technology and Intelligent Information Systems, University of Ioannina, GR45110, Ioannina, Greece, Ioannina, Greece, ⁷Pathophysiology Department, Athens School of Medicine, National and Kapodistrian University of Athens, Athens, Greece, Athens, Greece, ⁸Pathophysiology Department, Athens School of Medicine, National and Kapodistrian University of Athens, Athens, Greece, Ioannina, Greece

Meeting: 2019 ACR/ARP Annual Meeting

Keywords: SICCA and MALT lymphoma, Sjogren's syndrome

Session Information

Date: Monday, November 11, 2019

Title: Sjögrenʼs Syndrome – Basic & Clinical Science Poster II

Session Type: Poster Session (Monday)

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

Background/Purpose: Primary Sjögren’s syndrome (pSS) is a female predominant autoimmune disease and very few studies have been conducted to investigate the phenotypic differences of the disease between the two genders. The purpose of this study is to investigate whether gender in pSS interferes with clinical manifestations, serology, disease course and lymphoma development, in a multi-center analysis from three centers specializing in Sjögren’s Syndrome [Udine, Piza, Athens-(UPA)].

Methods: The combined cohorts of the 3 Mediterranean centers were harmonized and 82 male pSS patients, who fulfill the 2016 ACR/EULAR criteria for Sjögren’s, were recruited from a pool of 1605pSS patients (n=254from Udine, n=753from Piza, n=598fromAthens). Each male was matched to a female patient in a 1:2 ratio, according to age and disease duration from SS onset. Glandular (dry mouth, dry eyes, parotid gland enlargement) and extra-glandular manifestations (Raynaud’s phenomenon, lymphadenopathy, arthralgias/arthritis, palpable purpura, liver involvement, kidney involvement, lymphoma) as well as serology (anti Ro/SSA, anti La/SSB, rheumatoid factor, cryoglobulinemia, low C4 complement levels) and histologic features (focus score, presence of germinal centers) were recorded and compared. Statistical analysis for categorical data was performed by Fisher exact test or χ²square test accordingly and numerical data with Man-Whitney test.

Results: The median age of disease onset was 50 years (range: 15-77 years) for the male group and 50 years (range: 15-78 years) for the females. The median disease duration was 7,73 years (range: 0-26 years) and 8,38 years (range: 0-26 years) for males and females respectively. The male to female ratio in the total harmonized population was approximately 1:20. Interestingly, males with pSS had statistically significant higher frequency of lymphoma compared to females [15/82 (18.2%) vs 9/163 (5.5%) respectively, p=0.003]. Anti-La/SSB antibodies were more frequently detected in males compared to females [41/81 (50,6%) vs 54/162 (33.3%), respectively, p=0.015]. Finally, the prevalence of sicca manifestations including both dry mouth and dry eyes was higher among females compared to males [97,5% vs 91,2%, p=0.046, for dry mouth and 96,3% vs 87,8% for dry eyes, p=0.022, respectively].

Conclusion: To our knowledge this is the largest study comparing males with females pSS patients after applying the 2016 ACR/EULAR classification criteria. The higher frequency of lymphoma among males without classical risk factors may suggest distinct lymphomagenesis mechanisms between the two genders, implying that gender could be an independent risk factor for lymphoma development among pSS patients. Furthermore, the difference in the prevalence of anti-La/SSB antibodies and sicca symptoms indicates a potential role of gender and hormones in the production of autoantibodies and the clinical phenotype of pSS.

Disclosure: L. Chatzis, None; S. Gandolfo, None; F. Ferro, None; M. Binutti, None; V. Donati, None; S. Zandonella Callegher, None; V. Pezoulas, None; A. Venetsanopoulou, None; A. Ourania, None; G. Michalopoulos, None; m. pappa, None; C. Mavragani, None; D. fotiadis, None; C. Baldini, None; S. De Vita, None; A. Tzioufas, None; A. Goules, None.

To cite this abstract in AMA style:

Chatzis L, Gandolfo S, Ferro F, Binutti M, Donati V, Zandonella Callegher S, Pezoulas V, Venetsanopoulou A, Ourania A, Michalopoulos G, pappa m, Mavragani C, fotiadis D, Baldini C, De Vita S, Tzioufas A, Goules A. Comparison of Clinical Phenotype, Serological Characteristics and Histologic Features Between Males and Females Patients with Primary Sjögren’s Syndrome (pSS) [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/comparison-of-clinical-phenotype-serological-characteristics-and-histologic-features-between-males-and-females-patients-with-primary-sjogrens-syndrome-pss/. Accessed .

« Back to 2019 ACR/ARP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/comparison-of-clinical-phenotype-serological-characteristics-and-histologic-features-between-males-and-females-patients-with-primary-sjogrens-syndrome-pss/