Scleroderma is a diverse syndrome and multifaceted disease process. A veritable tidal wave of information on this subject has been published over the past two decades that has presented a unique challenge and opportunity to understand the data and be able to transform it to meaningful clinical algorithms and practice for our patients.
In this context, there is a major need for a comprehensive reference textbook on scleroderma. Scleroderma: From Pathogenesis to Comprehensive Management is eloquently written by more than 80 international expert authors, and edited by J. Varga, C.P. Denton and F.M. Wigley, reviewing the scientific and clinical aspects that shape this heterogeneous syndrome.
The book discusses in detail the most vex and serious complications including Raynauds phenomenon, ischaemic ulcers, gastrointestinal manifestations, cardiac abnormalities, pulmonary fibrosis, pulmonary hypertension and renal crisis. The authors also provide detailed discussion on the musculoskeletal complications, “overlooked” manifestations, and therapeutic and disease modulating management. With over 680 pages and an exhaustive reference list, the reader can easily navigate and learn about individual organ system manifestations, dysfunction and treatment strategies. The comprehensive format, however, lends the reader to occasional redundancy and overlap between sections; a flaw easily overlooked by the methodical dissection of each organ system. Finally, the use of appropriate figures aims to enhance the reader's understanding of the discussion and improve the applicability of the book to clinical practice.
The most significant challenge is our limited understanding of the underlying pathogenesis and genetic basis of this complex disease. The book steps up to this challenge and encompass the history of scleroderma discovery, characterisation of organ dysfunction and research mechanisms over the past 100 years. It outlines the clinical and pathological observations of the 1890s to 1960s, the discovery of fibroblasts, the extracellular matrix, growth factors and autoantibodies in the 1970s to 1980s, the development of animal models in the 1980s to 1990s, and, more recently, high- throughput discovery involving expression microarrays, candidate genes and biomarkers in the 2000s.
The book starts with chapters on the history of scleroderma, followed by the disease epidemiology, genetics, and classification. The chapters are followed by unique discussions on juvenile scleroderma and scleroderma-like disorders. The chapter on pathogenesis is well conceived, and divided into sections that deal with immunological and innate immunity, autoantibodies and biomarkers. The middle chapters detail an up-to-date discussion on the manifestations and management of organ- specific complications, including addressing treatment strategies and targeted therapies. The final chapter is dedicated to historical, current and future aspects of broad-based therapeutic therapies.
Scleroderma: From Pathogenesis to Comprehensive Management emphasises the multidisciplinary approach to comprehensive care of the scleroderma patient. The book serves as a valuable management guide and scientific review for both the “scleroderma- ologist”/clinician and the budding researcher seeking in-depth and authoritative discussions on all aspects of scleroderma. The book is recommended to any clinician who participates in the care of our patients with this complex, yet fascinating syndrome.
- ©ERS 2012
