The diagnosis and management of diffuse lung disease (DLD) remain problematic for clinicians, including pulmonologists. Although specific patterns seen in a patient may lead quickly to a diagnosis, many patients present with a constellation of features that is more complex.
This book is a practical aid to the physician faced with making difficult diagnoses in their own clinical setting. It opens with an introductory section covering general considerations - patient evaluation, including history and clinical signs, investigations, DLD classification according to the current guidelines, and therapeutic options. This is followed by a section devoted to specific DLDs, including sarcoidosis, idiopathic pulmonary fibrosis, occupational and iatrogenic DLDs, and other, less common, disorders. Within each topic, internationally renowned authors with specific expertise in the area have covered genetic aspects and epidemiology, presentation, diagnosis, and clinical management. Coverage concludes with a chapter of case histories to illustrate differing approaches to diagnosis and management, a feature unique to this text and emphasizing its clinical applicability. The text is supported and enhanced throughout with line diagrams, management algorithms, and high quality clinical photographs.
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Robert P. Baughman is Professor of Medicine, Pulmonary / Critical Care Division, University of Cincinnati Medical Center, Cincinnati, OH, USA
Roland M. du Bois is Professor of Respiratory Medicine and Head of the Interstitial Lung Disease & Clinical Genomics Groups at the Royal Brompton Hospital and National Heart & Lung Institute, Imperial College of Science, Technology & Medicine, London, UK
Joseph P. Lynch is Professor of Clinical Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA
Athol U. Wells is Respiratory Physician, Interstitial Lung Disease Unit, Royal Brompton Hospital, Chelsea, London, UK
The lung is exceptional in that pathologic changes can be seen clearly in radiographs because of the air-tissue contrast. When bilateral diffuse, ill-defined shadows are seen, one begins to speculate on the cause -- infection, inflammation, or neoplasm? This process is one of the most exciting by which chest physicians approach a diagnosis. As a practical guide, Diffuse Lung Disease provides current and approved approaches to diffuse lung diseases caused by complex inflammatory mechanisms. The book has two parts -- one on general considerations and one on specific diseases, which includes 11 case presentations. During the creation of a consensus statement on idiopathic pulmonary fibrosis in the late 1990s, the notion was established that the diagnosis of diffuse lung diseases should be collectively interpreted according to the clinical findings (crucial in the recording of patient history), radiologic results (obtained with the use of high-resolution computed tomography [CT]), and pathological findings (from biopsy specimens obtained during thoracoscopic surgery). Consistent with this notion, the book offers plenty of typical high-resolution CT scans and beautiful photomicrographs of pulmonary-biopsy specimens. In contrast to these diagnostic procedures, the reader may find that the therapeutic approaches to diffuse lung diseases are limited. Conventional immunosuppressive agents are the principal treatments for pulmonary fibrosis, granulomas, or vasculitis, although the effects are not always satisfactory. However, macrolides have been found to be effective in chronic bronchiolitis, and rapamycin may target lymphangioleiomyomatosis. New monoclonal antibodies are promising agents for future clinical trials. The implications with regard to the causes of diffuse lung diseases are surprisingly broad. For example, the common use of down clothing or bedding should be considered as a possible cause of hypersensitivity pneumonitis and the use of supplements such as vitamins as a possible cause of organizing pneumonia. Although it is not covered in this book, a recent example is the relatively high mortality associated with interstitial lung disease in patients taking gefitinib, a drug used in the treatment of non-small-cell lung cancer. For a better understanding of diffuse lung diseases, a clarification of the causes, in molecular terms, is needed. One example in this book involves pulmonary alveolar proteinosis. An autoantibody against granulocyte-macrophage colony-stimulating factor has been implicated in the causation of idiopathic pulmonary alveolar proteinosis, and treatment for B-cell diseases thus could be considered for this condition. For the global search for genes related to diffuse lung diseases, precise family histories are indispensable. For this purpose, high-resolution CT scanning in asymptomatic siblings of patients with diffuse or destructive lung diseases could provide crucial information. This book concisely summarizes our current understanding of diffuse lung diseases and will be a valuable guide to physicians in the examination of bilateral diffuse pulmonary shadows in chest radiographs. Toshihiro Nukiwa, M.D., Ph.D.
Copyright © 2005 Massachusetts Medical Society. All rights reserved. The New England Journal of Medicine is a registered trademark of the MMS.
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