Respiratory Medicine Series Editor: Sharon I.S. Rounds. Marc A. Judson Editor. Pulmonary Sarcoidosis A Guide for the Practicing Clinician

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2 Respiratory Medicine Series Editor: Sharon I.S. Rounds Marc A. Judson Editor Pulmonary Sarcoidosis A Guide for the Practicing Clinician

3 Respiratory Medicine Series Editor: Sharon I.S. Rounds For further volumes:

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5 Marc A. Judson Editor Pulmonary Sarcoidosis A Guide for the Practicing Clinician

6 Editor Marc A. Judson, M.D. Division of Pulmonary and Critical Care Medicine Department of Medicine Albany Medical College Albany, NY, USA ISBN ISBN (ebook) DOI / Springer New York Heidelberg Dordrecht London Library of Congress Control Number: Springer Science+Business Media New York 2014 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms 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 specifically 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 specific 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. Printed on acid-free paper Humana Press is a brand of Springer Springer is part of Springer Science+Business Media (

7 This book is dedicated to Sooyeon Kwon, my beautiful, loving, and intelligent wife. Her caring and support has made many things possible in my life, including this work.

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9 Preface Sarcoidosis is a disease with world-wide prevalence that affects all races, ethnic groups, individuals of all ages, and both sexes. The disease may affect any organ. Its severity may range from a radiographic or laboratory abnormality detected in a patient with no symptoms to a relentlessly progressive disease that is potentially life threatening. Despite the awareness of sarcoidosis as a specific disease entity for nearly one and one-half centuries, there is still no standard method of diagnosis, treatment approach, method of follow-up evaluation, or method to reliably predict prognosis. Because sarcoidosis is a relatively rare disease, few clinicians have extensive clinical experience in its management. Furthermore, medical evidence concerning the clinical approach to sarcoidosis is scarce. Therefore, clinicians typically lack the necessary tools to make reliable treatment decisions concerning sarcoidosis patients. The lung is the most common organ involved with sarcoidosis. Pulmonary manifestations of sarcoidosis include asymptomatic radiographic abnormalities, granulomatous inflammation of airways, and/or the lung interstitium causing pulmonary symptoms and fibrocystic disease that may result in severe pulmonary dysfunction and pulmonary hypertension. This book will serve as a valuable resource for clinicians concerning the care of the pulmonary sarcoidosis patient. The focus of this book concerns issues of diagnosis, management, and prognosis of the disease. The authors are all clinicians with extensive experience in caring for these patients. Although they drew on their own expertise, the authors were encouraged to focus on the clinical data that are available. I hope that this book will serve as a practical guide for clinicians and thus improve the care of sarcoidosis patients. Albany, NY, USA Marc A. Judson, M.D. vii

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11 Acknowledgments I would like to acknowledge several individuals who were essential in the production of this book. I would like to thank the major effort of all the authors in writing these scholarly and clinically relevant chapters. I thank Springer, and in particular, Ms. Amanda Quinn and Mr. Michael Wilt, whose efforts were essential in the publication of this work. I thank all of my mentors, colleagues, and mentees who constantly educated me and questioned me over the years and helped me develop my passion for learning. In particular, I would like to thank Dr. Steven A. Sahn for his guidance concerning my academic career and writing skills. I would like to acknowledge all the sarcoidosis patients who I have treated over the years for their courage and their kindness. Finally, I thank my parents, my wife, and my children for their support and love. ix

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13 Contents 1 The Diagnosis of Sarcoidosis... 1 Marc A. Judson 2 The Pulmonary Manifestations of Sarcoidosis Marcel Veltkamp and Jan C. Grutters 3 The Treatment of Pulmonary Sarcoidosis Robert P. Baughman and Marjolein Drent 4 Acute Pulmonary Exacerbation of Sarcoidosis Efstratios Panselinas and Marc A. Judson 5 Advanced ( End-Stage ) Pulmonary Sarcoidosis Divya C. Patel, Marie Budev, and Daniel A. Culver 6 Sarcoidosis-Associated Pulmonary Hypertension Oksana A. Shlobin and Steven D. Nathan 7 Monitoring Pulmonary Sarcoidosis Athol U. Wells 8 Extrapulmonary Sarcoidosis Hidenobu Shigemitsu, Hiren V. Patel, and Matthew P. Schreiber 9 Instructive Cases of Pulmonary Sarcoidosis Andrew J. Goodwin and Carlos E. Kummerfeldt Index xi

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15 Contributors Robert P. Baughman, M.D. Department of Internal Medicine, University of Cincinnati Medical Center, Cincinnati, OH, USA Marie Budev, D.O. Respiratory Institute, Cleveland Clinic, Cleveland, OH, USA Daniel A. Culver, D.O. Respiratory Institute, Cleveland Clinic, Cleveland, OH, USA Marjolein Drent, M.D., Ph.D. Department of Interstitial Lung Disease, Hospital Gelderse Vallei, Ede, The Netherlands Faculty of Health, Medicine, and Life Sciences, (FHML), Maastricht University, Maastricht, The Netherlands Andrew J. Goodwin, M.D., M.S.C.R. Division of Pulmonary, Critical Care, Allergy, and Sleep Medicine, Medical University of South Carolina, Charleston, SC, USA Jan C. Grutters, M.D., Ph.D. Department of Pulmonology, St. Antonius Hospital, Nieuwegein, The Netherlands Center of Interstitial Lung Diseases, St. Antonius Hospital, Nieuwegein, The Netherlands Division Heart & Lungs, University Medical Center, Utrecht, The Netherlands Marc A. Judson, M.D. Division of Pulmonary and Critical Care Medicine, Department of Medicine, Albany Medical College, Albany, NY, USA Carlos E. Kummerfeldt, M.D. Division of Pulmonary, Critical Care, Allergy, and Sleep Medicine, Medical University of South Carolina, Charleston, SC, USA Steven D. Nathan, M.D. Advanced Lung Disease and Transplant Program, Department of Medicine, Inova Fairfax Hospital, Falls Church, VA, USA Efstratios Panselinas, M.D. Department of Pulmonary Medicine, General Army Hospital, Tripoli, Greece Divya C. Patel, D.O. Respiratory Institute, Cleveland Clinic, Cleveland, OH, USA xiii