The presence of non-criteria manifestations negatively affects the prognosis of seronegative antiphospholipid syndrome patients: a multicenter study

• Published in: Arthritis research & therapy Vol. 24; no. 1; p. 9
• Main Authors: da Rosa, Gilberto Pires, Sousa-Pinto, Bernardo, Ferreira, Ester, Araújo, Olga, Barilaro, Giuseppe, Bettencourt, Paulo, Cervera, Ricard, Espinosa, Gerard
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 03.01.2022; BioMed Central; BMC
• Subjects: Antibodies; Antibodies, Antiphospholipid; Antiphospholipid antibodies; Antiphospholipid syndrome; Antiphospholipid Syndrome - complications; Antiphospholipid Syndrome - diagnosis; Arthritis; Autoimmune diseases; Blood clot; Care and treatment; Classification; Diagnosis; Disease; Female; Hospitals; Humans; Ischemia; Laboratories; Lupus Erythematosus, Systemic - complications; Lupus Erythematosus, Systemic - drug therapy; Medical prognosis; Miscarriage; Morbidity; Mutation; Non-criteria manifestations; Obstetrics; Patients; Pregnancy; Prevention; Prognosis; Proteins; Retrospective Studies; Risk factors; Seronegative; Single positive; Stroke; Testing; Thrombocytopenia; Thrombosis; Portugal; Non-criteria manifestations; Antiphospholipid antibodies; Seronegative; Single positive; Antiphospholipid syndrome
• ISSN: 1478-6362; 1478-6354; 1478-6362
• DOI: 10.1186/s13075-021-02702-9

Seronegative antiphospholipid syndrome (SN-APS) is often defined as the presence of APS criteria manifestations, negative antiphospholipid antibodies (aPL), and coexistence of APS non-criteria manifestations. Nevertheless, the impact of these non-criteria features is still unclear. On a different note, the relevance of one single aPL positive determination in patients with APS manifestations is another domain with limited evidence. We aim to compare the course of SN-APS and single-positive aPL (SP-aPL) patients with that of individuals with APS manifestations without non-criteria features/aPL positivity (controls). Retrospective analysis of patients with thrombosis/obstetric morbidity assessed in two European hospitals between 2005 and 2020. Patients were divided into SN-APS, SP-aPL, and control groups. Clinical characteristics, comorbidities, and therapies were compared. A total of 82 patients were included in the SN-APS group, 88 in the SP-aPL group, and 185 in the control group. In Cox regression model, SN-APS displayed more thrombosis recurrence than controls (HR 3.8, 95% CI 2.2-6.5, p < 0.001) even when adjusting for the presence of hereditary thrombophilia, systemic lupus erythematosus, or contraceptive hormonal treatment. In SP-aPL, the difference in thrombosis recurrence did not reach statistical significance (p = 0.078). Indefinite anticoagulation (p < 0.001 and p = 0.008, respectively) and vitamin K antagonist (VKA) use (p < 0.001 in both cases) were more common in SN-APS/SP-aPL. SN-APS displayed more thrombosis recurrence, indefinite anticoagulation, and VKA use than controls without non-criteria manifestations. The presence of such features in patients with thrombosis and negative aPL may negatively impact their clinical course.