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A106: Rituximab Use in Pediatric Antiphospholipid Antibody Positive Patients
Author(s) -
Aguiar Cassyanne,
Lehman Thomas,
Erkan Doruk
Publication year - 2014
Publication title -
arthritis and rheumatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.106
H-Index - 314
eISSN - 2326-5205
pISSN - 2326-5191
DOI - 10.1002/art.38527
Subject(s) - rituximab , medicine , antibody , antiphospholipid syndrome , immunology
Background/Purpose: Rituximab (RTX) has been increasingly used in Antiphospholipid Syndrome (APS); our objective was to review the literature on the outcomes of RTX‐treated antiphospholipid antibody (aPL) positive pediatric patients. Methods: PubMed/Ovid were queried for “rituximab” and “pediatric” or “children” and “aPL”, “APS”, “lupus anticoagulant (LA)”, “anticardiolipin antibodies (aCL)” and/or “anti‐β 2 ‐Glycoprotein‐I antibodies (aβ 2 GPI)”. Information obtained was demographics, aPL/APS history, RTX indication/dose/duration, medications, and pre/post RTX aPL results. We systematically analyzed: a) clinical outcomes only in patients treated with RTX for aPL‐specific indications; and b) pre/post RTX aPL profiles in all patients. Results: As of Jan 2014, 14 articles and one abstract reported 23 aPL‐positive pediatric patients (≤18 years) treated with RTX (mean age: 13 ± 4.6 [3m−17 y]; Primary aPL/APS: 5 [22%]; aPL/APS with lupus 17 [74%], and malignancy 1 [4%]). 11/23 (48%) patients received RTX for an aPL‐related indication (recurrent thromboses/microthromboses: 6, thrombocytopenia: 7, hemolytic anemia: 2, skin ulcer/necrosis: 2, aPL‐nephropathy: 1); all patients had “improvement” and/or “no further thrombosis” as per authors' assessment. describes the pre/post RTX aPL profiles. Rituximab regimens were: 375 mg/m 2 weekly for 2–4 weeks (8), 1 gm 1–2 weeks apart for two doses (2), 750 mg/m 2 weekly for 2 weeks (5), other (5), and unknown (3). Medications most commonly used prior to RTX were cyclophosphamide (14), azathioprine (9), mycophenolate mofetil (9), and IVIG (8). Steroids were used in all cases with cyclophosphamide and anticoagulation concomitantly in 8 and 6 cases, respectively.Pre‐RTX aPL (N = 23) Post‐RTX aPL Positive_Negative_UnknownLAPositive 9 (2_0_7) Negative 4 (0_2_2) Unknown 10 (0_0_10)aCL IgG/M/APositive (≥ 20U) 17 (1_9_7) Positive (≥ 40U) 6 (0_4 _2) Negative (< 20U) 3 (0_0_3) Unknown 3 (0_0_3)aβ 2 GPI IgG/M/APositive (≥ 20U) 3 (1_0_2) Positive (≥ 40U) 0 (0_0_0) Negative (< 20U) 1 (0_1_0) Unknown 19 (0_0_19)(1) tested 2/3 aPL with 1/2 aPL tested twice (aCL repeat value reported “positive”) pre‐RTX; (2) tested 1/3 aPL with repeat testing showing change to negative before RTX; (1) tested 3/3 aPL with repeat aCL positivity (tested only 2 weeks apart).Conclusion: Our systematic analysis of the literature for the outcomes of RTX‐treated aPL‐positive pediatric patients demonstrate: a) the number of patients is small with heterogeneous clinical manifestations and aPL profile; b) patients had clinical improvement of the aPL‐related manifestation, however in all cases RTX was used concomitantly with other immununosuppressive medications, steroids and/or anticoagulation; c) no patient with a clinically significant aPL profile (LA and/or moderate‐to high titer aCL/aβ 2 GPI) switched to a negative aPL (LA, aCL, and aβ 2 2GPI) following treatment only with Rituximab.