Autoimmune Diseases Munther A. Khamashta • Manuel Ramos-Casals (Editors) Autoimmune Diseases Acute and Complex Situations Editors Munther A. Khamashta, MD, FRCP, PhD Manuel Ramos-Casals, MD, PhD Lupus Research Unit Department of Autoimmune Diseases The Rayne Institute Laboratory of Autoimmune Diseases St. Thomas’ Hospital Josep Font, Institut d’Investigacions London Biomèdiques August Pi i Sunyer (IDIBAPS) UK Hospital Clinic, Barcelona Spain ISBN 978-0-85729-357-2 e-ISBN 978-0-85729-358-9 DOI 10.1007/978-0-85729-358-9 Springer London Dordrecht Heidelberg New York British Library Cataloguing in Publication Data A catalogue record for this book is available from the British Library Library of Congress Control Number: 2011921945 © Springer-Verlag London Limited 2011 Whilst we have made considerable efforts to contact all holders of copyright material contained in this book, we may have failed to locate some of them. Should holders wish to contact the Publisher, we will be happy to come to some arrangement with them. Apart from any fair dealing for the purposes of research or private study, or criticism or review, as permit- ted under the Copyright, Designs and Patents Act 1988, this publication may only be reproduced, stored or transmitted, in any form or by any means, with the prior permission in writing of the publishers, or in the case of reprographic reproduction in accordance with the terms of licences issued by the Copyright Licensing Agency. Enquiries concerning reproduction outside those terms should be sent to the publishers. The use of registered names, trademarks, etc. in this publication does not imply, even in the absence of a specifc statement, that such names are exempt from the relevant laws and regulations and therefore free for general use. Product liability: The publisher can give no guarantee for information about drug dosage and application thereof contained in this book. In every individual case the respective user must check its accuracy by consulting other pharmaceutical literature. Cover design: eStudioCalamar, Figueres/Berlin Printed on acid-free paper Springer is part of Springer Science+Business Media (www.springer.com) This book is dedicated to the memory of our friend and inspirational leader, Dr. Josep Font. Contents 1 Assessment and Management of the Rheumatological Patient in the Critical Care Unit ....................................................................................... 1 David P. D’Cruz and Duncan L.A. Wyncoll 2 Life-Threatening Complications of Systemic Lupus Erythematosus .............. 9 Michelle Petri 3 Catastrophic Antiphospholipid Syndrome ......................................................... 19 Maria J. Cuadrado, Giovanni Sanna, Maria Laura Bertolaccini, and Munther A. Khamashta 4 Complex Situations in Rheumatoid Arthritis .................................................... 27 Miriam García-Arias, Gema Bonilla, Luis Gómez-Carreras, and Alejandro Balsa 5 Sjögren’s Syndrome: Beyond Sicca Involvement .............................................. 45 Manuel Ramos-Casals, Pilar Brito-Zerón, Albert Bové Boada, and Antoni Sisó-Almirall 6 Systemic Sclerosis: Severe Involvement of Internal Organs ............................. 67 Niamh P. Quillinan and Christopher P. Denton 7 Vasculitic Emergencies in Patients with Large-Vessel Vasculitis ..................... 89 Kathleen Maksimowicz-McKinnon and Gary S. Hoffman 8 Life-Threatening Presentations of ANCA-Associated Vasculitis ..................... 101 Duvuru Geetha and Philip Seo 9 Severe Polyarteritis Nodosa ................................................................................. 119 Loïc Guillevin and Gregory Pugnet 10 Life-Threatening Cryoglobulinemia ................................................................... 133 Soledad Retamozo, Cándido Díaz-Lagares, Xavier Bosch, Salvatore de Vita, and Manuel Ramos-Casals vii viii Contents 11 Behçet’s Syndrome: Clinical Presentations Affecting Prognosis and Survival ......................................................................................... 163 Emire Seyahi, Koray Tascilar, and Hasan Yazici 12 Myositis: When Weakness Can Kill .................................................................... 185 Patrick Gordon and Ingrid E. Lundberg 13 Complicated Sarcoidosis: Challenges in Dealing with Severe Manifestations .................................................................................. 201 Robert P. Baughman and Hilario Nunes 14 Complex Situations in Patients with Adult-Onset Still’s Disease ..................... 221 Petros V. Efthimiou, Manil Kukar, and Olga Petryna 15 Managing Acute and Complex Dermatological Situations ............................... 233 Eduardo Fonseca and Rosa M. Fernández-Torres 16 Life-Threatening Autoimmune Hematological Disorders ................................ 259 Emmanuel Andrès, Helen Fothergill, and Mustapha Mecili 17 Autoimmune Ear, Nose, and Throat Emergencies ............................................. 275 Aharon Kessel, Zahava Vadasz, and Elias Toubi 18 Ophthalmological Emergencies in Rheumatic and Autoimmune Diseases ................................................................................... 291 Edward Pringle and Miles Stanford 19 Emergencies for the Vascular Surgeon ............................................................... 315 Ashish S. Patel and Matthew Waltham 20 Complicated Pregnancies in Patients with Autoimmune Systemic Diseases .................................................................................................. 331 Guillermo Ruiz-Irastorza and Munther A. Khamashta 21 The Complex Management of Viral-Related Autoimmune Diseases ............... 345 Dimitrios Vassilopoulos and Spilios Manolakopoulos 22 The Role of Intravenous Immunoglobulins in the Management of Acute Complex Autoimmune Conditions ..................... 259 Rotem Kedar, Yehuda Shoenfeld, and Howard Amital 23 Life-Threatening Complications of Biological Therapies ................................. 375 Ana Campar and David A. Isenberg Index ............................................................................................................................ 405 Contributors Howard Amital (IDIBAPS), Hospital Clinic, Barcelona, Department of Medicine ‘B’, Chaim Sheba Spain Medical Centre, Tel-Hashomer, Israel Pilar Brito-Zerón Emmanuel Andrès Department of Autoimmune Diseases, Service de Médecine Interne, Diabète et Laboratory of Autoimmune Diseases Maladies Métaboliques, Clinique Médicale Josep Font, Institut d’Investigacions B, Hôpital Civil, Hôpitaux Universitaires Biomèdiques August Pi i Sunyer de Strasbourg, Strasbourg Cedex, (IDIBAPS), Hospital Clinic, Barcelona, France Spain Alejandro Balsa Ana Campar Rheumatology Unit, Hospital Universitario Internal Medicine Department, La Paz, Madrid, Spain Santo António Hospital, Porto, Portugal Robert P. Baughman Maria J. Cuadrado Department of Medicine, University of Lupus Unit, The Rayne Institute, Cincinnati Medical Center, Cincinnati, St. Thomas’ Hospital, London, UK OH, USA David P. D’Cruz Maria Laura Bertolaccini The Louise Coote Lupus Unit, Guys’ Lupus Unit, The Rayne Institute, and St. Thomas’ Hospital NHS Foundation St. Thomas’ Hospital, London, UK Trust, Gassiott House, St. Thomas’ Hospital, Gema Bonilla Lambeth Palace Road, London, UK Rheumatology Unit, Hospital Universitario Christopher P. Denton La Paz, Madrid, Spain Center for Rheumatology, Royal Free Xavier Bosch Campus, University College Medical Department of Internal Medicine, ICMD School, London, UK Hospital Clinic, Barcelona, Spain Cándido Díaz-Lagares Albert Bové Boada Laboratory of Autoimmune Diseases Department of Autoimmune Diseases, Josep Font, Institut d’Investigacions Laboratory of Autoimmune Diseases Biomèdiques August Pi i Sunyer Josep Font, Institut d’Investigacions (IDIBAPS), Hospital Clinic, Barcelona, Biomèdiques August Pi i Sunyer Spain ix