The antiphospholipid-antibody syndrome is being diagnosed with increasing frequency in patients with thromboembolic disorders, pregnancy loss, or thrombocytopenia. Because it has only recently been well described, the diagnostic criteria for this syndrome and its optimal management are ill defined. Hughes Syndrome: Antiphospholipid Syndrome is a comprehensive overview of the current state of knowledge about this disorder. The title of the book may pose a problem, however, because many hematologists will not recognize ``Hughes syndrome'' as the antiphospholipid-antibody syndrome.
This multiauthored book contains chapters by experts in the field. The book is broadly divided into sections dealing with basic and clinical aspects of the syndrome and covers known and suspected complications of the disorder, including renal, pulmonary, hematologic, and nervous system manifestations. In general, the book is well structured, well written, and comprehensively referenced, and specific topics can easily be found through either the index or the table of contents.
The chapters on the differential diagnosis and clinical presentations of this disorder are well written and will be of particular interest to clinicians. The chapters on therapy review the data that define the current treatment of patients with antiphospholipid antibodies and provide specific recommendations. These chapters would have been improved by critical examination of the studies on which the treatment recommendations are based and by the inclusion of a system for rating the strength of the evidence supporting the recommendations. A notable omission is the results of a study by Laskin and associates (``Prednisone and Aspirin in Women with Autoantibodies and Unexplained Recurrent Fetal Loss.'' New England Journal of Medicine 1997;337:148-53), a double-blind, randomized trial that provided evidence of the ineffectiveness of corticosteroids combined with aspirin for the prevention of recurrent pregnancy loss in patients with antiphospholipid antibodies.
Although some chapters might be considered esoteric (for example, ``The Ear and Antiphospholipid Syndrome''), the inclusion of such chapters points to the varied clinical manifestations of the disorder. These chapters are intriguing and may help guide the care of patients with antiphospholipid antibodies and unusual clinical manifestations. The author of the chapter that deals with recurrent thrombosis in the antiphospholipid-antibody syndrome concludes that patients with a history of arterial thrombosis should be treated with warfarin to achieve an international normalized ratio of at least 3.0, but is careful to point out that the risks and benefits of this therapy have not been adequately evaluated.
Two chapters merit special mention. One deals with the differential diagnosis of the antiphospholipid-antibody syndrome and will be particularly useful to clinicians trying to understand the clinical importance and manifestations of this disorder. The chapter on prognosis highlights many unanswered questions. It may motivate investigators to design and perform methodologically rigorous investigations addressing some of these questions.
Undoubtedly, as properly designed clinical trials and basic research shed light on the syndrome, future editions of the book will be more useful to practicing clinicians, as evidence-based treatment guidelines become available. The prediction in the prologue -- that antiphospholipid-antibody syndrome will be ``one of the most common and important autoimmune diseases'' -- is provocative and remains to be verified.
Jeff Ginsberg, M.D.
Copyright © 2001 Massachusetts Medical Society. All rights reserved. The New England Journal of Medicine is a registered trademark of the MMS.
