Table Of ContentDoruk Erkan · Michael D. Lockshin
Editors
Antiphospholipid
Syndrome
Current Research Highlights
and Clinical Insights
123
Antiphospholipid Syndrome
Doruk Erkan • Michael D. Lockshin
Editors
Antiphospholipid Syndrome
Current Research Highlights
and Clinical Insights
Editors
Doruk Erkan, MD, MPH Michael D. Lockshin, MD
Associate Physician-Scientist Director
The Barbara Volcker Center for Women The Barbara Volcker Center for Women
and Rheumatic Diseases and Rheumatic Diseases
Associate Attending Physician Attending Physician
Hospital for Special Surgery Hospital for Special Surgery
Associate Professor of Medicine Professor of Medicine and
Weill Cornell Medicine Obstetrics-Gynecology
New York, NY, USA Weill Cornell Medicine
New York, NY, USA
ISBN 978-3-319-55440-2 ISBN 978-3-319-55442-6 (eBook)
DOI 10.1007/978-3-319-55442-6
Library of Congress Control Number: 2017935363
© Springer International Publishing AG 2017
This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of
the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation,
broadcasting, reproduction on microfilms or in any other physical way, and transmission or information
storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology
now known or hereafter developed.
The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication
does not imply, even in the absence of a specific statement, that such names are exempt from the relevant
protective laws and regulations and therefore free for general use.
The publisher, the authors and the editors are safe to assume that the advice and information in this book
are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the
editors give a warranty, express or implied, with respect to the material contained herein or for any errors
or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims
in published maps and institutional affiliations.
Printed on acid-free paper
This Springer imprint is published by Springer Nature
The registered company is Springer International Publishing AG
The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland
To:
ARIN, AYDIN, & RACHEL
(DE)
AMANDA & JANE
(MDL)
Preface
“Advancing the Field Together” – The Journey that started in planning for the 15th
International Congress on Antiphospholipid Antibodies continues with a State-of-
the-Art Antiphospholipid Syndrome book
The International Congress on Antiphospholipid Antibodies (aPL) takes place
every three years; its purpose is to discuss aPL and antiphospholipid syndrome
(APS) research and clinical care. The Congress is a venue in which scientists and
clinicians, from multiple disciplines, present and review the most recent innovative
and important research to improve understanding of the syndrome, prioritize
research questions, and set a roadmap for future research.
The 15th International Congress on aPL took place on September 21–24, 2016, in
North Cyprus, having been relocated from the original congress location of Istanbul,
Turkey1. The Congress offered a comprehensive program, including evidence-b ased
state-of-the art presentations from 70 internationally recognized physicians and scien-
tists. At the Congress, there were 24 main sessions including 4 from different task
forces, 147 oral and poster abstract presentations, 14 meet-the-e xpert sessions, 2 satel-
lite symposia, and 1 patient education workshop; the objectives of which were to
discuss the biology and new mechanisms of the disease, describe ongoing and planned
clinical trials, explore potential new treatments, and strengthen established and/or cre-
ate new international collaborations for both basic and clinical research in the field.
A novel aspect of the Congress was that multiple teams, chaired by Scientific
Planning Committee members, used evidence-based literature reviews and expert
discussions to answer specific predefined aPL/APS questions. These teams included
points of view from experts in rheumatology, hematology, cardiovascular medicine,
obstetrics, neurology, and immunology. The Scientific Planning Committee mem-
bers also chaired the congress sessions and supervised completion of the reports
that are the bases of the chapters of this book.
The journey that had started as a planning exercise for the 15th International
Congress on aPL did not stop in September 2016, when the Congress ended, but
1 www.apsistanbul2016.org.
vii
viii Preface
continues with this state-of-the-art book. This book is presented in six sections, each
section focusing on a different aspect of aPL/APS: history; basic science; clinical
and diagnostic features; current and future therapies; task forces; and patient educa-
tion. We want to thank all the authors who participated in this journey, and hope that
this book will add value to the field of thrombosis and APS.
New York, NY, USA Doruk Erkan*
Michael D. Lockshin**
* Congress Chair
** Congress Honorary Chair
Acknowledgment
We want to express our gratitude to:
Our Patients
The Authors
The Committee Members, Speakers, Registrants, Sponsors, and Grant Supporters
of the 15th International Congress on Antiphospholipid Antibodies
Doruk Erkan
Michael D. Lockshin
I want to express my personal gratitude to Dr Michael D. Lockshin for his
invaluable guidance and support over the years.
Doruk Erkan
ix
Contents
Part I Historical Aspects of Antiphospholipid Syndrome
1 History of Antiphospholipid Antibody .................................................. 3
Michael D. Lockshin and E. Nigel Harris
Part II Basic Science Aspects of Antiphospholipid Syndrome
2 Natural Proteins Involved in Antiphospholipid Syndrome ................. 15
Philip G. de Groot, Bas de Laat, Jacob Rand,
Panayiotis G. Vlachoyiannopoulos, Fatima El-Assaad,
Steven A. Krilis, and Bill Giannakopoulos
3 Origin of Antiphospholipid Antibodies ................................................. 29
Joyce Rauch, Miri Blank, Philip G. de Groot, Kathryn M. Dupnik,
Martin Alexander Kriegel, Jerrold S. Levine, Yehuda Shoenfeld,
and Rohan Willis
4 Recent Advances in Understanding of the Genetics
of Antiphospholipid Syndrome .............................................................. 53
Niti Goel and Thomas L. Ortel
5 Mechanisms of Antiphospholipid Antibody- Mediated
Thrombosis .............................................................................................. 77
Rohan Willis, Hannah Cohen, Ian Giles, Jason S. Knight,
Steven A. Krilis, Anisur Rahman, and Philip G. de Groot
6 Mechanisms of Antiphospholipid Antibody- Mediated
Pregnancy Morbidity .............................................................................. 117
Jane E. Salmon, Chieko Mineo, Ian Giles, Larry W. Chamley,
Pier Luigi Meroni, and Vikki M. Abrahams
xi
xii Contents
Part III Clinical and Diagnostic Aspects of Antiphospholipid Syndrome
7 Definition and Epidemiology of Antiphospholipid Syndrome ............ 147
Ozan Unlu, Vinicius Domingues, Guilherme Ramires de Jesús,
Stéphane Zuily, Gerard Espinosa, Ricard Cervera,
Roger Abramino Levy, Denis Wahl, Doruk Erkan,
and Michael D. Lockshin
8 Clinical and Prognostic Significance of Non- criteria
Antiphospholipid Antibody Tests ........................................................... 171
Maria Laura Bertolaccini, Olga Amengual, Bahar Artim-Eser,
Tatsuya Atsumi, Philip G. de Groot, Bas de Laat,
Katrien M.J. Devreese, Ian Giles, Pier Luigi Meroni,
Maria Orietta Borghi, Anisur Rahman, Jacob Rand,
Véronique Regnault, Rajesh Kumar, Angela Tincani,
Denis Wahl, Rohan Willis, Stéphane Zuily, and Giovanni Sanna
9 Disease and Risk Measurement Criteria
in Antiphospholipid Syndrome .............................................................. 189
Mary-Carmen Amigo, Kenji Oku, Savino Sciascia,
María Victoria Goycochea Robles, Stéphane Zuily,
Olga Amengual, Dario Roccatello, Kotaro Otomo, Tatsuya Atsumi,
Maria Laura Bertolaccini, and Denis Wahl
10 Neuropsychiatric Manifestations of Antiphospholipid Syndrome ..... 201
Cécile M. Yelnik, Simone Appenzeller, Giovanni Sanna,
Elizabeth Kozora, and Maria Laura Bertolaccini
Part IV Therapeutic Aspects of Antiphospholipid Syndrome
11 Prevention and Treatment of Thrombotic Antiphospholipid
Syndrome ................................................................................................. 223
Mark Crowther, Kimberly J. Legault, David A. Garcia,
Maria G. Tektonidou, Amaia Ugarte, Ian N. Bruce, Doruk Erkan,
and Guillermo Ruiz-Irastorza
12 Prevention and Treatment of Obstetric Antiphospholipid
Syndrome ................................................................................................. 235
Guilherme Ramires de Jesús, Karen J. Gibbins, Robert M. Silver,
and D. Ware Branch
13 Treatment of Non-criteria Manifestations in Antiphospholipid
Syndrome ................................................................................................. 247
Michelle Remião Ugolini-Lopes, Paulo Ricardo Criado,
Kurosh Parsi, Reyhan Diz Kucukkaya, Mary-Carmen Amigo,
Maria G. Tektonidou, and Danieli Andrade
