Aortitis in the setting of catastrophic antiphospholipid syndrome in a patient with systemic lupus erythematosus.

Lupus
Andrés González-GarcíaLuis Manzano

Abstract

Catastrophic antiphospholipid syndrome (CAPS) is a rare condition characterized by multiple thromboses affecting mainly small vessels in a short period of time in patients with antiphospholipid antibodies. A high suspicion index is mandatory in order to initiate rapidly aggressive immunomodulatory therapy to avoid a very poor prognosis. Systemic lupus erythematosus (SLE) is often associated with antiphospholipid syndrome, with a worse outcome when the catastrophic features occur. We report the case of a 64-year-old woman with a clinical debut of SLE who presented concomitantly with CAPS with several thrombosis affecting the kidney, spleen and bilateral limbs with blue toe syndrome in both legs. Furthermore, she presented with aortitis, with a malaise and myalgias and general syndrome (asthenia, hyporexia and mild weight loss). Fortunately, she had a good response to multi-target combination therapy (anticoagulants, corticosteroids, hydroxychloroquine, intravenous immunoglobulins, plasma exchange and rituximab). Here, we discuss the association between aortitis and CAPS secondary to SLE, and review the literature regarding similar conditions.

References

Jun 10, 2003·Clinical Reviews in Allergy & Immunology·Ronald A Asherson, Ricard Cervera
May 29, 2012·Lupus·R Cervera
Jun 20, 2013·Autoimmunity Reviews·Horacio BermanUNKNOWN Catastrophic Antiphospholipid Syndrome (CAPS) Registry Project Group (European Forum on Antiphospholipid Antibodies)
Mar 20, 2016·Seminars in Arthritis and Rheumatism·Chrong-Reen Wang, Ming-Fei Liu
Apr 17, 2018·Rheumatology·Ignasi Rodríguez-PintóUNKNOWN CAPS Registry Project Group
May 24, 2018·The New England Journal of Medicine·David Garcia, Doruk Erkan

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Methods Mentioned

BETA
electrophoresis
biopsy

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