Liala Moschetti1, Luisa Dal Pozzolo2, Veronique Le Guern3, Nathalie Morel3, Cecile M Yelnik4, Marc Lambert4, Eric Hachulla4, Ygal Benhamou5, Cecilia Nalli6, Micaela Fredi7, Franco Franceschini7, Laura Andreoli7, Nathalie Costedoat-Chalumeau8, Angela Tincani7. 1. Rheumatology and Clinical Immunology Unit, ASST Spedali Civili, Brescia, and Department of Clinical and Experimental Sciences, University of Brescia, Italy. l.moschetti001@unibs.it. 2. Department of Clinical and Experimental Sciences, University of Brescia, Italy. 3. AP-HP, Cochin Hospital, Internal Medicine Department, Centre de Référence Maladies Auto-Immunes et Systémiques Rares d'île de France; Université de Paris, France. 4. Université de Lille, INSERM and CHU LILLE, Service de Médecine Interne et d'Immunologie Clinique, Centre de Référence des Maladies Auto-Immunes Systémiques Rares du Nord et Nord-Ouest de France (CeRAINO), Lille, France. 5. Département de Médecine Interne, CHU Charles Nicolle and Normandie University, UNIROUEN, INSERM U1096 EnVI, Rouen, France. 6. Rheumatology and Clinical Immunology Unit, ASST Spedali Civili, Brescia, Italy. 7. Rheumatology and Clinical Immunology Unit, ASST Spedali Civili, Brescia, and Department of Clinical and Experimental Sciences, University of Brescia, Italy. 8. AP-HP, Cochin Hospital, Internal Medicine Department, Centre de Référence Maladies Auto-Immunes et Systémiques Rares d'île de France, Université de Paris, France.
Abstract
OBJECTIVES: Gender can influence incidence and clinical course of autoimmune diseases (ADs). Antiphospholipid syndrome (APS) is a rare AD characterised by thromboses and/or pregnancy morbidities and antiphospholipid antibodies (aPL) positivity. Our aim is to conduct a gender-oriented analysis of primary thrombotic APS (t-APS). METHODS: Consecutive patients diagnosed with primary t-APS, followed from 1967 to 2019 in four European Centres, were enrolled. RESULTS: The cohort included 296 women and 137 men. Median age at onset [31 (24-46) vs. 41 (29-53) years, p<0.001] was lower in females. In women, venous thromboses were more frequent while, among males, arterial events prevailed. During follow-up, 14% of patients suffered at least two relapses and this occurred especially among males (22% vs. 10%, p=0.001). No gender differences were found in the aPL profile (33% single, 24% double and 43% triple aPL positivity). Most patients had concomitant risk factors (RFs) for thrombosis: established cardiovascular RFs were represented especially among men while estrogenic exposure was the main RF in women. CONCLUSIONS: Women presented mostly with venous thromboses at a younger age, while men with arterial events, later in life and suffered more recurrent events. This different frequency of arterial and venous thromboses could be attributed mainly to the presence of additional RFs rather than to biological gender-specific issues. However, some RFs are exclusive or more represented in one gender rather than the other, so assessing the link of causality between gender and manifestations of t-APS remains difficult.
OBJECTIVES: Gender can influence incidence and clinical course of autoimmune diseases (ADs). Antiphospholipid syndrome (APS) is a rare AD characterised by thromboses and/or pregnancy morbidities and antiphospholipid antibodies (aPL) positivity. Our aim is to conduct a gender-oriented analysis of primary thrombotic APS (t-APS). METHODS: Consecutive patients diagnosed with primary t-APS, followed from 1967 to 2019 in four European Centres, were enrolled. RESULTS: The cohort included 296 women and 137 men. Median age at onset [31 (24-46) vs. 41 (29-53) years, p<0.001] was lower in females. In women, venous thromboses were more frequent while, among males, arterial events prevailed. During follow-up, 14% of patients suffered at least two relapses and this occurred especially among males (22% vs. 10%, p=0.001). No gender differences were found in the aPL profile (33% single, 24% double and 43% triple aPL positivity). Most patients had concomitant risk factors (RFs) for thrombosis: established cardiovascular RFs were represented especially among men while estrogenic exposure was the main RF in women. CONCLUSIONS: Women presented mostly with venous thromboses at a younger age, while men with arterial events, later in life and suffered more recurrent events. This different frequency of arterial and venous thromboses could be attributed mainly to the presence of additional RFs rather than to biological gender-specific issues. However, some RFs are exclusive or more represented in one gender rather than the other, so assessing the link of causality between gender and manifestations of t-APS remains difficult.