Is there a role for immunosuppression in antiphospholipid syndrome?

Abstract

Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by thrombosis, pregnancy morbidity, or nonthrombotic manifestations in patients with persistently positive antiphospholipid antibodies (aPL). Conventional APS treatment focuses on antithrombotic strategies, which are usually ineffective for the microvascular and nonthrombotic manifestations of aPL. Using a case-based presentation, this review focuses on the role of immunosuppression in nonobstetric APS, including B-cell inhibition (rituximab, belimumab, and bortezomib), complement inhibition (eculizumab), mechanistic target of rapamycin inhibition (sirolimus), vascular endothelial cell modulation (defibrotide), statins, and traditional rheumatologic disease–modifying agents (hydroxychloroquine, mycophenolate mofetil, azathioprine, and cyclophosphamide).

16th International Congress on Antiphospholipid Antibodies Task Force Report on Catastrophic Antiphospholipid Syndrome

“…The management of CAPS, which is usually complicated due to the high risk of bleeding, is discussed above and more information can be found elsewhere. 6 , 26 The task force concluded that, besides the lack of controlled clinical trials, a major management unmet need is to better understand the role of immunosuppressive approach 27 in the management of microvascular and CAPS patients.…”

“…There have been reports of CD 20 specific B-cell inhibition, terminal (C5) complement inhibition, mechanistic target of rapamycin inhibition, vascular endothelial cell modulation, statins, and traditional immune-mediated disease–modifying agents (hydroxychloroquine, mycophenolate mofetil, azathioprine, and cyclophosphamide) use in the management of microvascular APS and/or CAPS patients. 27 However, we hope that in parallel to: a) our increased understanding of aPL-mediated mechanisms, additional immunosuppressive pathways will be investigated in CAPS, and b) the improvement in diagnostic tools, CAPS patients will be better subgrouped for more targeted medication choices.…”

The Proteasome Inhibitor Bortezomib Induces Apoptosis and Activation in Gel-Filtered Human Platelets

“…Several clinical trials on the efficacy of BTZ in other hematological diseases, such as acute lymphoid and myeloid leukemia, indolent B-cell non-Hodgkin lymphoma and diffuse large B-cell lymphoma, are ongoing [21]. The role of BTZ in immunosuppression has also been reported in patients with nonobstetric antiphospholipid syndrome [22].…”

Successful Treatment of Catastrophic Antiphospholipid Syndrome Using Rituximab: Case Report and Review of the Literature

“…However, both trials were stopped prematurely, the first one because only one patient could be enrolled, and the second one because of slow patient enrollment. Eculizumab may play a role in the treatment of CAPS patients who associate complement-related regulatory gene mutations, but this issue needs to be addressed in future studies [ 48 ].…”