Table 1.

Summary of reports of eculizumab in patients with antiphospholipid syndrome.

–	Patient	Prior therapies	Eculizumab dose/duration	Outcome
Shapira [55]	28 M with SLE, APS with PE, arterial thrombosis leading to leg amputation, and recurrent CAPS	Heparin, argatroban, fondaparinux, cyclophosphamide (CYC), steroids, IVIG, lepirudin, bivalirudin, aspirin, clopidogrel, plasma exchange (PEX)	Eculizumab 900 mg once, then 1200 mg biweekly for 1 y	Normalization of cytopenias and resolution of thrombotic events
Strakhan [43]	36 F with HTN, acute renal failure, stroke, acute coronary syndrome, and MAHA	PEX, steroids	Eculizumab 900mg weekly for 4 wk then 1200 mg biweekly	Gradual improvement of MAHA, continued dialysis
Appenzeller [56]	30 F with ITP and primary APS who developed CAPS after pregnancy	Hydroxychloroquine (HCQ), heparin, steroids, rituximab, PEX, immunoadsorption, dialysis	Eculizumab for 3 mo, mycophenylate, steroids	Resolution of thrombocytopenia and MAHA, with late partial relapse, dialysis dependent
Lonze [57]	2 patients with prior CAPS, undergoing renal transplant	Prednisone, rituximab, anticoagulation	Eculizumab, 900mg the day after transplant, then 1200 mg biweekly	Both with functioning allografts and no recurrence of thrombotic events, follow up 4 mo −4 y
Zikos [58]	47 M with primary APS, then CAPS with thrombocytopenia, multifocal thrombi including renal and hepatic infarcts	Heparin, PEX, IVIG, steroids, argatroban, heparin	Eculizumab 900mg weekly for 2 wk, then 1,200 mg q 7–10 d	Gradual improvement in cytopenias, ascites, and splenomegaly, no further thrombotic events for 16 mo of follow up, continued dialysis
Gustavsen [59]	22 F with arterial thrombosis and ischemic ulcerations during pregnancy	Warfarin, LMWH, aspirin	Eculizumab 600mg weekly for 2 wk in anticipation of Cesarean section	No further thrombosis, improvement in ischemic pain, no adverse fetal effects
Hussain [47]	18 F with triple positive APS with recurrent thrombosis, hepativ infarction, PE	enoxaparin, fondaparinux, apixaban, rivaroxaban, warfarin, aspirin and clopidogrel, HCQ, steroids, rituximab, PEX	Eculizumab 600mg weekly x4, then 900mg biweekly plus fondaparinux, aspirin, clopidogrel, hydroxychloroquine	No recurrence of thrombosis with 27 mo of follow up
Tinti [41]	54 F with primary APS with intestinal infarction, then CAPS with ischemic limb, acute bilateral effusions, acute decompensated heart failure, acute kidney injury	HCQ, steroids, warfarin, steroids, IVIG, PEX	Eculizumab 600mg weekly for 1 mo with steroids, warfarin, hydroxychloroquine	Rapid improvement in respiratory involvement, progressive improvement in thrombocytopenia, anemia, and serum creatinine, and normalization of C3 and C4 serum levels. No recurrence of thrombosis for 1 y
Chitalia [60]	Three cases of CAPS	All received steroids and anticoagulation, one additionally treated with rituximab, and one with PEX	Eculizumab dosing and regimen details not available	One with initital improvement in skin mottling and no further thrombosis but died during initial hospitalization (after 4 doses of eculizumab) due to septic shock; 1 discharged with maintenance eculizumab, length of follow up not available; 1 with resolution of CAPS and continues on maintenance eculizumab, length of follow up N/A
Guillot [42]	78 F with history of PE presents with CAPS with renal injury, hypertension and cerebral vascular lesions	Steroids, anticoagulation, PEX	Eculizumab 900mg weekly x 4, then 1200mg biweekly for 2 mo with steroids, anticoagulation and HCQ	No recurrence of CAPS in 12 mo of follow up
Chidharla [61]	64 F with triple positive APS develops acute encephalopathy and COVID associated CAPS with acute venous thrombosis, new ischemic stroke, adrenal hemorrhage	Dexamethasone, remdesivir, PEX, rituximab, IVIG	Eculizumab 900mg weekly x2	No recurrent thrombotic events at 1 mo follow up
Skoczynska [62]	35 F with SLE and CAPS with severe TMA and respiratory, circulatory and renal failure	PEX, steroids, anticoagulation, dialysis	Eculizumab 900mg weekly for 1 mo, then 1200mg biweekly for 8 mo	Improvement in neurological and circulatory function, ongoing dialysis dependence, remission of CAPS at 9 mo follow up
Ruffatti [63]	32 F with pregnancy-associated CAPS with renal failure, respiratory and circulatory failure, ischemic digits	Anticoagulation, steroids, PEX, IVIG	1200mg weekly x3 then 900mg weekly x 6	Rapid remission of CAPS symptoms
Nauseef [64]	54 M with APS and Factor II G20210A carrier, and steroid-refractory ITP, develops CAPS with bilateral adrenal hemorrhage, acute thrombocytopenia, IVC thrombus and PE, and ear cartilage thrombosis	Steroids, IVIG, rituximab, oseltamivir, romiplostim, anticoagulation, HCQ	Eculizumab 900mg weekly x 4, then 1200mg biweekly x 6 mo	Found to have heterozygous deletion in CFHR3-CFHR1, no recurrence of thrombosis 1 y after cessation of eculizumab
Kello [65]	Case series of 9 patients with SLE and/or APS; 6 APS and 7 SLE, 4 SLE and APS	All APS patients received steroids and anticoagulation, 5 additionally received PEX, 2 cyclophosphamide, 1 mycophenolate mofetil, and 1 rituximab	Eculizumab duration ranged 3–60 mo	No recurrences of TMA, variable disease-free follow up after discontinuation from 1–30 mo, 100% survival at 3 mo, 2 subsequent deaths related to discontuation of dialysis
Rovere-Querini [66]	33 F with FVL, primary triple positive APS, prior PE and 2 miscarriages develops CAPS with acute hemorrage at 30 + 6 wk gestation with hemolytic anemia, thrombocytopenia, acute kidney injury	Warfarin replaced by LMWH during pregnancy, aspirin, HCQ, rituximab	Eculizumab 600mg hospitalization day 7 and 14, cesarean section day 9 due to thrombocytopenia.	Mild transient thrombocytopenia and transient inhibition of CH50 with low C3 in the newborn, with no adverse effects and spontaneous normalization
Kronbichler [67]	30 F with IgA deficiency, ITP, splenectomy, APS, SLE with pregnancy-associated CAPS with cerebral, myocardial, renal and pulmonary involvement	Steroids, rituximab, PEX (severe intolerance), immunoadsorption, dialysis	Eculizumab 900mg weekly x4, then 1200mg biweekly for 3 mo	Resolution of TMA, lupus flare while on eculizumab
Wig [68]	43 F with primary APS with thrombotic and pregnancy morbidity, with subsequent CAPS with HTN, intracranial hemorrhage, and recurrent thrombosis despite closely monitored anticoagulation	steroids, CYC, PEX, rituximab, IVIG, anticoagulation	Eculizumab 900mg once then 1200mg weekly x 6 until resolution of thrombocytopenia, then tapered 1200mg every other week, 900mg every other week, 900mg monthly	Improvement in thrombocytopenia, recovered of renal function and cessation of dialysis, reduction in steroids, functional improvement following strokes and living independently