Diverse antiphospholipid syndromes and beyond: A multicenter comprehensive analysis from the Egyptian College of...

Antiphospholipid syndrome (APS) is a complex autoimmune thrombo-inflammatory disorder characterized by diverse clinical manifestations and heterogeneous antibody profiles. We aimed to characterize the demographic, clinical, laboratory features, and treatment patterns of primary (PAPS) and secondary antiphospholipid syndrome (SAPS) patients in Egypt.

A total of 510 APS patients were recruited from 14 rheumatology centers. Data on demographics, clinical manifestations, serologic findings, and medications were analyzed.

The cohort included 406 females and 104 males (mean age: 32.0 ± 8.7 years); 42.4% had PAPS and 57.6% SAPS. SAPS patients had an earlier disease onset (p = 0.008). Thrombotic events and pregnancy morbidity were more common in PAPS (40.7% and 83.8%) than SAPS (19% and 47.3%) (p < 0.0001). In contrast, mucocutaneous symptoms, arthritis, oral ulcers, gastrointestinal, pulmonary, renal, and cardiovascular involvement, as well as ANA, anti-dsDNA positivity, and complement consumption, were significantly more frequent in SAPS (p < 0.0001). Antiphospholipid (APL) antibodies positivity was more common in PAPS (p < 0.0001). Ocular and CNS manifestations, along with APL positivity, were notably higher in males (p = 0.002, p = 0.02, and p < 0.0001, respectively). A lupus like presentation was seen in 10.6% of PAPS, All ANA and anti dsDNA positive. Seronegative PAPS constituted 7.9% of cases and had higher thrombotic rates (p = 0.004).

PAPS and SAPS share several key features but exhibit clinical and serological profiles. Male APS patients more frequently present with ocular, and CNS involvement, and are more likely to be APL positive. Subsets of PAPS, including lupus like and seronegative forms, require further investigation.