SARCOIDOSIS DIAGNOSIS AND MANAGEMENT doc

SARCOIDOSIS DIAGNOSIS AND MANAGEMENT Edited by Mohammad Hosein Kalantar Motamedi Sarcoidosis Diagnosis and Management Edited by Mohammad Hosein Kalantar Motamedi Published by InTech Janeza Trdine 9, 51000 Rijeka, Croatia Copyright © 2011 InTech All chapters are Open Access distributed under the Creative Commons Attribution 3.0 license, which permits to copy, distribute, transmit, and adapt the work in any medium, so long as the original work is properly cited. After this work has been published by InTech, authors have the right to republish it, in whole or part, in any publication of which they are the author, and to make other personal use of the work. Any republication, referencing or personal use of the work must explicitly identify the original source. As for readers, this license allows users to download, copy and build upon published chapters even for commercial purposes, as long as the author and publisher are properly credited, which ensures maximum dissemination and a wider impact of our publications. Notice Statements and opinions expressed in the chapters are these of the individual contributors and not necessarily those of the editors or publisher. No responsibility is accepted for the accuracy of information contained in the published chapters. The publisher assumes no responsibility for any damage or injury to persons or property arising out of the use of any materials, instructions, methods or ideas contained in the book. Publishing Process Manager Martina Blecic Technical Editor Teodora Smiljanic Cover Designer Jan Hyrat Image Copyright Apostrophe, 2011. Used under license from Shutterstock.com First published September, 2011 Printed in Croatia A free online edition of this book is available at www.intechopen.com Additional hard copies can be obtained from orders@intechweb.org Sarcoidosis Diagnosis and Management, Edited by Mohammad Hosein Kalantar Motamedi p. cm. ISBN 978-953-307-414-6 free online editions of InTech Books and Journals can be found at www.intechopen.com Contents Preface IX Part 1 Immunology 1 Chapter 1 Immunopathogenesis of Sarcoidosis 3 Giorgos A. Margaritopoulos, Foteini N. Economidou, Nikos M. Siafakas and Katerina M. Antoniou Chapter 2 Immunopathogenesis and Presumable Antigen Pathway of Sarcoidosis: A Comprehensive Approach 21 Atsushi Kurata Part 2 Diagnosis 35 Chapter 3 Basic Diagnostic Approaches in Sarcoidosis 37 Louis Gerolemou and Peter R. Smith Chapter 4 Diagnosis of Pulmonary Sarcoidosis 47 Tiberiu Shulimzon and Matthew Koslow Chapter 5 Imaging in Sarcoidosis 71 M. Reza Rajebi, Nicole A. Zimmerman, Roozbeh Sharif, Ernest M. Scalzetti, Stuart A. Groskin and Rolf A. Grage Chapter 6 Bronchoalveolar Lavage and Sampling in Pulmonary Sarcoidosis 101 Edvardas Danila Part 3 Management 123 Chapter 7 Updated Guidelines for the Treatment of Pulmonary Sarcoidosis 125 Luis Jara-Palomares, Candela Caballero-Eraso, Alicia Díaz-Baquero and Jose Antonio Rodríguez-Portal VI Contents Chapter 8 Prognostic Factors in Sarcoidosis 137 Wojciech J. Piotrowski Chapter 9 Lung Transplantation for Pulmonary Sarcoidosis 163 Dominic T. Keating Part 4 Extrapulmonary Sarcoidosis 189 Chapter 10 Clinical Features of Skin 191 Dilek Biyik Ozkaya Chapter 11 Acquired Ichthyosiform Erythrodermia Sarcoidosis 199 Hui-Jun Ma Chapter 12 Orofacial Sarcoidosis and Granulomatosis 203 Mohammad Hosein Kalantar Motamedi, Mohammad Ghasem Shams and Taghi Azizi Chapter 13 Diagnostic and Therapeutic Management of Cardiac Sarcoidosis -Application of High Resolution Electrocardiography 217 Kenji Yodogawa Chapter 14 Neurological Sarcoidosis 223 J. Chad Hoyle and Herbert B. Newton Chapter 15 Spinal Cord Sarcoidosis Accompanied with Compressive Cervical Myelopathy 239 Yoshihito Sakai, Yukihiro Matsuyama and Shiro Imagama Part 5 Sarcoid-Like Reactions 251 Chapter 16 Sarcoid-Like Reactions 253 Shweta Nag Chapter 17 Granulomatosis and Cancer 269 Michel Pavic and Florian Pasquet Preface Sarcoidosis is a type of inflammation that occurs in various locations of the body for no known reason. Normally, when foreign substances or organisms enter the body, the immune system retaliates by activating an immune response. Inflammation is a normal part of this immune response that subsides once the antigen is gone. In sarcoidosis, the inflammation persists, and immune cells form abnormal tissue called granulomas. Although the disease can affect any organ, it is most likely to occur in the lungs i.e. the skin, eyes, liver, or lymph nodes. The etiology of sarcoidosis is not known; research suggests that it may be due to an extreme immune response or sensitivity to certain substances and seems to have a genetic component as well. When sarcoidosis occurs in the lungs, it can lead to wheezing, coughing, shortness of breath, and chest pain. Other possible symptoms that affect other body systems include night sweats, fever, weight loss, and seizures. Some cases of sarcoidosis resolve spontaneously , while others may last indefinitely. Treatment of sarcoidosis is designed to reduce inflammation and usually includes corticosteroids and immunosuppression therapy. As a contemporary comprehensive book relating to sarcoidosis focusing on the aforementioned issues was lacking, INTECH took the opportunity to seek out top researchers on the subject worldwide in order to collate data and publish a diagnostic and management update on this mysterious disease. To this end more than 30 contemporary scientists worldwide were consulted. Based on their specific area of expertise and recently published research indexed in PUBMED, each contributed generously to a section of this book. This book has 5 basic sections : Immunology, Diagnosis, Management, Extrapulmonary Sarcoidosis and Sarcoid-like Reactions. It includes 17 chapters which cover the topics of: Immunopathogenesis and antigen pathway of sarcoidosis, Diagnostic Approaches in Sarcoidosis, Imaging in Sarcoidosis, Bronchoalveolar lavage and sampling, Treatment update in pulmonary sarcoidosis, Prognostic Factors in Sarcoidosis, Lung Transplantation, Extrapulmonary Sarcoidosis (skin, face, mouth, heart, brain, spine) and Sarcoid-like reactions. For me, it was indeed both an honor and a privilege to work with these noble researchers. Anyone who has authored a book knows how hard a task it is to compile,complete, edit and publish it. This indeed was a great undertaking on behalf of INTECH and the international authors and collaborators. I hereby express my X Preface gratitude and sincere appreciation to each and every one of them for their unyielding and relentless efforts in this arduous task. I would like to also thank INTECH open access publisher, Ms. Ana Nikolic Head of Editorial Consultants and the Publishing Managers Mr. Niksa Mandic and Ms. Martina Blecic for their kind help throughout the past 12 months without which this undertaking would not have been possible. Mohammad Hosein Kalantar Motamedi Professor, Trauma Research Center, Baqiyatallah University of Medical Sciences, Tehran, IR Iran [...]... by different stimuli and produce different types of cytokines and costimulatory molecules with different actions Activation by LPS or IFN- leads to 6 Sarcoidosis Diagnosis and Management inflammatory response and production of proinflammatory cytokines and increased expression of CD16, CD32 and CD64 On the other hand, activation by IL-4, IL-10 and IL13 leads to a fibrotic response and production of CCL17,... nodosum, bilateral hilar adenopathy in chest radiograph, and arthritis), and a gallium-67 uptake in the parotid and lacrimal glands (Panda sign) as well as in the right paratracheal and bilateral hilar (Lambda sign) Diagnostic criteria of sarcoidosis have been thus 22 Sarcoidosis Diagnosis and Management established; however, in practice, its diagnosis is made arbitrarily because complete exclusion... Marshall BG, Wangoo A, Cook HT, Shaw RJ (1996) Increased inflammatory cytokines and new collagen formation in cutaneous tuberculosis and sarcoidosis Thorax, 51:1253 1261 Martinetti M, Luisetti M, Cuccia M (2002) HLA and sarcoidosis: new pathogenetic insights SarcoidosisVasc Diffuse Lung Dis, 19:83-95 16 Sarcoidosis Diagnosis and Management Minami J, Eishi Y, Ishige Y, et al (2003) Pulmonary granulomas caused... in the innate immunity and their role in the pathogenesis of sarcoidosis has been investigated in many 4 Sarcoidosis Diagnosis and Management studies TLRs localize to various cellular compartments depending on the nature of the ligands they recognize Thus, TLRs involved in recognition of lipid and protein ligands are expressed on the plasma membrane (TLR-1, TLR-2, TLR-4, TLR-5 and TLR-6), whereas TLRs... affects people of all racial and ethnic groups, although the incidence of sarcoidosis varies widely throughout the world and is most common in women and in people of Scandinavian or African-American descent Sarcoidosis may occur at any age, but is usually seen in adults under the age of 50 (Dempsey et al., 2009; Iannuzzi et al., 2007; Fernandez-Faith & McDonnell, 2007) Sarcoidosis has long been characterized... interaction between AMs and T-cells are found increased in patients with sarcoidosis These include CD154 (ligand for CD40), CD72 (ligand for CD5), CD80 and CD86 (ligand for CD28), CD153 (ligand for CD30L) (Wahlstrửm et al,1999;Hoshino et al,1995;Nicod&Isler,1997;Kaneko et al,1999;Agostini et al,1999;Zissel,1999) Adhesion molecules such as CD54 and CD11a-c are also expressed highly in epithelioid cells forming... Respir Dis, 133:78-82 Saboor SA, Johnson NM, McFadden J (1992) Detection of mycobacterial DNA in sarcoidosis and tuberculosis with polymerase chain reaction Lancet, 339:1012-1015 18 Sarcoidosis Diagnosis and Management Sallusto F, Schaerli P, Loetscher P, Schaniel C, Lenig D, Mackay CR, et al (1998) Rapid and coordinated switch in chemokine receptor expression during dendritic cell maturation Eur J... TLR-2 and nuclear factor kB activation (Chen, 2010) Once TLRs bind to products of various PAMPs and DAMPs, intracellular signaling pathways are being activated and pro-inflammatory chemokines and cytokines are released (Bianchi,2007) TLR-9 has been observed to be overexpressed in the BAL of patients with sarcoidosis compared to normal controls (Margaritopoulos et al,2010) A higher expression of TLR-2 and. .. MHC class II and costimulatory molecules and mature into functional APCs (Banchereau&Steinman,1998;Sallusto et al,1998) They also express CCR7 in their surface and under the influence of its ligands such as CCL19 and CCL21 they migrate into the T-cell areas of regional lymphnodes replaced by peripheral blood precursors (Jang et al,2006;Legge&Braciale,2003) Therefore, lymphadenopathy seen in sarcoidosis. .. lymphnodes DCs are components of granuloma observed in sarcoidosis and studies have shown a premature and rapid involvement of these cells at the sites of inflammation and in the formation of granuloma (Iyonaga et al,2002;Ota et al,2004;Chiu et al,2004) 2.5 T-cells The presence and accumulation of T-cells is critical for the granuloma formation and this is supported by the fact that T-cell depleted . SARCOIDOSIS DIAGNOSIS AND MANAGEMENT Edited by Mohammad Hosein Kalantar Motamedi Sarcoidosis Diagnosis and Management Edited by Mohammad. AMs and involved in the interaction between AMs and T-cells are found increased in patients with sarcoidosis. These include CD154 (ligand for CD40), CD72 (ligand for CD5), CD80 and CD86 (ligand. that play a key role in the innate immunity and their role in the pathogenesis of sarcoidosis has been investigated in many Sarcoidosis Diagnosis and Management 4 studies. TLRs localize to