Background/Purpose: Non-criteria manifestations occur in 24% in Primary antiphospholipid syndrome (PAPS) . The efficacy of immunosuppressants in patients with above condition is not certain due to paucity of evidence. This stuyd aims to evaluate the outcome of patients who have been treated with immunosuppressive drugs for treatment of non-criteria manifestations of PAPS.

Methods: Patients with the diagnosis of APS or probable APS, attending our clinics between 2010 to 2024 were screened.Among these those satisfying the APL domain of ACR/EULAR 2023 criteria and had at-least one non criteria manifestation not attributable to any other defined CTD or disease and have been treated with immunosuppressive therapy were included.Data regarding clinical presentation, laboratory abnormality, treatment and response were included. The primary outcome was the clinical improvement or resolution of the presenting manifestation after initiation of therapy.

Results: Of the 334 patients screened ,a total of 44 patients 28 females, mean age 38±13 (SD) years satisfied the inclusion criteria. The most common non-criteria manifestation was thrombocytopenia (24, 54 %) followed by AIHA (12,27%) (Table-1). Rituximab was the most common immunosuppressant used in 19(43%) followed by mycophenolate (18 (40%). 90% received antiplatelet and anticoagulants. During a median follow up of 32 months (IQR: 20-63), 80% patients responded to treatment. The best response was noted among patients with thrombocytopenia. . Response rates of all manifestations is depicted in Table 2. Major adverse events included death in 2 patients due to sepsis and pneumocystis carinii pneumonia, in patients receiving rituximab and 1 death due to sepsis, disseminated TB, Ca breast in patients receiving MMF

Conclusion: Among the patients with PAPS with non criteria manifestation, the best response to immunosuppression was observed in those presenting with AIHA and thrombocytopenia. . All manifestions except LAHPS and skin ulcers showed similar response with rituximab as well as mycophenolate/ azathioprine

Comparison of response to therapy in

patients receiving Rtx and mycophenolate/

azathioprine

ACR Meeting Abstracts - https://acrabstracts.org/abstract/outcome-of-patients-with-non-criteria-manifestations-of-primary-antiphospholipid-syndrome-treated-with-immunosuppressants-a-single-centre-study/