J Rheum Dis.  2019 Jan;26(1):74-78. 10.4078/jrd.2019.26.1.74.

Catastrophic Antiphospholipid Syndrome Associated with Systemic Lupus Erythematosus Successfully Treated with Rituximab: A Case Report

• Shin J
• Kim KN

• KMID: 2442037
• DOI: http://doi.org/10.4078/jrd.2019.26.1.74

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.

Keyword

Antiphospholipid syndrome; Systemic lupus erythematosus; Rituximab

MeSH Terms

Adolescent
Adrenal Cortex Hormones
Antibodies, Antiphospholipid
Anticoagulants
Antiphospholipid Syndrome*
Clinical Protocols
Female
Follow-Up Studies
Humans
Immunoglobulins
Korea
Lupus Erythematosus, Systemic*
Mortality
Plasmapheresis
Rituximab*
Thrombosis
Adrenal Cortex Hormones
Antibodies, Antiphospholipid
Anticoagulants
Immunoglobulins
Rituximab

Figure

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  Figure 1 Contrast enhanced computed tomography angiography of lower extremity: multifocal stenosis at right distal common iliac artery, right upper superficial femoral artery, and left lower superficial femoral artery (white arrows).

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  Figure 2 Electrocardiography: ST elevation in lead II, III, aVF.

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  Figure 3 Contrast enhanced computed tomography of abdomen. (A) Hepatosplenomegaly. (B, C) Long segmental jejunal wall thickening (white arrows) with a large amount of ascites (black arrows).

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  Figure 4 Contrast enhanced computed tomography of chest. Low attenuation lesions considered as ischemic changes around the left ventricle (gray arrows) with a large amount of pleural fluid (white arrows).

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