Browse the corpus

Welcome to the RheumatologY Section of MKSAP 191 In these pages, you will flnd updated information on approaches to the patient with rheumatologic disease, principles of therapeutics, rheumatoid arthritis, osteoarthritis, spondyloarthritis, systemic lupus erythematosus, systemic sclerosis, infectious arthritis, and other clinical challenges. A11 ofthese topics are uniquely focused on the needs ofgeneralists and subspecialists outside of rheumatologz. MKSAP 19 strives to provide the clinical knowledge its learners need to navigate their longitudinal learning paths. MKSAP 19's core content contains essential, newly researched information in 11 subspecialty areas of internal medicine-created by dozens of expert generalists and subspecialists. Development of MKSAP 19's syllabus and its 1200 all new peer-reviewed, psychometrically validated multiple-choice questions (MCQs) has been informed by ABIM Certification and Maintenance of Certification (MOC) requirements, emerging internal medicine knowledge, and our learners' feedback. MKSAP 19 contin- ues to include HighValue Core (HVC) recommendations and MCQs, based on the concept of balancing clinical benefit with costs and harms. Hospital-based internists can continue to trust that MKSAP's comprehensive hospitalist content, integrated throughout the syllabus, and hospitalist focused MCQs, specially designated with the blue hospitalist icon (El), continue to align with the ABIM's Focused Practice in Hospital Medicine MOC exam blueprint and enhance learning for hospital-based practitioners.

MKSAP 19 strives to provide the clinical knowledge its learners need to navigate their longitudinal learning paths. MKSAP 19's core content contains essential, newly researched information in 11 subspecialty areas of internal medicine-created by dozens of expert generalists and subspecialists. Development of MKSAP 19's syllabus and its 1200 all new peer-reviewed, psychometrically validated multiple-choice questions (MCQs) has been informed by ABIM Certification and Maintenance of Certification (MOC) requirements, emerging internal medicine knowledge, and our learners' feedback. MKSAP 19 contin- ues to include HighValue Core (HVC) recommendations and MCQs, based on the concept of balancing clinical benefit with costs and harms. Hospital-based internists can continue to trust that MKSAP's comprehensive hospitalist content, integrated throughout the syllabus, and hospitalist focused MCQs, specially designated with the blue hospitalist icon (El), continue to align with the ABIM's Focused Practice in Hospital Medicine MOC exam blueprint and enhance learning for hospital-based practitioners. More than ever before, MKSAP 19 Digital focuses on individualized learning and convenience. In addition to custom quizzes and interlinked questions and sy'labus sections, MKSAP 19 Digital's new learning dashboard enables users to create a self directed learning plan, with topic-specific links to resources within MKSAP and ACP Online. Multimedia formats, including whiteboard animations and clinical videos, will benefit our audiovisual learners, while MKSAP's Earn as You Go CME/MOC feature now allows subscribers to earn CME/MOC as they answer individual questions. In addition to Extension Questions and New Info Updates, MKSAP 19 Complete and Complete Green continue to offer Virtual Dx and Flashcards and now offer brand new enhancements: MKSAP Quick Qs, a set of concise questions mapped to high-frequency/high-importance areas of the ABIM blueprint mirroring boards-style MCQs, and an embedded digital version of Board Basics for easy access exam prep.

More than ever before, MKSAP 19 Digital focuses on individualized learning and convenience. In addition to custom quizzes and interlinked questions and sy'labus sections, MKSAP 19 Digital's new learning dashboard enables users to create a self directed learning plan, with topic-specific links to resources within MKSAP and ACP Online. Multimedia formats, including whiteboard animations and clinical videos, will benefit our audiovisual learners, while MKSAP's Earn as You Go CME/MOC feature now allows subscribers to earn CME/MOC as they answer individual questions. In addition to Extension Questions and New Info Updates, MKSAP 19 Complete and Complete Green continue to offer Virtual Dx and Flashcards and now offer brand new enhancements: MKSAP Quick Qs, a set of concise questions mapped to high-frequency/high-importance areas of the ABIM blueprint mirroring boards-style MCQs, and an embedded digital version of Board Basics for easy access exam prep. Language can be imprecise and imperfect, but MKSAP 19's Editors and contributors commit to using language and images that support ACP's commitment to being an anti-racist organization that supports diversity, equity, and inclusion through- out health care and health education. ACP also continues to ensure diversity among MKSAP's physician-contributors. When appropriate, the MKSAP Editors also rely on MKSAP 19 Digital's expanded use of multimedia enhancements, including video and audio, to explore and more fully explain issues surrounding the presentation of MKSAP 19 clinical content as it relates to race and ethnicity. MKSAP 19 users are encouraged to contact the Editors at mksap_editors6acponline.org to help us identifiz opportunities for improvement in this area. On behalf of the many internists and editorial staff who have helped us create our new edition, we are honored that you have chosen to use MKSAP 19 to meet your lifelong learning needs. Sincerely, Davoren Chick, MD, FACP Editor-in-Chief Senior Vice President Medical Educat ion Division American College of Physicians

\ I I \ \ : Rheumatology Committee Editor-in-Chief Michael Pillinger, MD, EACE Section Editor Davoren Chick, MD, FACP Professor of Medicine and Biochemistry and Molecular Senior Vice President. Medical Education Pharmacologz American College of Physicians Division of Rheumatologz Philadelphia. Pennsylvania NYU Grossman School of Medicine New York. New York Senior Deputy Editor Aryeh M. Abeles, MD Patrick C. Alguire, MD, EACP Adjunct Associate Professor American College of Physicians Division ol Rheumatologz Phi ladelphia. Pennsylvan ia NYU Grossman School of Medicine New York. New York Deputy Editor Gregory C. Gardner, MD, MACP Janet A. Jokela, MD, MPH, EACP Gilliland Henderson Professor of Medicine Professor & Head, Department of Medicine Division of Rheumatologz Acting Regional Dean University of Washington University of Illinois College of Medicine Seattle, Washington Urbana, Illinois Sharon L. Kolasinski, MD, FACP Professor of Clinical Medicine Division of Rheumatologz Rheumatology Medicine Reviewers University of Pennsylvania Perelman School of Medicine David Armstrong, DO, FACP Ph iladelphia, Pen nsylvania Fawad Aslam, MBBS, MSc, FACP Angelique Collamer, MD, FACP Susan Faye Kroop, MD Michael Guma, DO, FACP Associate Professor of Medicine Marie Kuchynski, MD Division of Rheumatologz Ashima Makol. MD Vanderbilt University Medical Center J. Suzanne Moore, MD, FACP Nashville, Tennessee Patricia J. Papadopoulos, MD Vikas Majithia, MD, MPH, FACP Avis Ware, MD, FACP Professor of Medicine Andrew L. Wong, MD, FACP Division Director and Fellowship Program Director Division of Rheumatologz Hospital Medicine Rheumatology University of Mississippi Medical Center Jackson, Mississippi Reviewers Bernard Hildebrand, MD, MA, FACP Bethany A. Marston, MD Julie Hildebrand, MD Division Director, Pediatric Rheumatologz Kam A. Newman, MD, FACP Program Director, Rheumatologz Fellowship Niluka Weerakoon, MD Associate Professor of Medicine and Pediatrics Pediatric Rheumatologr and Allergz/lmmunologz and Rheumatologr Divisions University of Rochester Rochester, New York

Committee Editor-in-Chief Michael Pillinger, MD, EACE Section Editor Davoren Chick, MD, FACP Professor of Medicine and Biochemistry and Molecular Senior Vice President. Medical Education Pharmacologz American College of Physicians Division of Rheumatologz Philadelphia. Pennsylvania NYU Grossman School of Medicine New York. New York Senior Deputy Editor Aryeh M. Abeles, MD Patrick C. Alguire, MD, EACP Adjunct Associate Professor American College of Physicians Division ol Rheumatologz Phi ladelphia. Pennsylvan ia NYU Grossman School of Medicine New York. New York Deputy Editor Gregory C. Gardner, MD, MACP Janet A. Jokela, MD, MPH, EACP Gilliland Henderson Professor of Medicine Professor & Head, Department of Medicine Division of Rheumatologz Acting Regional Dean University of Washington University of Illinois College of Medicine Seattle, Washington Urbana, Illinois Sharon L. Kolasinski, MD, FACP Professor of Clinical Medicine Division of Rheumatologz Rheumatology Medicine Reviewers University of Pennsylvania Perelman School of Medicine David Armstrong, DO, FACP Ph iladelphia, Pen nsylvania Fawad Aslam, MBBS, MSc, FACP Angelique Collamer, MD, FACP Susan Faye Kroop, MD Michael Guma, DO, FACP Associate Professor of Medicine Marie Kuchynski, MD Division of Rheumatologz Ashima Makol. MD Vanderbilt University Medical Center J. Suzanne Moore, MD, FACP Nashville, Tennessee Patricia J. Papadopoulos, MD Vikas Majithia, MD, MPH, FACP Avis Ware, MD, FACP Professor of Medicine Andrew L. Wong, MD, FACP Division Director and Fellowship Program Director Division of Rheumatologz Hospital Medicine Rheumatology University of Mississippi Medical Center Jackson, Mississippi Reviewers Bernard Hildebrand, MD, MA, FACP Bethany A. Marston, MD Julie Hildebrand, MD Division Director, Pediatric Rheumatologz Kam A. Newman, MD, FACP Program Director, Rheumatologz Fellowship Niluka Weerakoon, MD Associate Professor of Medicine and Pediatrics Pediatric Rheumatologr and Allergz/lmmunologz and Rheumatologr Divisions University of Rochester Rochester, New York llt

l '! \ Rheumatology ACP Editorial Staff Aryeh M. Abeles, MD Consultantship Suzanne Meyers, Medical Editor, Assessment and Education Bristol-Myers Squibb, Johnson & Johnson Programs Beclg Krumm, Director, Assessment and Education Programs Vikas Majithia, MD, MPH, FACP Jackie Twomey, Managing Editor, Assessment and Research G rants / Contracts Education Programs GlaxoSmithKline, Bristol-Myers Squibb, Janssen Consultonfship Novartis ACP PrincipalStaff Aduisory Board Davoren Chick, MD, FACP UCB Senior Vice President, Medicql Educqtion Bethany Marston, MD Tabassum Salam, MD, MBA, FACP Re search G rants / Co ntracts Vice President, Medical Educqtion Rheumatologr Research Foundation Margaret Wells, EdM Michael Pillinger, MD, FACP Vice President, Learning Assessment, Accreditation, Reseo rch Grants / Contracts ctnd Research Hikma Pharmaceuticals, Horizon Pharmaceuticals Patrick C. Alguire, MD, FACP Consultontships MKSAP Senior Deputy Editor Horizon Pharmaceuticals, Sobi, Ampel Biosciences BeckyKrumm Tabassum Salam, MD, MBA, FACP Director, Assessment and Education Programs Consultantship Johnson & Johnson Jackie Twomey Managing Editor

Rheumatology ACP Editorial Staff Aryeh M. Abeles, MD Consultantship Suzanne Meyers, Medical Editor, Assessment and Education Bristol-Myers Squibb, Johnson & Johnson Programs Beclg Krumm, Director, Assessment and Education Programs Vikas Majithia, MD, MPH, FACP Jackie Twomey, Managing Editor, Assessment and Research G rants / Contracts Education Programs GlaxoSmithKline, Bristol-Myers Squibb, Janssen Consultonfship Novartis ACP PrincipalStaff Aduisory Board Davoren Chick, MD, FACP UCB Senior Vice President, Medicql Educqtion Bethany Marston, MD Tabassum Salam, MD, MBA, FACP Re search G rants / Co ntracts Vice President, Medical Educqtion Rheumatologr Research Foundation Margaret Wells, EdM Michael Pillinger, MD, FACP Vice President, Learning Assessment, Accreditation, Reseo rch Grants / Contracts ctnd Research Hikma Pharmaceuticals, Horizon Pharmaceuticals Patrick C. Alguire, MD, FACP Consultontships MKSAP Senior Deputy Editor Horizon Pharmaceuticals, Sobi, Ampel Biosciences BeckyKrumm Tabassum Salam, MD, MBA, FACP Director, Assessment and Education Programs Consultantship Johnson & Johnson Jackie Twomey Managing Editor Iulia Nawrocki Acknowledgments Digital Con ten f Asso ciate / Edito r The American College of Physicians (ACP) gratefully acknowledges the special contributions to the development Linnea Donnarumma and production of the 19th edition of the Medical Senior Medical Editor Knowledge Self-Assessment Program' (Vt<SAp' 19) made Amanda Cowley by the lollowing people: Medical Editor Graphic Design: Barry Moshinski (Director, Graphic Sandy Crump Services), Raymond DeJohn (Designer, Graphic Services), Medical Editor Tom Malone (Print/Mail Production Manager, Graphic Services), Mike Ripca (Technical Administrator, Graphic Georgette Forgione Services). Medicol Editor P roduction / Systems: DanHoffmann (Vice President, Beth Goldner Information Technology), Scott Hurd (Manager, Content MedicalEditor (Senior Systems), Neil Kohl Architect), and Chris Suzanne Meyers Patterson (Senior Architect). liledical Editor MKSAP 19 Digital: Under the leadership of Steven Spadt Elise Paxson (Senior Vice President, Information Technology and MedicalEditor Chief Technology Officer), the development of the dig- ital version of MKSAP 19 was implemented by ACP's Chuck Graver Digital Products and Services Department, directed and Finance and Operations Administrqtor led by Brian Sweigard (Vice President, Digital Products Kimberly Kerns and Services). Other members of the team included Dan Administratiu e Coordinator Barron (Senior Web Application Developer/Architect), Callie Cramer (Data Visualization/Web Developer), Disclosures of relationships with any entity producing, Chris Forrest (Senior Web Application Developer), marketing, reselling, or distributing health care goods or Kathleen Hoover (Manager, User Interface Design and services consumed by, or used on, patients. Individuals not Development), Kara Regis (Director, Product Design listed below have nothing to disclose. and Development), Brad Lord (Senior Web Application

Iulia Nawrocki Acknowledgments Digital Con ten f Asso ciate / Edito r The American College of Physicians (ACP) gratefully acknowledges the special contributions to the development Linnea Donnarumma and production of the 19th edition of the Medical Senior Medical Editor Knowledge Self-Assessment Program' (Vt<SAp' 19) made Amanda Cowley by the lollowing people: Medical Editor Graphic Design: Barry Moshinski (Director, Graphic Sandy Crump Services), Raymond DeJohn (Designer, Graphic Services), Medical Editor Tom Malone (Print/Mail Production Manager, Graphic Services), Mike Ripca (Technical Administrator, Graphic Georgette Forgione Services). Medicol Editor P roduction / Systems: DanHoffmann (Vice President, Beth Goldner Information Technology), Scott Hurd (Manager, Content MedicalEditor (Senior Systems), Neil Kohl Architect), and Chris Suzanne Meyers Patterson (Senior Architect). liledical Editor MKSAP 19 Digital: Under the leadership of Steven Spadt Elise Paxson (Senior Vice President, Information Technology and MedicalEditor Chief Technology Officer), the development of the dig- ital version of MKSAP 19 was implemented by ACP's Chuck Graver Digital Products and Services Department, directed and Finance and Operations Administrqtor led by Brian Sweigard (Vice President, Digital Products Kimberly Kerns and Services). Other members of the team included Dan Administratiu e Coordinator Barron (Senior Web Application Developer/Architect), Callie Cramer (Data Visualization/Web Developer), Disclosures of relationships with any entity producing, Chris Forrest (Senior Web Application Developer), marketing, reselling, or distributing health care goods or Kathleen Hoover (Manager, User Interface Design and services consumed by, or used on, patients. Individuals not Development), Kara Regis (Director, Product Design listed below have nothing to disclose. and Development), Brad Lord (Senior Web Application tv

Developer/Architect), and John McKnight (Senior Web r Pass the ABIM Certification Examination Developer). r Pass the ABIM Maintenance of Certification Examination i The College also wishes to acknowledge that many other persons, too numerous to mention, have contributed to the production of this program. Without their dedicated Target Audience efforts, this program would not have been possible. o General internal medicine specialists, including primary care physicians and hospitalists o Internal medicine subspecialists who desire to remain up MKSAP Resource Page to date in internal medicine . Residents preparing for the ABIM Internal Medicine The MKSAP Resource Page (wwwacponline.org/mksap19- Certifi cat ion Examination resources) provides access to MKSAP 19 online answer . Physicians engaged in the ABIM Maintenance of sheets for transcribing answers from the print eclition; Certification Longitudinal Assessment Option, pre- access to MKSAP 19 Digital; Board Basics '; information paring for the ABIM Internal Medicine Maintenance ol on Continuing Medical Education (CME), Maintenance Certitlcation Examination, or engaged with the ABIM of Certification (MOC), and international Continuing Focused Practice in Hospital Medicine program Professional Development (CPD) and MOC; errata; and other new information.

Developer/Architect), and John McKnight (Senior Web r Pass the ABIM Certification Examination Developer). r Pass the ABIM Maintenance of Certification Examination i The College also wishes to acknowledge that many other persons, too numerous to mention, have contributed to the production of this program. Without their dedicated Target Audience efforts, this program would not have been possible. o General internal medicine specialists, including primary care physicians and hospitalists o Internal medicine subspecialists who desire to remain up MKSAP Resource Page to date in internal medicine . Residents preparing for the ABIM Internal Medicine The MKSAP Resource Page (wwwacponline.org/mksap19- Certifi cat ion Examination resources) provides access to MKSAP 19 online answer . Physicians engaged in the ABIM Maintenance of sheets for transcribing answers from the print eclition; Certification Longitudinal Assessment Option, pre- access to MKSAP 19 Digital; Board Basics '; information paring for the ABIM Internal Medicine Maintenance ol on Continuing Medical Education (CME), Maintenance Certitlcation Examination, or engaged with the ABIM of Certification (MOC), and international Continuing Focused Practice in Hospital Medicine program Professional Development (CPD) and MOC; errata; and other new information. Earn CME Credits or MOC Points Online lnternational MOC/CPD To earn CME credits or to apply for MOC points, MKSAP Information and instructions on submission ol inter users need to answer at least one of two questions cor- national MOC/CPD is available by accessing the CME/ rectly (earning a score of at least 50%) and click the Submit MOC/CPD tab on the left navigation menu of MKSAP 19 CME button. Each single MKSAP 19 self-assessment ques- Digital. tion qualifies for one quarter of a CME credit hour or ABIM MOC point.

Earn CME Credits or MOC Points Online lnternational MOC/CPD To earn CME credits or to apply for MOC points, MKSAP Information and instructions on submission ol inter users need to answer at least one of two questions cor- national MOC/CPD is available by accessing the CME/ rectly (earning a score of at least 50%) and click the Submit MOC/CPD tab on the left navigation menu of MKSAP 19 CME button. Each single MKSAP 19 self-assessment ques- Digital. tion qualifies for one quarter of a CME credit hour or ABIM MOC point. Continuing Medical Education MKSAP 19 Subscribers can enter their self assessment question answers and submit for CME/MOC in two The American College of Physicians is accredited by the ways: Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for 1. Users of MKSAP 19 Complete who pref'er to use their physicians. print books and a paper answer sheet to study and record their answers can use the printed answer sheet The American College of Physicians designates this enduring at the back of this book to record their answers. The material, MKSAP 19, for a maximum of 300 AMA PRA corresponding online answer sheets, which are avail- Category 1 CreditsrM. Physicians should claim only the able on the MKSAP 19 Resource Page, may be used to credit commensurate with the extent of their participation transcribe answers onto the online answer sheets. Users in the activity. may then submit their answers to qualify for CME cred Up to 24 AMA PRA Category 1 Creditdtu are available from its or MOC points (see below for information on Opting January 31,2022, to January 31, 2025, for the MKSAP l9 in for MOC). Users who preler to record their answers Rheumatology section. on a paper answer sheet should save their answer sheet for future use. Users who study with MKSAP 19 print can also submit their answers directly within MKSAP 19 Learning Objectives Digital by accessing the self assessment questions dash The learning objectives of MKSAP 19 are to: board and selecting the preferred subspecialty section to begin answering questions. . Close gaps between actual care in your practice and preferred standards of care, based on best evidence 2. Users of MKSAP l9 Digital can enter their answers within . Diagnose disease states that are less common and sometimes the digital program by accessing the self-assessment overlooked or confusing questions dashboard and selecting the preferred sub . Improve clinical management decisions that affect patient specialty section to begin answering questions and safety and quality of care clicking the Submit CME button once they qualify for o Determine when to ref'er patients for care by medical CME and are ready to submit. Learners should keep in subspecialists, surgeons, and other members olthe health mind their yearly CME and MOC deadlines when deter care team mining the appropriate time to submit.

Continuing Medical Education MKSAP 19 Subscribers can enter their self assessment question answers and submit for CME/MOC in two The American College of Physicians is accredited by the ways: Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for 1. Users of MKSAP 19 Complete who pref'er to use their physicians. print books and a paper answer sheet to study and record their answers can use the printed answer sheet The American College of Physicians designates this enduring at the back of this book to record their answers. The material, MKSAP 19, for a maximum of 300 AMA PRA corresponding online answer sheets, which are avail- Category 1 CreditsrM. Physicians should claim only the able on the MKSAP 19 Resource Page, may be used to credit commensurate with the extent of their participation transcribe answers onto the online answer sheets. Users in the activity. may then submit their answers to qualify for CME cred Up to 24 AMA PRA Category 1 Creditdtu are available from its or MOC points (see below for information on Opting January 31,2022, to January 31, 2025, for the MKSAP l9 in for MOC). Users who preler to record their answers Rheumatology section. on a paper answer sheet should save their answer sheet for future use. Users who study with MKSAP 19 print can also submit their answers directly within MKSAP 19 Learning Objectives Digital by accessing the self assessment questions dash The learning objectives of MKSAP 19 are to: board and selecting the preferred subspecialty section to begin answering questions. . Close gaps between actual care in your practice and preferred standards of care, based on best evidence 2. Users of MKSAP l9 Digital can enter their answers within . Diagnose disease states that are less common and sometimes the digital program by accessing the self-assessment overlooked or confusing questions dashboard and selecting the preferred sub . Improve clinical management decisions that affect patient specialty section to begin answering questions and safety and quality of care clicking the Submit CME button once they qualify for o Determine when to ref'er patients for care by medical CME and are ready to submit. Learners should keep in subspecialists, surgeons, and other members olthe health mind their yearly CME and MOC deadlines when deter care team mining the appropriate time to submit. v

t Learners' CME/MOC submission progress will be shown updated clinical care guidelines in developing content, on the MKSAP 19 Digital CME/MOC/CPD page. when such evidence and guidelines were available' A1l content underwent review by external peer reviewers not on the committee to ensure that the material was balanced Opting in for MOC and unbiased. Contributors' disclosure information can be found with the Iist of contributors' names and those of ACP MKSAP 19 users can opt in for simultaneous submission of principal staff listed in the beginning of this book' CME and MOC points as they answer self-assessment ques- tions. To opt in, users will be required to complete a form requesting their name, date of birth, and ABIM number' La nguage Reflecti ng Diversity,_Equity, The MOC Opt in Form will be presented during a user's and lnclusion Within MKSAP 19 first CME submission and needs to be completed only once. MKSAP 19's Editors and contributors commit to using Ianguage and images that support ACP's commitment to ABIM Maintenance of Certification being an anti racist organization that supports diversity' Successful completion of the CME activity, which includes equiry and inclusion throughout health care and health participation in the evaluation component, enables the par education. ACP also continues to ensure diversity among ticipant to earn up to 300 medical knowledge MOC points MKSAP's physician-contributors. When appropriate, in the ABIM's MOC program. It is the CME activity provider's the MKSAP Editors will also rely on MKSAP 19 Digital's responsibility to submit participant completion information expanded use of multimedia enhancements, including video to ACCME for the purpose of granting MOC credit. and audio, to explore and more fully explain issues sur- rounding the presentation of MKSAP 19 clinical content as it relates to race and ethnicity. MKSAP 19 users are encouraged Disclosure Policy to contact the Editors at mksap editors6acponline.org to It is the policy of the American College of Physicians help us identiSr opportunities for improvement in this area. (ACP) to ensure balance, independence, objectivity, and \ scientific rigor in all ofits educational activities. To this end, and consistent with the policies of the ACP and the Hospital-Based Medici ne Accreditation Council for Continuing Medical Education For the convenience ofsubscribers who provide care in (ACCME), contributors to all ACP continuing medical hospital settings, comprehensive hospital-focused content education activities are required to disclose all relevant aligned with the ABIM Focused Practice in Hospital financial relationships with any entity producing, mar Medicine blueprint is integrated throughout the syllabus, keting, re-selling, or distributing health care goods or and self-assessment questions that are specific to the services consumed by, or used on, patients. Contributors hospital setting are specially designated with the blue are required to use generic names in the discussion of hospitalist icon (E). therapeutic options and are required to identify any unap proved, off label, or investigative use of commercial prod- ucts or devices. Where a trade name is used, all available High Value Care Key Points trade names for the same product type are also included. Key Points in the text that relate to High Value Care con- If trade name products manufactured by companies with cepts (that is, concepts that discuss balancing clinical whom contributors have relationships are discussed, benefit with costs and harms) are designated by the HVC contributors are asked to provide evidence based citations icon [HVC]. in support of the discussion. The information is reviewed by the committee responsible flor producing this content. If necessary adiustments to topics or contributors' roles in Educational Disclaimer content development are made to balance the discussion. The editors and publisher of MKSAP l9 recognize that the AII relevant relationships are mitigated. Readers of this development of new material offers many opportunities content are asked to evaluate it for evidence of commercial for error. Despite our best efforts, some errors may persist bias and send any relevant comments to mksap editors6r in print. Drug dosage schedules are, we believe, accurate acponline.org so that future decisions about content and and in accordance with current standards. Readers are contributors can be made in light of this information. advised, however, to ensure that the recommended dos ages in MKSAP 19 concur with the information provided in the product information material. This is especially Mitigation of Conflicts important in cases of new, infrequently used, or highly To mitigate all conflicts of interest and influences of vested toxic drugs. Application of the information in MKSAP 19 interests, ACP's content planners used best evidence and remains the professional responsibility of the practitioner

Learners' CME/MOC submission progress will be shown updated clinical care guidelines in developing content, on the MKSAP 19 Digital CME/MOC/CPD page. when such evidence and guidelines were available' A1l content underwent review by external peer reviewers not on the committee to ensure that the material was balanced Opting in for MOC and unbiased. Contributors' disclosure information can be found with the Iist of contributors' names and those of ACP MKSAP 19 users can opt in for simultaneous submission of principal staff listed in the beginning of this book' CME and MOC points as they answer self-assessment ques- tions. To opt in, users will be required to complete a form requesting their name, date of birth, and ABIM number' La nguage Reflecti ng Diversity,_Equity, The MOC Opt in Form will be presented during a user's and lnclusion Within MKSAP 19 first CME submission and needs to be completed only once. MKSAP 19's Editors and contributors commit to using Ianguage and images that support ACP's commitment to ABIM Maintenance of Certification being an anti racist organization that supports diversity' Successful completion of the CME activity, which includes equiry and inclusion throughout health care and health participation in the evaluation component, enables the par education. ACP also continues to ensure diversity among ticipant to earn up to 300 medical knowledge MOC points MKSAP's physician-contributors. When appropriate, in the ABIM's MOC program. It is the CME activity provider's the MKSAP Editors will also rely on MKSAP 19 Digital's responsibility to submit participant completion information expanded use of multimedia enhancements, including video to ACCME for the purpose of granting MOC credit. and audio, to explore and more fully explain issues sur- rounding the presentation of MKSAP 19 clinical content as it relates to race and ethnicity. MKSAP 19 users are encouraged Disclosure Policy to contact the Editors at mksap editors6acponline.org to It is the policy of the American College of Physicians help us identiSr opportunities for improvement in this area. (ACP) to ensure balance, independence, objectivity, and \ scientific rigor in all ofits educational activities. To this end, and consistent with the policies of the ACP and the Hospital-Based Medici ne Accreditation Council for Continuing Medical Education For the convenience ofsubscribers who provide care in (ACCME), contributors to all ACP continuing medical hospital settings, comprehensive hospital-focused content education activities are required to disclose all relevant aligned with the ABIM Focused Practice in Hospital financial relationships with any entity producing, mar Medicine blueprint is integrated throughout the syllabus, keting, re-selling, or distributing health care goods or and self-assessment questions that are specific to the services consumed by, or used on, patients. Contributors hospital setting are specially designated with the blue are required to use generic names in the discussion of hospitalist icon (E). therapeutic options and are required to identify any unap proved, off label, or investigative use of commercial prod- ucts or devices. Where a trade name is used, all available High Value Care Key Points trade names for the same product type are also included. Key Points in the text that relate to High Value Care con- If trade name products manufactured by companies with cepts (that is, concepts that discuss balancing clinical whom contributors have relationships are discussed, benefit with costs and harms) are designated by the HVC contributors are asked to provide evidence based citations icon [HVC]. in support of the discussion. The information is reviewed by the committee responsible flor producing this content. If necessary adiustments to topics or contributors' roles in Educational Disclaimer content development are made to balance the discussion. The editors and publisher of MKSAP l9 recognize that the AII relevant relationships are mitigated. Readers of this development of new material offers many opportunities content are asked to evaluate it for evidence of commercial for error. Despite our best efforts, some errors may persist bias and send any relevant comments to mksap editors6r in print. Drug dosage schedules are, we believe, accurate acponline.org so that future decisions about content and and in accordance with current standards. Readers are contributors can be made in light of this information. advised, however, to ensure that the recommended dos ages in MKSAP 19 concur with the information provided in the product information material. This is especially Mitigation of Conflicts important in cases of new, infrequently used, or highly To mitigate all conflicts of interest and influences of vested toxic drugs. Application of the information in MKSAP 19 interests, ACP's content planners used best evidence and remains the professional responsibility of the practitioner vl

The primary purpose of MKSAP 19 is educational. of MKSAP sold by unauthorized sellers (e.g., Amazon, Information presented, as well as publications, technol- eBay), with whom ACP has no relationship. We do not ogies, products, and/or services discussed, is intended to honor third party sales. CME credits and MOC points can inform subscribers about the knowledge, techniques, and not be awarded to those purchasers who have purchased experiences of the contributors. A diversity of professional the program from non authorized sellers. opinion exists, and the views of the contributors are their own and not those of the ACP. Inclusion of any material in the program does not constitute endorsement or rec Unauthorized Use of This Book ls ommendation by the ACP. The ACP does not warrant the Against the Law safety, reliability, accuracy, completeness, or usefulness of Unauthorized reproduction of this publication is unlaw and disclaims any and all liability for damages and claims ful. ACP prohibits reproduction of this publication or that may result from the use of information, publications, any of its parts in any form either for individual use or for technologies, products, and/or services discussed in this distribution. program. ACP will consider granting an individual permission to reproduce only limited portions of this publication for Publisher's Information his or her own exclusive use. Send requests in writing to Copyright ,: 2022 American College of Physicians. A11 MKSAP" Permissions, American College of Physicians, 190 N rights reserved. Independence Mall West, Philadelphia, PA 19106-1572, or email your request to mksap editorspacponline.org. This publication is protected by copyright. No part of this publication may be reproduced, stored in a retrieval MKSAP 19ISBN: 978 1-938245-7s 6 system, or transmitted in any form or by any means, elec- Rheumatology ISBN: 978 I-938245 87 9 tronic or mechanical, including photocopy, without the Printed in the United States of America. express consent of the ACP. MKSAP 19 is for individual use only. Only one account per subscription will be per- For order information in the U.S. or Canada, call mitted for the purpose of earning CME credits and MOC 800-ACP-1915. In all other countries, call 215-351-2600 points and for other authorized uses of MKSAP 19. (Monday to Friday, 9.4u-5 plr ET). Fax inquiries to 215-351 2799 or email to custserv@acponline.org.

The primary purpose of MKSAP 19 is educational. of MKSAP sold by unauthorized sellers (e.g., Amazon, Information presented, as well as publications, technol- eBay), with whom ACP has no relationship. We do not ogies, products, and/or services discussed, is intended to honor third party sales. CME credits and MOC points can inform subscribers about the knowledge, techniques, and not be awarded to those purchasers who have purchased experiences of the contributors. A diversity of professional the program from non authorized sellers. opinion exists, and the views of the contributors are their own and not those of the ACP. Inclusion of any material in the program does not constitute endorsement or rec Unauthorized Use of This Book ls ommendation by the ACP. The ACP does not warrant the Against the Law safety, reliability, accuracy, completeness, or usefulness of Unauthorized reproduction of this publication is unlaw and disclaims any and all liability for damages and claims ful. ACP prohibits reproduction of this publication or that may result from the use of information, publications, any of its parts in any form either for individual use or for technologies, products, and/or services discussed in this distribution. program. ACP will consider granting an individual permission to reproduce only limited portions of this publication for Publisher's Information his or her own exclusive use. Send requests in writing to Copyright ,: 2022 American College of Physicians. A11 MKSAP" Permissions, American College of Physicians, 190 N rights reserved. Independence Mall West, Philadelphia, PA 19106-1572, or email your request to mksap editorspacponline.org. This publication is protected by copyright. No part of this publication may be reproduced, stored in a retrieval MKSAP 19ISBN: 978 1-938245-7s 6 system, or transmitted in any form or by any means, elec- Rheumatology ISBN: 978 I-938245 87 9 tronic or mechanical, including photocopy, without the Printed in the United States of America. express consent of the ACP. MKSAP 19 is for individual use only. Only one account per subscription will be per- For order information in the U.S. or Canada, call mitted for the purpose of earning CME credits and MOC 800-ACP-1915. In all other countries, call 215-351-2600 points and for other authorized uses of MKSAP 19. (Monday to Friday, 9.4u-5 plr ET). Fax inquiries to 215-351 2799 or email to custserv@acponline.org. Disclaimer Regarding Direct Purchases from Online Retailers Errata and Revisions CME and/or MOC for MKSAP 19 is available only to cus- Errata and Revisions for MKSAP 19 will be available tomers who purchase the program directly from ACP. ACP through MKSAP 19 Digital at mksaplg.acponline.org as will not set up MKSAP CME/MOC accounts for purchasers new information becomes known to the editors.

Disclaimer Regarding Direct Purchases from Online Retailers Errata and Revisions CME and/or MOC for MKSAP 19 is available only to cus- Errata and Revisions for MKSAP 19 will be available tomers who purchase the program directly from ACP. ACP through MKSAP 19 Digital at mksaplg.acponline.org as will not set up MKSAP CME/MOC accounts for purchasers new information becomes known to the editors. vll

Table of Contents Approach to the PatientWith Rheumatologic Disease Uricosuric Agents 15 InflammatoryVersus NoninflammatoryPain . .. . . . . . . 1 Pegloticase 15 TheMusculoskeletalExamination ........... 1 Medications and Pregnancy 15 Arthritis .....1 Vaccination and Screening in Immunosuppression 15 Monoarthritis...... .....1 Nonpharmacologic and Nontraditional Oligoarthritis ...........2 Management 15 Polyarthritis Physical and Occupational Therapy .....2 15 Soft-TissueAbnormalities..... ......2 Complementary and Alternative Medicine . . . L7 Extra-Articular Manifestations of Rheumatologic RoleofSurgery .... 77 Disease ......2 Constitutionalsymptoms. .......2 Rheumatoid Arthiltis Skinlnvolvement... ...........2 Pathophysiologr and Risk Factors. t7 Eyelnvolvement... .....2 Genetic Factors 17 InternalOrganlnvolvement.. ........... 2 Environmental Factors 17 LaboratoryStudies ..........2 Infectious Agents. t7 TestsThatMeasurelnflammation ........ 2 Hormones 17 AutoantibodyTests .. ...........5 Diagnosis 1B

LaboratoryStudies ..........2 Infectious Agents. t7 TestsThatMeasurelnflammation ........ 2 Hormones 17 AutoantibodyTests .. ...........5 Diagnosis 1B ImagingStudies. .....5 ClinicalManifestations. . . . . . 18 Radiography. ...........5 Laboratory Studies 19 \t cT.... .........7 Imagrng Studies. t9 t t MRI... .........7 Complications and Extra-Articular Ultrasonography . . . . . . . . . . . . . . .7 Manifestations..... 20 I JointAspiration.... .........7 Joints. . 20 TissueBiopsy ........8 Skin... 20 MentalHealthScreening .....8 Eyes... 20 Lungs . 21.

TissueBiopsy ........8 Skin... 20 MentalHealthScreening .....8 Eyes... 20 Lungs . 21. Heart.. 2l Principles of Therapeutics Hematologic. 2t Overview 8 Blood Vessels 2t Anti-lnflammatory Agents. ,8 Management 2t Glucocorticoids . . . . ,8 GeneralConsiderations. . . . . . 2l NSAIDS ,9 Disease-Modi$zing Antirheumatic Drugs 22 Colchicine ,9 NSAIDS 22 Analgesics and Pain Pathway Modulators ,9 I Acetaminophen. . . . 10 Glucocorticoids.... 22 I

Heart.. 2l Principles of Therapeutics Hematologic. 2t Overview 8 Blood Vessels 2t Anti-lnflammatory Agents. ,8 Management 2t Glucocorticoids . . . . ,8 GeneralConsiderations. . . . . . 2l NSAIDS ,9 Disease-Modi$zing Antirheumatic Drugs 22 Colchicine ,9 NSAIDS 22 Analgesics and Pain Pathway Modulators ,9 I Acetaminophen. . . . 10 Glucocorticoids.... 22 I Surgery 23 I Tramadol 10 Pregnancy 23 Serotonin Norepinephrine Reuptake Inhibitors 10 : Gabapentinoids.... 10 r Disease-Modi$ring Antirheumatic Drugs. 10 Osteoarthritis : Nonbiologic Disease-Modi$ring Pathophysiolory .... 23 ! i Antirheumatic Drugs 10 Epidemiolory and Risk Factors 23 i I Biologic Disease-Modifying Antirheumatic Drugs t2 Classification 24

Surgery 23 I Tramadol 10 Pregnancy 23 Serotonin Norepinephrine Reuptake Inhibitors 10 : Gabapentinoids.... 10 r Disease-Modi$ring Antirheumatic Drugs. 10 Osteoarthritis : Nonbiologic Disease-Modi$ring Pathophysiolory .... 23 ! i Antirheumatic Drugs 10 Epidemiolory and Risk Factors 23 i I Biologic Disease-Modifying Antirheumatic Drugs t2 Classification 24 Urate-Lowering Therapy 13 PrimaryOsteoarthritis. . . . . 24 i I Allopurinol. 13 Secondary Osteoarthritis 25 t Febuxostat t4 Diffuse Idiopathic Skeletal Hyperostosis 25 tx

Diagnosis 25 Diagnosis 46 ClinicalManifestations. . . . . . .. 25 General Considerations. . . . . 46 Laboratory and Imaging Studies 27 Laboratory Studies 46 Differential Diagnosis 27 Differential Diagnosis. . . . . . 48 Management 28 Management 48 Nonpharmacologic Therapy. . . . 29 Pregnancy and Childbirth Issues. 50 Pharmacologic Therapy 29 Prognosis 51 Surgical Therapy 30 Sjtigren Syndrome Fibromyalgia Epidemiologz and Pathophysiologr 51 Epidemiologr and Pathophysiologz 30 Clinical Manifestations. . . . . . . . . . . 5t Diagnosis 30 Diagnosis 51 Management 32 Management 52 Nonpharmacologic . 32 Prognosis 52 Pharmacologic..... 32

Diagnosis 25 Diagnosis 46 ClinicalManifestations. . . . . . .. 25 General Considerations. . . . . 46 Laboratory and Imaging Studies 27 Laboratory Studies 46 Differential Diagnosis 27 Differential Diagnosis. . . . . . 48 Management 28 Management 48 Nonpharmacologic Therapy. . . . 29 Pregnancy and Childbirth Issues. 50 Pharmacologic Therapy 29 Prognosis 51 Surgical Therapy 30 Sjtigren Syndrome Fibromyalgia Epidemiologz and Pathophysiologr 51 Epidemiologr and Pathophysiologz 30 Clinical Manifestations. . . . . . . . . . . 5t Diagnosis 30 Diagnosis 51 Management 32 Management 52 Nonpharmacologic . 32 Prognosis 52 Pharmacologic..... 32 ldiopathic lnflammatory Myopathies Spondyloarthritis Overview 53 Overview 32 Evaluation of the Patient With Muscle Pain Pathophysiolory .......... en or Weakness. 53 Genetic Factors 33 Epidemiologr and Pathophysiologz 53 Environmental Factors . 33 Clinical Features 54 Tissue Factors....... . . JJ Muscle Involvement 54 Classification JJ Pulmonary Disease 54 Anl<ylosing Spondylitis . J.) Skinlnvolvement... 55 PsoriaticArthritis . .. . . 36 Cardiac Disease 55 Enteropathic Arthritis. . Malignancy. 55 Reactive Arthritis. . . . . . 3B Types of Idiopathic Inflammatory Myositis . . . 55 Diagnosis 3B Dermatomyositis. . . 55 LaboratoryStudies .... 3B Polymyositis. 55 Imaging Studies. . . . . . . 39 Antisynthetase Syndrome 57 Management 4I Immune-Mediated Necrotizing Myopathy 57 General Considerations. 4t Inclusion Body Myositis 57 Ankylosing Spondylitis. 47 Overlap Syndromes. 57 PsoriaticArthritis ... . . 42 Diagnosis 57 Enteropathic Arrhritis. . 42 Classifi cation Criteria 57 Reactive Arthritis. . .. . . 42 Muscle-Related Enzymes 57 Autoantibodies .... 57 Systemic Lupus Erythematosus Imaging 58 Epidemiologr and Pathophysiologz 42 Electromyography. . . 58 Clinical Manifestations... . . . . . . .. 43 Histopathologz s8 Mucocutaneous Involvement. . 43 Management 59 Musculoskeletal Involvement. . 44 Prognosis 59 Kidney Involvement 44 Neuropsychiatric Involvement . 45 Systemic Sclerosis Cardiovascular Involvement. . . 45 Epidemiologr and Pathophysiologz . 59 Pulmonary Involvement. . . . . . 45 Classification 59 Hematologic Involvement. . . . . 45 Clinical Manifestations and Diagnosis 60 Gastrointestinal Involvement . . 46 Cutaneous Involvement 60 Comorbidities .. _..... 46 Musculoskeletal Involvement. . ., 62

ldiopathic lnflammatory Myopathies Spondyloarthritis Overview 53 Overview 32 Evaluation of the Patient With Muscle Pain Pathophysiolory .......... en or Weakness. 53 Genetic Factors 33 Epidemiologr and Pathophysiologz 53 Environmental Factors . 33 Clinical Features 54 Tissue Factors....... . . JJ Muscle Involvement 54 Classification JJ Pulmonary Disease 54 Anl<ylosing Spondylitis . J.) Skinlnvolvement... 55 PsoriaticArthritis . .. . . 36 Cardiac Disease 55 Enteropathic Arthritis. . Malignancy. 55 Reactive Arthritis. . . . . . 3B Types of Idiopathic Inflammatory Myositis . . . 55 Diagnosis 3B Dermatomyositis. . . 55 LaboratoryStudies .... 3B Polymyositis. 55 Imaging Studies. . . . . . . 39 Antisynthetase Syndrome 57 Management 4I Immune-Mediated Necrotizing Myopathy 57 General Considerations. 4t Inclusion Body Myositis 57 Ankylosing Spondylitis. 47 Overlap Syndromes. 57 PsoriaticArthritis ... . . 42 Diagnosis 57 Enteropathic Arrhritis. . 42 Classifi cation Criteria 57 Reactive Arthritis. . .. . . 42 Muscle-Related Enzymes 57 Autoantibodies .... 57 Systemic Lupus Erythematosus Imaging 58 Epidemiologr and Pathophysiologz 42 Electromyography. . . 58 Clinical Manifestations... . . . . . . .. 43 Histopathologz s8 Mucocutaneous Involvement. . 43 Management 59 Musculoskeletal Involvement. . 44 Prognosis 59 Kidney Involvement 44 Neuropsychiatric Involvement . 45 Systemic Sclerosis Cardiovascular Involvement. . . 45 Epidemiologr and Pathophysiologz . 59 Pulmonary Involvement. . . . . . 45 Classification 59 Hematologic Involvement. . . . . 45 Clinical Manifestations and Diagnosis 60 Gastrointestinal Involvement . . 46 Cutaneous Involvement 60 Comorbidities .. _..... 46 Musculoskeletal Involvement. . ., 62 x

Vascularlnvolvement . . . . . . 62 LymeArthritis..... 75 Cardiac Involvement 63 My cobacterium tuberculosis Infection 7S Gastrointestinal Involvement 63 Fungal Infections. 75 Kidney lnvolvement 63 Virallnfections ..... 76 Lunglnvolvement. . . . . . . . . 64 Prosthetic Joint Infections . . . . 77 Management 64 Management 77 Pregnancy 65 Systemic Uasculitis Mixed Connectiye lissue Disease and Undifferentiated Overview ..........78 Connective fissue Disease Large-VesselVasculitis ......79 Overview ..........66 GiantCellArteritis .....79 EpidemiolograndPathophysiologz . . .......66 PolymyalgiaRheumatica .......80 ClinicalManifestations andDiagnosis . . . . . . . .......66 TakayasuArteritis ...... 8l Management .......66 Medium-VesselVasculitis .... 81 Prognosis ..........66 PolyarteritisNodosa ....81 Primary Angiitis of the Central Nervous System . . . 82 CrystalArthropathies KawasakiDisease ......83 Gout .. 67 Small-VesselVasculitis ......83 Epidemiologr 67 ANCA-AssociatedVasculitis...... ...... 83 Pathophysiolory .... 67 Immune Complex-MediatedVasculitis. . . . . . . . . 85 Clinical Manifestations. . . . . . 67 Diagnosis 68 Other Rheumatologir Diseases Management 69 Behqet Syndrome 87 Calcium Pyrophosphate Deposition. . . . ., 7l RelapsingPolychondritis . . ... ..... . 88

Vascularlnvolvement . . . . . . 62 LymeArthritis..... 75 Cardiac Involvement 63 My cobacterium tuberculosis Infection 7S Gastrointestinal Involvement 63 Fungal Infections. 75 Kidney lnvolvement 63 Virallnfections ..... 76 Lunglnvolvement. . . . . . . . . 64 Prosthetic Joint Infections . . . . 77 Management 64 Management 77 Pregnancy 65 Systemic Uasculitis Mixed Connectiye lissue Disease and Undifferentiated Overview ..........78 Connective fissue Disease Large-VesselVasculitis ......79 Overview ..........66 GiantCellArteritis .....79 EpidemiolograndPathophysiologz . . .......66 PolymyalgiaRheumatica .......80 ClinicalManifestations andDiagnosis . . . . . . . .......66 TakayasuArteritis ...... 8l Management .......66 Medium-VesselVasculitis .... 81 Prognosis ..........66 PolyarteritisNodosa ....81 Primary Angiitis of the Central Nervous System . . . 82 CrystalArthropathies KawasakiDisease ......83 Gout .. 67 Small-VesselVasculitis ......83 Epidemiologr 67 ANCA-AssociatedVasculitis...... ...... 83 Pathophysiolory .... 67 Immune Complex-MediatedVasculitis. . . . . . . . . 85 Clinical Manifestations. . . . . . 67 Diagnosis 68 Other Rheumatologir Diseases Management 69 Behqet Syndrome 87 Calcium Pyrophosphate Deposition. . . . ., 7l RelapsingPolychondritis . . ... ..... . 88 i Epidemiologz and Pathophysiolory . , 7l AutoinflammatoryDiseases. . . . . . . . . 89 Clinical Manifestations and Diagnosis 7l Adult-Onset Still Disease 90 t Management 72 Sarcoidosis 90 \ Basic Calcium Phosphate Deposition . . . . 73 Igc4-Related Disease 92 Genetic Diseases of Connective Tissue 92 lnfectious Arthritis Diagnosis 73 Bibliography 93 Clinical Manifestations. . . . . 73 Laboratory and Imaging Studies 73 Self-Assessment Test. 97 Causes. 74 Infection With Gram-Positive Organisms 74 Infection With Gram-Negative Organisms 74 lndex t75

i Epidemiologz and Pathophysiolory . , 7l AutoinflammatoryDiseases. . . . . . . . . 89 Clinical Manifestations and Diagnosis 7l Adult-Onset Still Disease 90 t Management 72 Sarcoidosis 90 \ Basic Calcium Phosphate Deposition . . . . 73 Igc4-Related Disease 92 Genetic Diseases of Connective Tissue 92 lnfectious Arthritis Diagnosis 73 Bibliography 93 Clinical Manifestations. . . . . 73 Laboratory and Imaging Studies 73 Self-Assessment Test. 97 Causes. 74 Infection With Gram-Positive Organisms 74 Infection With Gram-Negative Organisms 74 lndex t75 xl