[Antiphospholipid syndrome (APS) primary (PAPS) and secondary (SAPS)]

Area of Science:

• Rheumatology and Immunology
• Clinical Epidemiology
• Autoimmune Diseases

Context:

• Antiphospholipid syndrome (APS) presents with diverse clinical and serological manifestations.
• Understanding the prevalence and specific associations of clinical features with autoantibody-cofactor systems in APS requires further epidemiological study.
• Distinguishing between primary APS (PAPS) and secondary APS (SAPS) in patients with systemic lupus erythematosus (SLE) is crucial for management.

Purpose:

• To evaluate the frequency of clinical and serological symptoms of SLE and APS in patients with PAPS and SAPS.
• To compare the epidemiological, clinical, and serological profiles of PAPS and SAPS cohorts.

Summary:

• A cohort study analyzed 78 SAPS patients (meeting ARA criteria for SLE) and 43 PAPS patients (meeting Sapporo criteria for APS).
• PAPS patients were younger at disease onset, with shorter diagnostic and observation times compared to SAPS patients.
• SAPS patients displayed a wider range of SLE clinical criteria, recurrent miscarriages, livedo reticularis, and anti-Annexin V antibodies. PAPS patients showed higher frequencies of pulmonary embolism, deep venous thrombosis, IgM aCL, LAC, anti-beta2GP1, and anti-oxyLDL antibodies. Arterial thrombosis, migraine, crural ulceration, thrombocytopenia, and VDRL positivity were similarly prevalent in both groups.

Impact:

• This study highlights distinct clinical and serological patterns differentiating PAPS and SAPS, aiding in diagnosis and patient stratification.
• Findings underscore the need for larger sample sizes and future research to precisely define the relationship between specific clinical symptoms and antibody-cofactor systems in APS.
• The results contribute to a better understanding of the heterogeneity within antiphospholipid syndrome, particularly in the context of SLE.