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Preface

Interstitial Pulmonary and Bronchiolar Disorders provides a comprehensive review of clinical and investigative aspects of diverse interstitial lung diseases (ILDs) and bronchiolar inflammatory disorders. Immune-mediated mechanisms have been implicated as the cause of most of these diseases. However, the etiologic agents responsible for many of these lung and bronchiolar disorders remain enigmatic. Management of these diseases is difficult, owing to the rarity of these disorders and the lack of placebo-controlled therapeutic trials for many ILDs. Most practicing clinicians have inadequate personal experience to deal with these rare and diverse disorders with confidence. This book enlists internationally recognized experts to discuss controversies and evolving concepts in the management of diffuse ILDs and bronchiolar disorders. The first five chapters address an overall approach to ILDs (chap. 1 by Drs. Collard and King), and discuss in depth radiographic (chap. 2 by Dr. Lynch et al.), and histopathological (chap. 4 by Dr. Wallace et al.) features and patterns. In chapter 3, Drs. Woodhead and du Bois explore in detail the role of genetics in specific ILDs, and the importance of genetic polymorphisms in the clinical expression of diseases. Corticosteroids and immunosuppressive and cytotoxic agents are the cornerstone of therapy for many (but not all) of these disorders. These agents have potential serious toxicities, and many clinicians lack expertise with these agents. In chapter 5, Drs. Baughman and Lower provide a comprehensive review of the diverse agents utilized to treat ILDs, indications to treat, toxicities, and appropriate monitoring strategies.

All of the remaining chapters provide in-depth discussions of specific ILDs or bronchiolar disorders including epidemiology, pathogenesis, clinical features, and treatment. Within each chapter, histopathological and radiographic images (particularly computed tomographic) highlight the key features of the respective diseases. Chapters 6 to 11 review specific disorders manifesting granulomatous character. Three chapters are devoted to sarcoidosis. In chapter 6, Drs. Zissel, Prasse, and Müller-Quernheim elegantly discuss the epidemiology and immunopathogenesis of this enigmatic disorder. In chapter 7, Drs. Lynch, Fishbein, and White discuss the pulmonary manifestations of sarcoidosis. In chapter 8, Dr. Judson elegantly reviews the protean extrapulmonary manifestations of sarcoidosis. The remaining topics encompassed in the granulomatous section include hypersensitivity pneumonia (triggered by diverse organic and sometimes inorganic irritants/antigens) (chap. 9 by Dr. Selman et al.), berylliosis (a mimic of sarcoidosis caused by inhaled beryllium) (chap. 10 by Drs. Newman and Sackett), and other pneumoconioses (chap. 11 by Dr. Brody). The third section in the book (chaps. 12-18) reviews the idiopathic interstitial pneumonias and pulmonary complications of connective tissue disorders (CTDs). Separate chapters are included for idiopathic pulmonary fibrosis (Dr. Lynch et al.), nonspecific interstitial pneumonia (Dr. Flaherty), respiratory bronchiolitis interstitial lung disease and desquamative interstitial pneumonia (Dr. Ryu), acute interstitial pneumonia (Drs. Vourlekis and Brown), and lymphocytic interstitial pneumonia (Drs. Koss and Shigemitsu). In chapter 17, Dr. Nunes and colleagues provide an elegant and comprehensive review of ILDs complicating CTDs. In chapter 18, Dr. Strange reviews the other diverse pleuropulmonary complications of CTDs not encompassed in chapter 17. The fourth section in the book discusses diverse bronchiolar disorders (some idiopathic, some due to well-recognized causes). Organizing pneumonia (formerly termed bronchiolitis obliterans organizing pneumonia) and obliterative bronchiolitis (OB) exhibit striking differences in prognosis and responsiveness to therapy. Hence, these disorders are discussed separately by Dr. Lazor et al. and Dr. Poletti et al. in chapters 19 and 20, respectively. Additional chapters focus on the devastating complications of OB in the context of lung or heart-lung (Dr. Verleden et al.) and hematopoietic stem cell transplantation (Drs. Afessa and Peters). Recent data linked inhalation exposure among rescue personnel in New York City to diverse pulmonary and bronchiolar disorders (discussed in depth in chap. 23 by Dr. Prezant et al.). The fifth section of the book discusses vasculitic syndromes that may affect the lung. The initial chapter in this section (chap. 24 by Drs. Jennette and Falk) provides an in-depth view of epidemiology and pathogenesis of ANCA-associated vasculitides (AAV). The following chapters discuss Wegener's granulomatosis (Dr. Silva et al.), Churg-Strauss Vasculitis (Dr. Guilpain et al.), and microscopic polyangiitis (Drs. Salama and Pusey). Each of these AAV has overlapping characteristics yet differ in important respects articulated in the individual chapters. In Chapter 28, Drs. Biddinger and Panos review anti-glomerular basement membrane disease, a rare but important cause of pulmonary hemorrhage and renal failure that mimics pulmonary vasculitis. Finally, Drs. Hajj-Ali and Langford discuss Behçet's disease, an uncommon cause of pulmonary vasculitis with marked variability in prevalence in different countries/regions worldwide. The final section in the book discusses what have been termed "orphan lung disorders" and includes chapters on eosinophilic pulmonary disorders (Dr. Cottin et al.), Langerhans cell histiocytosis (Dr. Vassallo et al.), lymphangioleiomyomatosis (Dr. McCormack), pulmonary alveolar proteinosis (Dr. Wang et al.), amyloidosis (Dr. Berk), and drug-induced pulmonary disorders (Drs. Maldonado and Limper).

This book assembles the best international experts in ILD and bronchiolar disorders and provides a global perspective of the current and future management of these rare and often puzzling disorders. Each of these chapters not only comprehensively outlines the salient clinical features of these diverse ILD and bronchiolar disorders but also reviews in depth the pathogenic mechanisms of these disorders, and the role of current and novel therapies. The bibliography is extensive, allowing ready access to the sentinel and original articles in the field. The book will be of great interest and value to pulmonologists, rheumatologists, immunologists, allergists, pathologists, and radiologists, as well as basic scientists with an interest in immunologically mediated pulmonary and bronchiolar disordres.