Antiphospholipid Antibody Syndrome: Much Remains to be Learned

Limited Disease Awareness

Doruk Erkan, MD, MPH, attending physician at the Hospital for Special Surgery, New York, and associate professor of medicine at Weill Cornell Medicine, New York, explained one of the biggest problems associated with APS is limited disease awareness. This causes patient frustration, missed diagnosis and overdiagnosis. He said APS is one of the few conditions in which both under and overdiagnosis are problems.

Limited awareness and missed signs and symptoms can lead to misdiagnosis. Over­diagnosis can result from overinterpretation of slightly elevated aPL tests. For example, a young patient with a clot may go to the emergency department and because APS is never under consideration, the antibody levels won’t be checked. A similar patient could enter the emergency department and undergo an antibody test that reveals levels one unit above normal. That patient would inaccurately be diagnosed with APS. Dr. Erkan thus detailed the importance of physician and patient education, and the necessity for an antiphospholipid antibody profile assessment and risk assessment.

He encouraged the audience to download a free book from iTunes that describes APS in detail, Antiphospholipid Syndrome.²

Dr. Erkan also emphasized the importance of collaboration among physicians and/or among organizations. One means of accomplishing this is the APS ACTION group he leads. This collaboration of 57 members from 33 international centers works on multiple projects, including a large registry of approximately 800 patients.

Dr. Erkan is co-primary investigator of a multidisciplinary international project developing rigorous new consensus-based classification criteria to identify patients with a high likelihood of having APS. The project is supported by the ACR and the European League Against Rheumatism (EULAR). The investigators presented its very early results in Chicago.³ The classification criteria can be used for research purposes but should have no impact on reimbursement.

Lastly, Dr. Erkan discussed the most challenging presentations of aPL-positive patients including microthrombotic APS, catastrophic APS and any major bleeding in anti-coagulated APS patients. He stressed the importance of timely multi­disciplinary decisions in these patients, reviewed some immunosuppressive agents for microthrombotic APS and introduced the most recent clinical practice guideline.

Off-Label Treatments

Thomas Lee Ortel, MD, PhD, chief, Division of Hematology, Department of Medicine at Duke University School of Medicine in Durham, North Carolina, explained that most of the treatment options for APS are off label. Research suggests, however, patients should receive three months of anti-coagulant therapy in response to venous thromboembolism provoked by a transient risk factor.⁴ This is because a decrease to one month of therapy is likely to increase recurrent venous thrombo­embolism without achieving a clinically important decrease in bleeding.