J Rheum Dis 2019; 26(1): 74-78  
Catastrophic Antiphospholipid Syndrome Associated with Systemic Lupus Erythematosus Successfully Treated with Rituximab: A Case Report
JinShik Shin, Kwang Nam Kim
Department of Pediatrics, Hallym University Sacred Heart Hospital, Anyang, Korea
Correspondence to: Kwang Nam Kim http://orcid.org/0000-0003-4024-5128 Department of Pediatrics, Hallym University Sacred Heart Hospital, 22 Gwanpyeong-ro 170beon-gil, Dongan-gu, Anyang 14068, Korea. E-mail:kwangnamkim@naver.com
Received: April 5, 2018; Revised: May 10, 2018; Accepted: May 28, 2018; Published online: January 1, 2019.
© Korean College of Rheumatology. All rights reserved.

This is a open Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
The catastrophic variant of antiphospholipid syndrome (APS) is a very rare and life-threatening condition of APS. This condition is characterized by thrombosis in multiple organs within a short period of time in the presence of positive antiphospholipid antibodies (aPL). Over the past few decades, considerable progress has been made in the treatment of patients with catastrophic APS; however, the mortality rate still remains very high. Although some cases of rituximab treatment in patients with catastrophic APS have been reported, there is no clear treatment protocol. A 14-year-old girl with systemic lupus erythematosus was diagnosed with catastrophic APS. She received several medications: corticosteroids, intravenous immunoglobulin, and plasmapheresis with anticoagulants. Unfortunately, she did not improve, and rituximab was started with four courses. After the rituximab treatment, she did not experience further thrombotic events during the follow up. This paper reports a pediatric case of catastrophic APS treated successfully with rituximab in Korea. 
Keywords: Antiphospholipid syndrome, Systemic lupus erythematosus, Rituximab


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