POS1132 Skin involvement in the Catastrophic Antiphospholipid Syndrome (CAPS): Prevalence, clinical manifestations, histopathological features, correlation with antibody profile and mortality

Le résumé fourni par la source

Background: Catastrophic antiphospholipid syndrome (CAPS) is a severe form of antiphospholipid syndrome (APS), characterized by widespread thrombosis and multiorgan failure that develops within a week and affects 1% of APS patients [1]. Cutaneous involvement in CAPS is often easily recognized. A retrospective analysis of French APS/Systemic Lupus Erythematosus (SLE) registry [2] showed that half of CAPS patients exhibited cutaneous manifestations, with a broad spectrum of clinical presentations, including distal inflammatory oedema. Objectives: This study aimed to describe the prevalence, clinical manifestations, and histopathological features of skin involvement in CAPS. In addition, we analysed its correlation with antibody profile and mortality. Methods: We performed a cross-sectional study of the patients included in the "CAPS Registry," a registry developed by the European Forum on Antiphospholipid Antibodies (aPL) [3, 4]. This database contains data from patients with CAPS collected from April 1992 to December 2024. Demographic, clinical and pathological features, aPL profile, and outcomes were retrieved, along with information on the association with SLE diagnosis at the time of the CAPS event, the time to CAPS development, and mortality. Statistical analysis was performed using IBM-SPSS v.22 software. Mean values are reported with standard deviations. The chi-square test was used to assess differences between categorical data in patients with and without skin involvement. All statistical tests were two-tailed, and p-values <0.05 were considered statistically significant. Results: The CAPS Registry includes 854 patients with 875 episodes of CAPS. Data on cutaneous involvement were available for 377 episodes (43.1%). A female sex prevalence of 49.4% was observed. The average age was 39.43 (SD ±17.9) years. One hundred and ten patients (29.2%) had associated SLE, and 154 (40.8%) had primary APS (PAPS). All patients met the current classification criteria for CAPS [5]. Trigger events of CAPS were more frequent in the group with skin involvement (61.5% vs. 32.1%, p<0.000). However, each individual trigger was more frequent in the group without skin involvement (infection 29.7% vs. 63.4%, p<0.0001, surgery 9.5% vs. 83.3%, p<0.0001, pregnancy 6.9% vs. 83.5%, p<0.0001, oral contraceptives 4.8% vs. 86.2%, p<0.0001, neoplasia 2.7% vs. 89.7%, p<0.0001, puerperium 2.6% vs. 87.5%, p<0.0001, caesarean section 0.9% vs. 89.3%, p<0.0001). The most frequently affected organs in CAPS episodes with skin involvement were kidneys (72%), lungs (61%), central nervous system (54.5%) and heart (54.4%). The more frequent skin manifestations observed were livedo reticularis (17.6%), skin necrosis (13%), ischemic ulcers (10.4%), skin ischemic (9.7%), skin purpura (7.1%), gangrene (5%), splinter haemorrhages (2.6%), and Raynaud's phenomenon (1.5%). Thrombotic microangiopathy (TMA) features were present in 193 skin biopsies (22.1%), vasculitis in 7 (0.8%), cutaneous necrosis in 4 (0.5%), necrosis and thrombosis in 3 (0,3%), fibrosis in 3 (0.3%), capillaritis in 1 (0,1%) and necrosis and vasculitis in 1 (0.1%) sample. Only IgG β2GPI antibodies were significantly associated with the finding of TMA in skin biopsies (29.7% vs. 11.5%, p<0.0001). Thrombocytopenia, hemolysis, schistocytes, and the presence of IgG/IgM anticardiolipin (aCL) antibodies, IgG/IgM β2-glycoprotein (β2GPI), and lupus anticoagulant (LA) were more common in patients with skin involvement (p<0.005 for all) (Tables 1 and 2). Mortality was lower in CAPS episodes with skin involvement (29.3% vs. 32.1%, p<0.0001). Conclusion: The presence of skin involvement in patients with CAPS is frequent and it is mainly due to TMA. REFERENCES: [1] Cervera R. Antiphospholipid syndrome. Thromb Res 2017; 151(Suppl 1): S43–S71. [2] Dupré A, Morel N, Yeinik C, et al. Cutaneous involvement in catastrophic antiphospholipid syndrome in a multicenter cohort of 65 patients. JAMA Dermatol 2023 Jan 1; 159: 62-67. [3] Asherson RA, Cervera R, De Groot PG, et al. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus 2003; 12: 530–534. [4] Erkan D, Espinosa G, and Cervera R. Catastrophic antiphospholipid syndrome: updated diagnostic algorithms. Autoimmun Rev 2010; 10: 74–79. [5] Asherson RA, Cervera R, De Groot PG, et al. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus 2003; 12: 530–534. Acknowledgements: NIL . Disclosure of Interests: Dra Ana Ponce GSK, GSK, Ignasi Rodriguez-Pinto GSK, Gerard Espinosa GSK, Ricard Cervera Segura GSK. © The Authors 2025. This abstract is an open access article published in Annals of Rheumatic Diseases under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). Neither EULAR nor the publisher make any representation as to the accuracy of the content. The authors are solely responsible for the content in their abstract including accuracy of the facts, statements, results, conclusion, citing resources etc.

Ce résumé expose les affirmations des auteurs. BNTIC ne l’interprète pas comme une validation indépendante des résultats.