Book Review

The Granulomatous Disorders

N Engl J Med 2000; 343:74-75July 6, 2000

Article

The Granulomatous Disorders
Edited by D. Geraint James and Alimuddin Zumla. 616 pp., illustrated. New York, Cambridge University Press, 1999. $240. ISBN: 0-521-59221-6

The granulomatous disorders encompass a wide spectrum of common and rare afflictions that together impose a tremendous burden of disease. Common causes include infections such as tuberculosis and idiopathic disorders such as sarcoidosis and Crohn's disease. Undoubtedly, these latter disorders elicit interest in part because of their uncertain, but possibly infectious, origins. Over the past decade, use of the tools of molecular biology has led to major advances in our understanding of granulomatous disorders. However, the challenge remains for the clinician and scientist to understand the complex genetic, microbial, immunologic, and environmental factors that are responsible for the varied clinical manifestations and disparate outcomes of these disorders.

The Granulomatous Disorders, a beautifully illustrated and thoughtful textbook, provides a substantive overview of both infectious and idiopathic granulomatous disorders. The editors, authorities on sarcoidosis and infectious granulomatous diseases, have brought expert clinicians and pathologists together in a largely successful attempt to provide a seamless account of the clinical and pathological correlates of these diseases. Although the book was initially conceived to fill a widening gap between the extensive updates of the literature on sarcoidosis and the relatively few updates available for most other granulomatous disorders, its appeal resides not so much in its conceptual originality as in its exceptional readability. High-quality photographs, illustrations, and tables complement the well-written text and tables in chapters that provide concise overviews of specific granulomatous disorders and related topics. This is a true reference book, with the chapters organized in such a way that they can be read independently of one another.

The book contains four parts, with a total of 36 chapters. Specific disorders are grouped according to whether they have an infectious or noninfectious origin and then according to the body system. The references have generally been well chosen, with a combination of classic articles and up-to-date reports. The index is reasonably comprehensive for a reference book.

The first section of the book provides an excellent introduction to the biology and immunology, and the characteristic fibrosis, of granuloma formation, followed by a general classification scheme and sections on imaging, electron microscopy, and experimental models of granuloma formation. These chapters provide an important conceptual framework for considering clinically relevant pathogenetic pathways of specific granulomatous disorders discussed later in the book.

The second and largest part of the book reviews granulomatous disorders caused by known infectious agents. This section begins with an overview and is followed by chapters on microbiologic diagnosis, tuberculosis, leprosy, and specific fungal, parasitic, and helminthic granulomatous disorders. These chapters present a wealth of pertinent clinical and pathological information clearly, blending time-tested dogma with discussions relevant to clinical practice in the 21st century. For example, many chapters contain sections that review the role of these infections in human immunodeficiency virus disease and other immunocompromised states. Unfortunately, considering the overall importance of mycobacterial disease, there is relatively little discussion of the molecular biology, microbiology, and immunology of tuberculosis and leprosy — a rich body of knowledge that has emerged over the past decade. Although the fundamental pathogenetic mechanisms in these disorders are not yet known conclusively, relevant scientific findings merit more attention. The chapters on leishmaniasis and toxoplasmosis provide excellent syntheses of both current immunopathogenetic concepts and basic clinicopathological information.

The third section reviews several of the most common idiopathic granulomatous disorders, including sarcoidosis and granulomatous vasculitis. James's passion for sarcoidosis is evident as he encourages the reader to question old and new ideas about the causes of sarcoidosis. This stimulating and informal discourse enhances the basic clinical descriptions of the disease but could have been augmented by more information on the immunology of sarcoidosis, which is discussed in the first section. In a short chapter on granulomas in primary immunodeficiency diseases, the authors beautifully incorporate immunopathogenetic and clinicopathological information on common variable immunodeficiency and chronic granulomatous disease, both relatively rare disorders. The chapter on chronic beryllium disease is also remarkably successful, integrating recent advances in immunogenetics and clinicopathological aspects of this occupational disorder.

A strength of this book is the last section, which discusses granulomatous disorders according to the organ system. The usefulness of this section for clinicians and students is based on the fact that although most granulomatous diseases are multisystem disorders, the approach to diagnosis is usually organ-specific. By selecting a chapter according to the organ system, the reader can review the diagnostic possibilities and pitfalls of finding granulomas in a specific organ and find information about the critical clinical aspects of the underlying disorders. Although some of the material is redundant, the chapters on granulomas of the heart, central nervous system, skin, genital tract, and eye are particularly useful as references. The chapter on granulomas of the gut is noteworthy for its scholarly discussion of the pathogenesis and clinical features of Crohn's disease. The earlier chapters on bone marrow granulomas and granulomas associated with cancer also contain valuable information that is not often found in other textbooks; these chapters would naturally fit in this part of the book. Finally, a chapter on pediatric granulomas is likely to be useful to a general audience because of its selected review of granulomatous disorders that predominate in children rather than adults.

This book will be of considerable value to clinicians and students at all levels, providing them with a remarkably effortless way to approach a complex group of disorders. Scientists are likely to find the book a ready source of relevant clinicopathological information that can provide the foundation for investigations of the pathogenesis of granulomatous diseases. In brief, this handsome, eminently readable book is an excellent reference for general clinicians, specialists, students, and scientists facing the challenge of understanding and treating the granulomatous disorders.

David R. Moller, M.D.
Johns Hopkins University School of Medicine, Baltimore, MD 21224