A Visual Guide to Scleroderma and Approach to Treatment Maureen D. Mayes Editor A Visual Guide to Scleroderma and Approach to Treatment ThiSisaFMBlankPage Maureen D. Mayes Editor A Visual Guide to Scleroderma and Approach to Treatment Editor MaureenD.Mayes,MD,MPH DivisionofRheumatologyandImmunogenetics UniversityofTexasHealthScienceCenter Houston,TX,USA ISBN978-1-4939-0979-7 ISBN978-1-4939-0980-3(eBook) DOI10.1007/978-1-4939-0980-3 SpringerNewYorkHeidelbergDordrechtLondon LibraryofCongressControlNumber:2014942076 ©SpringerScience+BusinessMediaNewYork2014 Thisworkissubjecttocopyright.AllrightsarereservedbythePublisher,whetherthewholeorpart of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation,broadcasting,reproductiononmicrofilmsorinanyotherphysicalway,andtransmissionor informationstorageandretrieval,electronicadaptation,computersoftware,orbysimilarordissimilar methodologynowknownorhereafterdeveloped.Exemptedfromthislegalreservationarebriefexcerpts inconnectionwithreviewsorscholarlyanalysisormaterialsuppliedspecificallyforthepurposeofbeing enteredandexecutedonacomputersystem,forexclusiveusebythepurchaserofthework.Duplication ofthispublicationorpartsthereofispermittedonlyundertheprovisionsoftheCopyrightLawofthe Publisher’s location, in its current version, and permission for use must always be obtained from Springer.PermissionsforusemaybeobtainedthroughRightsLinkattheCopyrightClearanceCenter. ViolationsareliabletoprosecutionundertherespectiveCopyrightLaw. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publicationdoesnotimply,evenintheabsenceofaspecificstatement,thatsuchnamesareexempt fromtherelevantprotectivelawsandregulationsandthereforefreeforgeneraluse. While the advice and information in this book are believed to be true and accurate at the date of publication,neithertheauthorsnortheeditorsnorthepublishercanacceptanylegalresponsibilityfor anyerrorsoromissionsthatmaybemade.Thepublishermakesnowarranty,expressorimplied,with respecttothematerialcontainedherein. Printedonacid-freepaper SpringerispartofSpringerScience+BusinessMedia(www.springer.com) This book is dedicated to our patients in the hope that it will result in early recognition, prompt therapy, and better outcomes. ThiSisaFMBlankPage Preface Althoughclinicalmanifestationsofsclerodermamakeitavery“visual”disease,the low prevalence (estimated to be approximately 1 in 4,000 US adults) means that many physicians, rheumatologists included, may fail to recognize the disease particularly in its early stages. In addition, scleroderma varies widely in severity sothatthesometimessubtlefindingsofmilddiseasemaybemissed. In addition, there is often confusion between localized scleroderma (morphea) which does not involve internal organs and systemic sclerosis (including limited anddiffusedisease)whichdoes.Thesimilarterminologyonlyservestocompound thisconfusion. This bookisintendedtobeaneasily accessibletoolfor residents,fellows, and practicing physicians to recognize and accurately categorize scleroderma in its diverse forms and multiple clinical manifestations. Chapter 4 on nailfold capillaroscopy is particularly relevant, since this technique is now included in the 2013ACR/EULARclassificationcriteriaforsystemicsclerosis. Several chapters also include therapeutic approaches. Although a detailed dis- cussion of treatment is beyond the scope of this book, our goal is to increase knowledgeofavailabletreatmentoptions. Houston,TX MaureenD.Mayes vii ThiSisaFMBlankPage Contents 1 BriefOverviewofScleroderma:LocalizedScleroderma andSystemicSclerosis(SSc). . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1 MaureenD.Mayes 2 LocalizedScleroderma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5 CarolynA.Bangert,AndrewKim,andHeidiJacobe 3 SkinManifestationsandMusculoskeletalDiseaseinSSc. . . . . . . . 23 JanetPopeandMaureenD.Mayes 4 NailfoldCapillaroscopy. . .. . . . . . .. . . . . . . .. . . . . . .. . . . . . . .. 37 MichaelHughesandArianeL.Herrick 5 IschemicDigitalUlcers. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 51 FredrickM.Wigley 6 LungInvolvementinSystemicSclerosis. . . . . . . . . . . . . . . . . . . . . 63 JohnT.Huggins,JamesG.Ravenel,andRichardM.Silver 7 GastrointestinalInvolvementinSystemicSclerosis. . . . . . . . . . . . . 77 DineshKhanna,JasonBaker,PhilipJ.Clements, andChristopherP.Denton 8 CalcinosisCutisinSystemicSclerosis. . . . . . . . . . . . . . . . . . . . . . . 89 GloriaA.SalazarandMaureenD.Mayes 9 SystemicSclerosisMimics. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 97 GloriaA.Salazar,VirginiaD.Steen,andMaureenD.Mayes Index. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 111 ix