Immunosuppressive Treatment in Antiphospholipid Syndrome: Is It Worth It?

Abstract

The antiphospholipid syndrome (APS) is characterized by the development of venous and/or arterial thrombosis and pregnancy morbidity in patients with persistent antiphospholipid antibodies (aPL). Catastrophic antiphospholipid syndrome (CAPS) is a life-threatening form of APS occurring in about 1% of cases. Lifelong anticoagulation with vitamin K antagonists remains the cornerstone of the therapy for thrombotic APS, but frequently the use of anticoagulation may be problematic due to the increased risk of bleeding, drug interactions, or comorbidities. Immunosuppressant drugs are widely used to treat several autoimmune conditions, in which their safety and effectiveness have been largely demonstrated. Similar evidence in the treatment of primary APS is limited to case reports or case series, and studies on a large scale lack. Immunomodulatory drugs may be an emerging tool in managing such particular situations, like refractory obstetrical complications, CAPS, or so-called APS non-criteria manifestations. In addition, immunomodulatory drugs may be useful in patients experiencing recurrent thromboembolic events despite optimized anticoagulant therapy. We did a comprehensive review of literature analyzing the possible role of immunomodulation in primary APS to provide a broad overview of potentially safe and effective target treatments for managing this devastating disease.

Antisynthetase syndrome with rare EJ‐1 antibodies with antiphospholipid syndrome

“…However, it should be noted that APLS has been described in patients with coeliac disease, and antiphospholipid titres in coeliac patients are significantly higher than control populations. 23,24 Although repeat testing for APLS antibodies in our case was negative, treatment with rituximab has been demonstrated to normalize APLS antibody titres and there is evidence to suggest correlation with clinical [25][26][27] Pulmonary embolism or APLS should therefore be considered amongst ASS patients given it can be treated. This was especially important in our case, as our patient already had poor prognostic factors due to EJ-1 antibody positivity and cardiac comorbidities.…”

“… 23 , 24 Although repeat testing for APLS antibodies in our case was negative, treatment with rituximab has been demonstrated to normalize APLS antibody titres and there is evidence to suggest correlation with clinical improvement. 25 , 26 , 27 Pulmonary embolism or APLS should therefore be considered amongst ASS patients given it can be treated. This was especially important in our case, as our patient already had poor prognostic factors due to EJ‐1 antibody positivity and cardiac comorbidities.…”

Disability and cognitive impairment are interdependent in primary antiphospholipid syndrome

“…However, in APS, the use of these drugs is limited to case series and there is a lack of controlled clinical studies. 46 Based on our findings, it is necessary the development of controlled clinical trials with immunomodulatory drugs to achieve greater benefit for APS patients.…”

The challenges in diagnosing pediatric primary antiphospholipid syndrome

“…23 However, particularly non-thrombotic aPL-related clinical problems require immunosuppressive treatment along with anticoagulation. [23][24][25] The combination of anticoagulation, corticosteroids, and plasma exchange is considered the gold standard treatment for adult CAPS patients based on the data from the CAPS Registry. 26,27 Based on these data, the SHARE Initiative recommends also the same triple therapy with or without IVIG for pediatric APS patients.…”