Antiphospholipid syndrome

EDITORIAL

Antiphospholipid syndrome

The antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by arterial and venous thrombosis, gestational morbidity and presence of elevated and persistently positive levels of antiphospholipid antibodies.¹

The treatment of APS is controversial, especially because of the absence of good quality clinical studies. In the primary prevention of thrombosis, clinical studies report different results.^2,3 The association of risk factors with thrombotic events and the type and number of positive antiphospholipid antibodies are current concepts and should be considered in the therapeutic decision, be it medicamentous or a change in lifestyle.

Full anticoagulation for undetermined time is indicated for secondary prevention, but the therapeutic target is still discussed. Although retrospective studies have suggested a lower number of recurrences with an elevated international normalized ratio (INR), prospective studies do not corroborate such findings.^4,5 However, the inclusion of patients with arterial thrombosis in those protocols was small, making the definitive conclusion about the target INR in APS difficult.

The obstetric questions have also generated discussion: teratogenicity associated with warfarin⁶ and the dosing of heparin in patients with vascular APS have little scientific evidence.⁷

International efforts aimed at designing and conducting good quality prospective studies for patients with antiphospholipid antibodies, such as the creation of a multicenter data bank, have been observed.⁸ Thus, new perspectives arise and, in the near future, we will have answers based on better evidence regarding the treatment of APS, including the use of hydroxychloroquine for primary prevention, the use of new anticoagulants, and biologic therapy.

In face of the need to improve understanding and treatment, and to establish recommendations for rheumatologists and other specialists about the management of APS, the Committee of Vasculopathies and APS of the Brazilian Society of Rheumatology (SBR), with the support of the Brazilian Medical Association (AMB), publishes this guideline, elaborated from nine relevant and controversial clinical questions related to the treatment of APS, based on the best scientific evidence available.

Once finished this first publication, our committee proceeds to the objectives established for the next two years: elaboration and publication of guidelines for the diagnosis of APS and elaboration and publication of guidelines and manuals for patients with Granulomatosis with Polyangiitis and Takayasu's Artheritis. In addition, the committee has been working along with AMB to include the following tests in the table of procedures: IgM/IgG anti-beta 2-glycoprotein I test; IgA anticardiolipin test; antiproteinase 3 test; and antimyeloperoxidase test.

Adriana Danowski

Hospital Federal dos Servidores do Estado (HFSE),

Rio de Janeiro, RJ, Brazil

E-mail: [e-mail]

Roger A. Levy

Universidade do Estado do Rio de Janeiro (UERJ),

Rio de Janeiro, RJ, Brazil

REFERENCES

• ¹
  Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera R, et al. International consensus statement on an update to the classification for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006;4:295-306.
• ²
  Tarr T, Lakos G, Bhattoa HP, Shoenfeld Y, Szegedi G, Kiss E. Analysis of risk factors for the development of thrombotic complications in antiphospholipid antibody positive lupus patients. Lupus. 2007;16:39-45.
• ³
  Erkan D, Harrison MJ, Levy RA, Peterson M, Petri M, Sammaritano L, et al. Aspirin for primary thrombosis prevention in the antiphospholipid syndrome: a randomized, double-blind, placebo-controlled trial in asymptomatic antiphospholipid antibodypositive individuals. Arthritis Rheum. 2007;56:2382-91.
• ⁴
  Finazzi G, Marchioli R, Brancaccio V, Schinco P, Wisloff F, Musial J, et al. A randomized clinical trial of high-intensity warfarin vs. conventional antithrombotic therapy for the prevention of recurrent thrombosis in patients with the antiphospholipid syndrome (WAPS). J Thromb Haemost. 2005;3:848-53.
• ⁵
  Crowther MA, Ginsberg JS, Julian J, Denburg J, Hirsh J, Douketis J, et al. A comparison of two intensities of warfarin for the prevention of recurrent thrombosis in patients with the antiphospholipid antibody syndrome. N Engl J Med. 2003;349:1133-8.
• ⁶
  Levy RA, Jesús GR, Jesús NR. Obstetric antiphospholipid syndrome: still a challenge. Lupus. 2010;19:457-9.
• ⁷
  Hunt BJ, Gattens M, Khamashta M, Nelson-Piercy C, Almeida A. Thromboprophylaxis with unmonitored intermediate-dose low molecular weight heparin in pregnancies with a previous arterial or venous thrombotic event. Blood Coagul Fibrinolysis. 2003;14:735-9.
• ⁸
  APS ACTION - AntiPhospholipid Syndrome Alliance For Clinical Trials and InternatiOnal Networking. D Erkan, MD Lockshin on behalf of APS ACTION members. Lupus. 2012;21:695-8.

Publication Dates

• Publication in this collection
  30 Oct 2015
• Date of issue
  Apr 2013