Antiphospholipid syndrome: Clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients
Ricard Cervera,J.C. Piette,Josep Font,Munther A. Khamashta,Yehuda Shoenfeld,María Teresa Camps,Søren Jacobsen,Gabriella Lakos,Angela Tincani,Irene Kontopoulou-Griva,Mauro Galeazzi,Pier Luigi Meroni,Ronald H. W. M. Derksen,Philip G. de Groot,Erika Gromnica-Ihle,Marta Baleva,Marta Mosca,Stefano Bombardieri,Frédéric Houssiau,Jean-Christophe Gris,Isabelle Quéré,Eric Hachulla,Carlos Vasconcelos,B. Roch,Antonio Fernández-Nebro,Marie Claire Boffa,Graham R. V. Hughes,Miguel Ingelmo +27 more
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An association with SLE, the patient's sex, and the patient’s age at disease onset can modify the disease expression and define specific subsets of APS.Abstract:
Objective. To analyze the clinical and immunologic manifestations of antiphospholipid syndrome (APS) in a large cohort of patients and to define patterns of disease expression. Methods. The clinical and serologic features of APS (Sapporo preliminary criteria) in 1,000 patients from 13 European countries were analyzed using a computerized database. Results. The cohort consisted of 820 female patients (82.0%) and 180 male patients (18.0%) with a mean +/- SD age of 42 +/- 14 years at study entry. "Primary" APS was present in 53.1% of the patients; APS was associated with systemic lupus erythematosus (SLE) in 36.2%, with lupus-like syndrome in 5.0%, and with other diseases in 5.9%. A variety of thrombotic manifestations affecting the majority of organs were recorded. A catastrophic APS occurred in 0.8% of the patients. Patients with APS associated with SLE had more episodes of arthritis and livedo reticularis, and more frequently exhibited thrombocytopenia and leukopenia. Female patients had a higher frequency of arthritis, livedo reticularis, and migraine. Male patients had a higher frequency of myocardial infarction, epilepsy, and arterial thrombosis in the lower legs and feet. In 28 patients (2.8%), disease onset occurred before age 15; these patients had more episodes of chorea and jugular vein thrombosis than the remaining patients. In 127 patients (12.7%), disease onset occurred after age 50; most of these patients were men. These patients had a higher frequency of stroke and angina pectoris, but a lower frequency of livedo reticularis, than the remaining patients. Conclusion. APS may affect any organ of the body and display a broad spectrum of manifestations. An association with SLE, the patient's sex, and the patient's age at disease onset can modify the disease expression and define specific subsets of APS.read more
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International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS).
Spiros Miyakis,Michael D. Lockshin,Tatsuya Atsumi,D W Branch,Robin L. Brey,Ricard Cervera,R. H. W. M. Derksen,P. G. De Groot,Takao Koike,Pier Luigi Meroni,Guido Reber,Yehuda Shoenfeld,Angela Tincani,Panayiotis G. Vlachoyiannopoulos,Steven A. Krilis +14 more
TL;DR: This document appraise the existing evidence on clinical and laboratory features of APS addressed during the forum and proposes amendments to the Sapporo criteria, including definitions on features ofAPS that were not included in the updated criteria.
Journal ArticleDOI
The antiphospholipid syndrome.
TL;DR: The broad spectrum of renal diseases that have been observed in association with this syndrome are discussed, and the impact that APS may have on pre-existing renal disease as well as current recommendations for treatment of APS are discussed.
Journal ArticleDOI
2021 Guideline for the Prevention of Stroke in Patients With Stroke and Transient Ischemic Attack: A Guideline From the American Heart Association/American Stroke Association
Dawn Kleindorfer,Amytis Towfighi,Seemant Chaturvedi,Kevin M. Cockroft,Jose Gutierrez,Debbie Lombardi-Hill,Hooman Kamel,Walter N. Kernan,Steven J. Kittner,Enrique C. Leira,Olive Lennon,James F. Meschia,Thanh N. Nguyen,Peter M. Pollak,Pasquale Santangeli,Anjail Sharrief,Sidney C. Smith,Tanya N. Turan,Linda S. Williams +18 more
Journal ArticleDOI
Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines
Ronald A. Asherson,Ricard Cervera,P. G. De Groot,Doruk Erkan,M C Boffa,J.C. Piette,Munther A. Khamashta,Yehuda Shoenfeld +7 more
TL;DR: Anticoagulation, corticosteroids, plasma exchange, intravenous gammaglobulins and, if associated with lupus flare, cyclophosphamide, are the most commonly used treatments for catastrophic APS patients.
Journal ArticleDOI
Morbidity and mortality in the antiphospholipid syndrome during a 10-year period: a multicentre prospective study of 1000 patients
Ricard Cervera,Munther A. Khamashta,Y Shoenfeld,M. T. Camps,Søren Jacobsen,Emese Kiss,Margit Zeher,Angela Tincani,I. Kontopoulou-Griva,Mauro Galeazzi,Francesca Bellisai,Pier Luigi Meroni,R. H. W. M. Derksen,P. G. De Groot,Erika Gromnica-Ihle,Marta Baleva,Marta Mosca,Stefano Bombardieri,Frédéric Houssiau,Jean-Christophe Gris,Isabelle Quéré,Eric Hachulla,Carlos Vasconcelos,B. Roch,Antonio Fernández-Nebro,J.C. Piette,Gerard Espinosa,Silvia Bucciarelli,C. N. Pisoni,Maria Laura Bertolaccini,M. C. Boffa,G. R. V. Hughes +31 more
TL;DR: Patients with APS still develop significant morbidity and mortality despite current treatment, and it is imperative to increase the efforts in determining optimal prognostic markers and therapeutic measures to prevent these complications.
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