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J Autoimmun. 2018 Jun;90:1-27. doi: 10.1016/j.jaut.2018.02.003. Epub 2018 Feb 13.

The treatment of anti-phospholipid syndrome: A comprehensive clinical approach.

Author information

1
Department of Clinical Sciences and Community Health, University of Milan, Via Festa del Perdono 7, 20122, Milan, Italy; Experimental Laboratory of Immunological and Rheumatologic Researches, IRCCS Istituto Auxologico Italiano, Via Zucchi 18, 20095 Cusano Milanino, Milan, Italy; Allergology, Clinical Immunology and Rheumatology, Piazzale Brescia 20, 20149, Milan, Italy. Electronic address: cecilia.chighizola@unimi.it.
2
Rheumatology and Clinical Immunology, Spedali Civili of Brescia, Department of Clinical and Experimental Sciences, University of Brescia, Piazzale Spedali Civili 1, 25123, Brescia, Italy.
3
Department of Clinical Sciences and Community Health, University of Milan, Via Festa del Perdono 7, 20122, Milan, Italy; Experimental Laboratory of Immunological and Rheumatologic Researches, IRCCS Istituto Auxologico Italiano, Via Zucchi 18, 20095 Cusano Milanino, Milan, Italy; Department of Rheumatology, ASST Istituto Gaetano Pini & CTO, Piazza Cardinal Ferrari, 1 20122, Milan, Italy.
4
Rheumatology Unit, Department of Medicine, University of Padua, Via Giustiniani 2, 35128, Padua, Italy.
5
Experimental Laboratory of Immunological and Rheumatologic Researches, IRCCS Istituto Auxologico Italiano, Via Zucchi 18, 20095 Cusano Milanino, Milan, Italy.

Abstract

Anti-phospholipid syndrome (APS) is an acquired pro-thrombotic autoimmune disease that predisposes to thrombotic events and/or obstetric complications, in the persistent presence of anti-phospholipid antibodies (aPL). Life long moderate-intensity anticoagulation is the option of choice for aPL-positive patients with a previous thrombosis; critical issues concern the management of those with a history of arterial event due to the high rate of recurrence. Alternatives comprise anti-platelet agents and high-intensity anticoagulation. Low dose aspirin (LDASA) and low molecular weight heparin provide the mainstay of the treatment of obstetric APS, allowing a birth rate in 70% of cases. The management of refractory APS, thrombotic as well as obstetric, is highly debated, but an increasing burden of evidence points towards the beneficial effects of multiple treatments. Similarly, a management envisaging multiple drugs (anticoagulation, steroids, plasma exchange and/or intravenous immunoglobulins) is the most effective approach in catastrophic APS. Asymptomatic aPL carriers are at higher risk of thrombotic and obstetric complications compared to the general population, thus potentially benefitting of a pharmacological intervention. LDASA and hydroxychloroquine can be considered as options, in particular in case of high risk aPL profile, concomitant cardiovascular risk factors or associated autoimmune disease. APS is apparently a simple condition, but its multifaceted nature requires a complex and tailored treatment.

KEYWORDS:

Anti-phospholipid antibodies; Anti-phospholipid syndrome; Asymptomatic carriers; Catastrophic anti-phospholipid syndrome; Obstetric complications; Thrombosis

PMID:
29449131
DOI:
10.1016/j.jaut.2018.02.003
[Indexed for MEDLINE]

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