AB0340 PATIENTS WITH LABORATORY CRITERIA OF ANTIPHOSPHOLIPID SYNDROME AND “NON-CRITERIA” MANIFESTATIONS: A MULTICENTER STUDY
Authors: G. Pires Da Rosa, E. Ferreira, B. Sousa-Pinto, P. Bettencourt, R. Cervera, G. Espinosa
Journal: Annals of the Rheumatic Diseases (2021)

Background: Patients with laboratory criteria for antiphospholipid syndrome (APS) and presenting only “non-criteria” clinical manifestations are scarcely described in the literature [1-3]. Objectives: To describe a cohort of these patients, portraying potential associations between “non-criteria” manifestations, antiphospholipid antibodies (aPL) and treatments. Methods: Retrospective analysis of patients with aPL positivity fulfilling APS criteria assessed in two tertiary European hospitals between 2005 and 2020. Those without criteria clinical manifestations but with the presence of “non-criteria” manifestations were included. Results: A total of 75 patients were analyzed. The predominant non-obstetric involvements were hematologic (thrombocytopenia, hemolytic anemia) and “mild” neurological (white matter lesions, migraine), with an absence or near absence of cutaneous, cardiac, pulmonary and more severe neurologic manifestations (Table 1). In obstetric morbidity, two spontaneous abortions before 10 weeks and infertility were the more frequent manifestations. LA positivity was positively associated with non-obstetric manifestations (OR=4.2, 95% CI 1.5-11.9; p=0.007) while an aCL profile was associated with obstetric manifestations (OR 7.0, 95% CI 2.3-21.3; p=0.001). A suspicion of progression towards SLE was considered plausible in 13 (19%) of non-SLE individuals. Twelve (40.0%) of patients with thrombocytopenia as their only hematological involvement required corticosteroids. History of infertility (OR 6.1, 95% CI 1.3-28; p=0.02) and IVF recurrent failure (OR 6.5, 95% CI 1.2-36.3; p=0.032) were associated with pregnancy treatment with LMWH/LDA combination. Among patients under anticoagulation, clinical manifestations included brain white matter lesions, APS nephropathy, superficial vein thrombosis, amaurosis fugax, and livedoid vasculopathy. A higher value of GAPSS was observed in patients with non-obstetric (median 9, IQR 5-13) in comparison to those with obstetric manifestations (median 5, IQR 4-5.5). Conclusion: The present article stresses the clinical relevance of these patients, with an impression that at least some manifestations will contribute in the future to the classification of individuals as definite APS. References: [1]J. Alijotas-Reig et al. , Comparative study of obstetric antiphospholipid syndrome (OAPS) and non-criteria obstetric APS (NC-OAPS): report of 1640 cases from the EUROAPS registry. Rheumatology (Oxford ), (2019). [2]M. Fredi et al. , Risk Factors for Adverse Maternal and Fetal Outcomes in Women With Confirmed aPL Positivity: Results From a Multicenter Study of 283 Pregnancies. Front Immunol 9 , 864 (2018). [3]E. Sevim et al. , Characteristics of Antiphospholipid Antibody Positive Patients in AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking. Arthritis Care &amp; Research n/a . Table 1. “Non-criteria” clinical manifestations present in the analyzed population. Clinical manifestation n (% ) Non-obstetric n = 49 Thrombocytopenia 34 (69.4) Coombs´ positivity 11 (22.4) Migraine 11 (22.4) Hemolytic anemia 5 (10.2) Brain MRI white matter lesions 5 (10.4) Raynaud´s phenomenon 5 (10.2) Superficial vein thrombosis 4 (8.1) Evans syndrome 4 (8.1) Amaurosis fugax 3 (6.1) APS nephropathy 2 (4.1) Livedo reticularis 1 (2.0) Livedoid vasculopathy 1 (2.0) Memory lapses 1 (2.0) Pseudo-multiple sclerosis 1 (2.0) Sensorineural hearing loss 1 (2.0) Splinter hemorrhages 1 (2.0) Obstetric n = 38 Two spontaneous abortions &lt;10 weeks 16 (42.1) Infertility 14 (36.8) ≥ 2 or more IVF failures 11 (28.9) Late IUGR (&gt;34 weeks) 7 (18.4) Premature birth between 34 and 37 weeks 7 (18.4) Placental ischemia 5 (13.1) Late preeclampsia (&gt;34 weeks) 3 (8.0) Placental abruption 2 (5.3) Placental hematoma 2 (5.3) Abbreviations –aCL: anticardiolipin antibodies; AI: Autoimmune; AID: Autoimmune disease; aPL: Antiphospholipid antibodies; IUGR: Intrauterine growth restriction; IVF: in vitro fertilization; LA: Lupus anticoagulant. Disclosure of Interests: None declared