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Daniel J. Wallace - Lupus: The Essential Clinicians Guide

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One million people in the United States suffer from systemic lupus erythematosus (SLE or lupus), a chronic autoimmune disease that is potentially debilitating and sometimes fatal as the immune system attacks the bodys cells and tissue, resulting in inflammation and tissue damage. SLE can affect any part of the body, but most often damages the heart, joints, skin, lungs, blood vessels, liver, kidneys and nervous system. The course of the disease is unpredictable, with periods of illness alternating with remission. It can be treated symptomatically, mainly with corticosteroids and immunosuppressants, though there is currently no cure. The symptoms of lupus vary widely, come and go unpredictably, and often mimic or are mistaken for other illnesses. As a result, patients present with varied symptoms to different practitioners and diagnosis can be elusive, with patients sometimes suffering unexplained symptoms and untreated lupus for years. Hence, a practical guide to the diagnosis and treatment of lupus is a valuable resource to an array of clinicians and may help expedite earlier diagnosis and better patient care. Part of the Oxford American Rheumatology Library, this concise, authoritative pocketbook describes the pathophysiology of lupus, diagnostic strategies and tools, and current and emerging therapeutic options, focusing on direct clinical applications for busy healthcare professionals. With 20 new tables and the most up-to-date information on the diagnosis, treatment and management of the condition, this second edition fulfills a need for a concise, practical guide to Lupus for clinicians that distills the principal features of this complicated, often misunderstood disorder.

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OARL

OXFORD AMERICAN RHEUMATOLOGY LIBRARY

Lupus This material is not intended to be and should not be considered a - photo 1

Lupus

This material is not intended to be, and should not be considered, a substitute for medical or other professional advice. Treatment for the conditions described in this material is highly dependent on the individual circumstances. While this material is designed to offer accurate information with respect to the subject matter covered and to be current as of the time it was written, research and knowledge about medical and health issues are constantly evolving, and dose schedules for medications are being revised continually, with new side effects recognized and accounted for regularly. Readers must therefore always check the product information and clinical procedures with the most up-to-date published product information and data sheets provided by the manufacturers and the most recent codes of conduct and safety regulation. Oxford University Press and the authors make no representations or warranties to readers, express or implied, as to the accuracy or completeness of this material, including without limitation that they make no representations or warranties as to the accuracy or efficacy of the drug dosages mentioned in the material. The authors and the publishers do not accept, and expressly disclaim, any responsibility for any liability, loss, or risk that may be claimed or incurred as a consequence of the use and/or application of any of the contents of this material.

OARL

OXFORD AMERICAN RHEUMATOLOGY LIBRARY

Lupus The Essential Clinicians Guide 2nd Edition Daniel J Wallace MD - photo 2

Lupus

The Essential
Clinicians Guide

2nd Edition

Daniel J. Wallace, MD, FACP, FACR

Executive Series Editor,
Oxford American Rheumatology Library
Clinical Professor of Medicine
Division of Rheumatology
Cedars-Sinai Medical Center
David Geffen School of Medicine at UCLA
Los Angeles, California

Lupus The Essential Clinicians Guide - image 3

Lupus The Essential Clinicians Guide - image 4

Oxford University Press is a department of the University of Oxford.
It furthers the Universitys objective of excellence in research, scholarship,
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Published in the United States of America by
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Oxford University Press 2014

All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or by any means, without the prior permission in writing of Oxford University Press, or as expressly permitted by law, by license, or under terms agreed with the appropriate reproduction rights organization. Inquiries concerning reproduction outside the scope of the above should be sent to the Rights Department, Oxford University Press, at the address above.

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Library of Congress Cataloging-in-Publication Data
Wallace, Daniel J. (Daniel Jeffrey), 1949 author.
Lupus: the essential clinicians guide/Daniel J. Wallace.2nd edition.

p.; cm.(Oxford American rheumatology library)

Includes bibliographical references and index.
ISBN 978-0-19-936196-0 (alk. paper)
I. Title. II. Series: Oxford American rheumatology library.
[DNLM: 1. Lupus Erythematosus, Systemic. WD 380]
RC312.5.S5
616.772dc23
2013031123

9 8 7 6 5 4 3 2 1
Printed in the United States of America
on acid-free paper

Contents
Chapter 1
The History of Lupus

Lupus (meaning wolf in Latin) was a Roman family name, and there was a St. Lupus who lived in central France in about A.D. 600. Moritz Kaposi (for whom Kaposis sarcoma is named) first used the description discoid to describe cutaneous lupus in the 1860s.

Credit for describing disseminated, or systemic, lupus and connecting its rashes with organ involvement goes to Sir William Osler (18491919), who initially described our current concepts of the clinical aspects of the disease in three long articles published between 1872 and 1895. Between 1900 and 1950, pathological descriptions of cardiac, pulmonary, and central nervous system involvement increased our general knowledge.

In 1941, Paul Klemperer coined the term collagen vascular disorders to apply to a group of what are now known to be autoimmune conditions. This led to initiatives to classify this family of disorders. The first criteria for systemic lupus erythematosus (SLE) were published in 1971, followed by revisions in 1981 and 1997, and a new classification in 2012. Other related variants of lupus, such as drug-induced lupus (1945), neonatal lupus (which is not actually lupus), overlap syndromes, mixed connective tissue disease, antiphospholipid syndrome (1983), and incomplete forms of lupus-like inflammation (undifferentiated connective tissue disease), followed. By the late 1970s, a nosological compilation of cutaneous manifestations of lupus was put together by James Gilliam.

Although a few cases of false-positive syphilis serologies were reported as early as 1909 in lupus patients, and hypergammaglobulinemia associated with lupus in 1943, the single major advance in identifying lupus by blood testing was published in 1949. Malcolm Hargraves, a hematologist at the Mayo Clinic, found globular antibodies taking purple stain in the marrow aspirate of a child with undiagnosed disease. The discovery of the LE cell greatly advanced the field, as it became possible for the first time for a blood test to be used to diagnose lupus, and biopsies were no longer mandatory. The LE cell turned out to be a DNA-histone nucleoprotein. Efforts to improve identification of protein markers in lupus resulted in antinuclear antibody and anti-DNA testing becoming available in the late 1950s. (Advances are summarized in .) Insights into the role of complement in inflammation and advances in immune techniques allowed anti-Sm, anti-RNP, anti-ENA, anti-Ro (SSA), and anti-La (SSB) to be identified and elucidated in the 1960s.

Figure 11 The first modern illustration of cutaneous lupus labeled lupus - photo 5

Figure 1.1 The first modern illustration of cutaneous lupus, labeled lupus erythemateux (1856). Source: Wallace DJ, Lyon I. Pierre Cazenave and the first detailed modern description of lupus erythemotosus. Semin Arthritis Rheum. 1999;28:305313. Reprinted with permission, Elsevier, 1999.

Table 1.1 First descriptions of components of lupus

Description

Year

Author

Butterfly rash

1845

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