The most pressing treatment challenges in catastrophic APS include delay in diagnosis for the reasons discussed above, ongoing thrombosis despite anticoagulation, high risk of simultaneous thrombosis and bleeding, and high prevalence of accompanying comorbidities (such as sepsis) that directly affect the mortality.
SAN: How has the availability of the International CAPS Registry changed the understanding and management of catastrophic APS?
DR. ERKAN: Our current knowledge of catastrophic APS is mostly based on the international Web-based registry, coordinated by Dr. Cervera. Patients with a catastrophic APS diagnosis have been included in this registry since 2000 through published or voluntary physician reports. The registry, which can be acc essed freely contains clinical, laboratory, and therapeutic data on all reported cases of catastrophic APS, with approximately 300 patients as of May 2011; the most recent descriptive analysis of the registry reported by Dr. Cervera on behalf of the CAPS Registry group was published in April 2010 (Lupus 2010;19:412-8).
Because of the rarity of catastrophic APS, it is very difficult to study this life-threatening disease. The CAPS Registry is currently the only source that allows researchers to systematically analyze the demographic and clinical characteristics of these patients. Furthermore, there are no randomized, controlled trials evaluating the efficacy of various therapies, and the treatment outcome data are also based on the analysis of the registry. Thus, the registry has been crucial in our understanding as well as the management of catastrophic APS.
Dr. Erkan is an associate physician-scientist at the Barbara Volcker Center for Women and Rheumatic Diseases; assistant attending rheumatologist and clinical researcher at Hospital for Special Surgery; and assistant professor of medicine at Weill Cornell Medical College in New York. Dr. Erkan disclosed having a research grant from Genentech.
--Reported and written by Diana Mahoney