Table 1.
Medication | Mechanism | Perspective |
---|---|---|
Eculizumab | Complement 5 inhibitor | For CAPS refractory to standard treatment, CAPS receiving kidney transplantation, acute TMA in patients with aPL-related nephropathy, APS during pregnancy, or pediatric CAPS (2, 33, 34) |
Rituximab | Type I anti-CD20 monoclonal antibody | For thrombocytopenia, hemolytic anemia, or other aPL-mediated hematological and microthrombotic manifestations or noncriteria manifestations; an alternative option for CAPS which is refractory to standard treatment, refractory obstetric APS, and pediatric CAPS (2, 33, 35) |
Obinutuzumab | Type II anti-CD20 monoclonal antibody (B-cell depletion mainly via DCD) | Alternative option for rituximab in APS (36) |
Belimumab | BAFF/Blys inhibitor | Potential treatment for aPL-positive patients, or primary APS with high thrombotic risk (37–39) |
Daratumumab | Anti-CD38 monoclonal antibody | Potential treatment for refractory APS (40, 41) |
Zanubrutinib | BTK inhibitor | Unclear, evidence still being collected |
Anti-TNF-α therapy | Anti-TNF-α monoclonal antibody: adalimumab, certolizumab | In refractory obstetric APS (42) |
CAPS, catastrophic antiphospholipid syndrome; TMA, thrombotic microangiopathy; APS, Antiphospholipid syndrome; DCD, direct cell death; BAFF/Blys, B cell activating factor/B-lymphocyte stimulator; BTK, bruton tyrosine kinase.