Lupus Erythematosus

Peter H. Schur � Elena M. MassarottiEditors

Lupus Erythematosus

Clinical Evaluation and Treatment

EditorsPeter H. SchurDivision of Rheumatology,Immunology, AllergyBrigham and Women’s HospitalBoston, MA, USA

Elena M. MassarottiDivision of Rheumatology, Immunology, AllergyCenter for Clinical TherapeuticsBrigham and Women’s HospitalBoston, MA, USA

ISBN 978-1-4614-1188-8 ISBN 978-1-4614-1189-5 (eBook)DOI 10.1007/978-1-4614-1189-5Springer New York Heidelberg Dordrecht London

Library of Congress Control Number: 2012941092

© Springer Science+Business Media New York 2012This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, speci fi cally the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on micro fi lms 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. Exempted from this legal reservation are brief excerpts in connection with reviews or scholarly analysis or material supplied speci fi cally for the purpose of being entered and executed on a computer system, for exclusive use by the purchaser of the work. Duplication of this publication or parts thereof is permitted only under the provisions of the Copyright Law of the Publisher’s location, in its current version, and permission for use must always be obtained from Springer. Permissions for use may be obtained through RightsLink at the Copyright Clearance Center. Violations are liable to prosecution under the respective Copyright Law.The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a speci fi c statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. While the advice and information in this book are believed to be true and accurate at the date of publication, neither the authors nor the editors nor the publisher can accept any legal responsibility for any errors or omissions that may be made. The publisher makes no warranty, express or implied, with respect to the material contained herein.

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v

Preface

Most physicians, regardless of their area of expertise, will encounter a patient with lupus during the course of their careers, or must consider the diagnosis when evaluat-ing a patient with a particular complaint. Often considered the prototypic autoim-mune disease, it is characterized by protean manifestations and affects a wide range of organ systems. Despite the widespread availability of antinuclear antibody testing and other technological diagnostic advances, the diagnosis of lupus can be elusive, dif fi cult, and inexact. Treatment of the disease can also be challenging, and focused upon the immune system and the speci fi c organ system affected. Advances in immu-nology and biotechnology have led to a burgeoning world of new therapies in develop-ment that offer patients the real possibility of new therapies, and physicians and scientists insights into the pathogenesis of this complicated immunological disease.

This textbook summarizes the clinical aspects of lupus facing the general clini-cian in the twenty- fi rst century. The reader will fi nd introductory chapters regarding general diagnostic and treatment principles, followed by chapters addressing the lupus-speci fi c organ manifestations. Special topics regarding pregnancy and comor-bidities are also presented. All these chapters were written by highly experienced physicians with special expertise in lupus.

We hope you will fi nd this text to be a valuable, single source, “go-to” reference for the common and not so common problems affecting patients with lupus that you may confront in your practices.

Boston, MA, USA Peter H. Schur, M.D., F.A.C.P. Elena M. Massarotti, M.D.

vii

Contents

1 SLE Epidemiology: Epidemiologic Subtypes and Risk Factors for Development ....................................................... 1Julia F. Simard and Karen H. Costenbader

2 The Immunopathogenesis and Immunopathology of Systemic Lupus Erythematosus ....................................................... 13David S. Pisetsky

3 Diagnosing and Monitoring Lupus....................................................... 27Elena M. Massarotti and Peter H. Schur

4 Recommendations on How to Monitor the Patient with Systemic Lupus Erythematosus in the Clinic or at the Bedside ..................................................................................... 41Vivian P. Bykerk

5 The Treatment of Lupus: General Principles ...................................... 53Elena M. Massarotti and Peter H. Schur

6 Cutaneous Manifestations of Lupus Erythematosus .......................... 67Henry Townsend and Ruth Ann Vleugels

7 Musculoskeletal Manifestations of SLE ............................................... 95Simon M. Helfgott

8 Cardiac and Vascular Disease in SLE .................................................. 107Robert A. Sands

9 Pulmonary Manifestations of Systemic Lupus Erythematosus ............................................................................ 115Hilary J. Goldberg and Paul F. Dellaripa

10 Hematologic Manifestations of SLE ..................................................... 127Ami S. Bhatt and Nancy Berliner

viii Contents

11 Lupus Nephritis ...................................................................................... 141Mary Anne Dooley

12 Gastrointestinal Manifestations of Systemic Lupus Erythematosus ............................................................................ 153R.S. Kalman and J.L. Wolf

13 Neuropsychiatric Aspects of Lupus ...................................................... 169S. Khoshbin

14 Systemic Lupus Erythematosus and Pregnancy ................................. 183Bonnie L. Bermas

15 Antiphospholipid Syndrome ................................................................. 197Bonnie L. Bermas

16 Lupus-Like Syndromes Related to Drugs ............................................ 211Joseph F. Merola

17 New and Emerging Therapies ............................................................... 223Elena M. Massarotti

18 Co-morbidities in Systemic Lupus Erythematosus ............................. 229Mary Gayed, Chee-Seng Yee, Sasha Bernatsky, and Caroline Gordon

Index ................................................................................................................ 251

ix

Contributors

Nancy Berliner , M.D. Division of Hematology , Brigham and Women’s Hospital , Boston , MA , USA

Harvard Medical School , Boston , MA , USA

Bonnie L. Bermas , M.D. Division of Rheumatology, Director, Lupus Center , Brigham and Women’s Hospital , Boston , MA , USA

Harvard Medical School , Boston , MA , USA

Sasha Bernatsky , Ph.D. Royal Victoria Hospital , Montreal , QC , Canada

Ami S. Bhatt Harvard Medical School, Boston, MA, USA

Clinical Fellow in Hematology and Oncology, Brigham and Women’s Hospital and Dana Farber Cancer Institute, Boston, MA, USA

Vivian P. Bykerk , M.D. Hospital for Special Surgery , New York , NY , USA

Karen H. Costenbader , M.D., M.P.H. Lupus Center , Brigham and Women’s Hospital , Boston , MA , USA

Harvard Medical School , Boston , MA , USA

Paul F. Dellaripa , M.D. Division of Rheumatology, Brigham and Women’s Hospital , Boston , MA , USA

Interstitial Lung Disease (ILD) Clinic , Brigham and Women’s Hospital , Boston , MA , USA

Mary Anne Dooley , M.D., M.P.H. Division of Rheumatology and Immunology , University of North Carolina at Chapel Hill , Chapel Hill , NC , USA

Mary Gayed , M.B.Ch.B. Rheumatology Department, City Hospital , Sandwell and West Birmingham Hospitals, NHS Trust , Birmingham , UK

Hilary J. Goldberg , M.D., M.P.H. Division of Pulmonary and Critical Care Medicine , Brigham and Women’s Hospital , Boston , MA , USA

Harvard Medical School , Boston , MA , USA

x Contributors

Caroline Gordon , M.D. Rheumatology Department, City Hospital , Sandwell and West Birmingham Hospitals, NHS Trust , Birmingham , UK

Rheumatology Research Group, School of Immunity and Infection, College of Medical and Dental Sciences , University of Birmingham , Birmingham , UK

Simon Helfgott , M.D. Division of Rheumatology , Brigham and Women’s Hospital , Boston , MA , USA

Harvard Medical School , Boston , MA , USA

R. S. Kalman , M.D. Beth Israel Deaconess Medical Center , Boston , MA , USA

Harvard Medical School , Boston , MA , USA

S. Khoshbin , M.D. Division of Neurology, Brigham and Women’s Hospital, Boston , MA , USA

Harvard Medical School , Boston , MA , USA

Elena M. Massarotti , M.D. Division of Rheumatology, Center for Clinical Therapeutics, Brigham and Women’s Hospital , Boston , MA , USA

Harvard Medical School , Boston , MA , USA

Joseph F. Merola , M.D. Department of Medicine, Division of Rheumatology and Department of Dermatology , Brigham and Women’s Hospital , Boston , MA , USA

Department of Medicine , Harvard Medical School , Boston , MA , USA

David Pisetsky , M.D., Ph.D. Department of Medicine and Immunology , Duke University Medical Center , Durham , NC , USA

Medical Research Service , Durham VA Hospital , Durham , NC , USA

Robert A. Sands , M.D. Division of Rheumatology, Brigham and Women’s Hospital, Boston , MA , USA

Harvard Medical School , Boston , MA , USA

Peter H. Schur , M.D., F.A.C.P. Division of Rheumatology, Brigham and Women’s Hospital, Boston , MA , USA

Harvard Medical School , Boston , MA , USA

Julia F. Simard , Sc.D. Clinical Epidemiology Unit , Karolinska University Hospital , Stockholm , Sweden

Henry Townsend , M.D. Division of Clinical Immunology and Rheumatology, Department of Medicine , The University of Alabama at Birmingham , Birmingham , AL , USA

Ruth Ann Vleugels , M.D., M.P.H. Connective Tissue Disease Clinic , Brigham and Women’s Dermatology , Boston , MA , USA

Harvard Medical School , Boston , MA , USA

xiContributors

J. L. Wolf , M.D. Beth Israel Deaconess Medical Center, Boston , MA , USA

Harvard Medical School , Boston , MA , USA

Chee-Seng Yee , Ph.D. Rheumatology Department, City Hospital , Sandwell and West Birmingham Hospitals, NHS Trust , Birmingham , UK

Rheumatology Research Group, School of Immunity and Infection, College of Medical and Dental Sciences , University of Birmingham , Birmingham , UK