Sarcoidosis PDF

Preface

Sarcoidosis is an inflammatory lung disease of unknown etiology. It affects any part of the body, but the lungs are affected in over ninety percent of patients with the disease. It has a wide range of manifestations and variable prognosis. Since the majority of patients do well, many view this as a benign process. However, there are a significant percentage of patients who are severely affected by the disease with resulting dyspnea, visual disturbances, disfiguring skin lesions, or other chronic manifestations.

This book assimilates the large amount of information available about sarcoidosis into a single source. The last time this was done was in 1994, when Dr. Geraint James prepared the prior edition of this book. In our book, a worldwide contingent of experts in the field of sarcoidosis have pooled their expertise to provide a comprehensive view of the disease. The information in this book reflects the tremendous changes that have occurred over the past few years with regard to sarcoidosis. Recent studies have examined the potential interaction between environmental exposure and genetic risk factors in sarcoidosis. These studies have expanded earlier observations that some groups seem to experience worse disease than others. New therapies, including the anti-tumor necrosis factor agents, have added insight into the mechanisms of resolution versus persistence of the disease.

The book is designed to explore all aspects of sarcoidosis in detail. This includes discussions on the epidemiology, etiology, clinical manifestations, and treatment of the disease. The purpose of this book is to be the single best source of information about sarcoidosis. This book should be useful for the casual reader wishing to answer a question about a specific issue in sarcoidosis. In addition, we have provided detailed information for clinicians and researchers with more broad ranging questions about the disease.

After Dr. Sharma's historical review of the disease, a review of the epidemiology of the disease is provided by Dr. Hosado and Dr. Selroos. The various immune responses of the sarcoidosis reaction are dissected, with comments on the granuloma formation (Dr. Semenzato), the lymphocytic response (Dr. Agostini), the antigen presenting cells (Dr. Muller- Quernheim), and the cytokine interaction (Dr. Moller). The current best model for this immune response is the Kveim reaction, and this is discussed by Dr. Kataria.

Genetic risk factors are discussed by three groups. Dr. Iannuzzi provides a general overview of the genetic risk factors. Dr. Rossman discusses the human leukocyte antigen and its role in manifestations of sarcoidosis. Finally, Dr. Eklund discusses the current status of knowledge regarding genetic pattern and subsequent disease.

The role of infection has been long speculated in sarcoidosis. Dr. Hunninghake provides a review of the evidence for and against many of the putative agents. Dr. Eishi provides information regarding one of the most likely candidates as a possible cause of sarcoidosis, while providing insight into what is needed to confirm the role of any agent as a cause of this disease.

We also have a section discussing other granulomatous diseases, which can be mistaken for sarcoidosis. These include hypersensitivity pneumonitis (Dr. Ando) and various occupational lung diseases (Dr. Newman).

A summary of the evaluation starts with an overview of evaluation (Drs. Baughman and du Bois), followed by specific testing. This includes radiologic evaluation (Dr. Wells), bronchoalveolar lavage (Dr. Costabel), pulmonary function testing (Dr. Martinez), serum markers (Drs. Baughman and Costabel), quality of life instruments (Dr. Drent), and pathology (Dr. Popper).

We then discuss specific organ involvement. This includes neurologic disease (Dr. Nagai), cardiac disease (Dr. Padilla), ocular disease (Dr. Ohara), hepato-splenomegaly (Dr. Judson), dermatologic disease (Dr. Yeager), musculoskeletal disease (Dr. Shorr), calcium metabolism (Dr. Rizzato), and rare forms of the disease (Dr. Lower). Discussion of treatment includes a chapter on corticosteroid therapy (Dr. Lynch) as well as corticosteroid alternatives (Drs. Baughman and Mihailovic-Vucinic). Dr. Judson completes this section with a chapter on transplant.

The last two chapters of the book deal with speculative areas of sarcoidosis. This includes a review by Dr. Teirstein who synthesizes information regarding the etiology of sarcoidosis. The final chapter by Drs. Baughman and Lower examines new directions in therapy.

This book reflects the work of all the contributors. It also would not be possible without the support ofDr. Claude Lenfant and Taylor & Francis. Adia Tucker assisted with the early phases of the book development. I particularly want to thank Sandra Beberman from Taylor & Francis who has worked with me from the beginning on this book and Vanessa Sanchez for assistance in completing the project. Finally, I would like to thank my wife, Dr. Elyse Lower, for her help over the twenty years of our sarcoidosis clinic, in which her knowledge and imagination helped us introduce new drugs for sarcoidosis.