Textbook of systemic vasculitis

The systemic vasculitides are a group of multisystem diseases characterized by inflammation of blood vessels. The diagnosis of vasculitis is a challenge because its presentations are heterogeneous in severity and organ distribution and there is a lack of diagnostic criteria. Yet, the consequences of a missed or delayed diagnosis are potentially severe. Systemic vasculitis is not common but certainly underdiagnosed and under-reported. Varied clinical presentations of these conditions, better understanding of their pathogenesis and recent advances in managing these disorders necessitate a single desktop reference. The book covers the entire field of systemic vasculitis and is unique as it has contributions from international and national leaders in the field.

The book is divided into six sections, beginning with an introduction to the condition and its basic science, followed by sections on imaging, manifestations of vasculitis, principles of management, and a final section on a range of individual vasculitis syndromes. Each chapter is summarized in the end with ‘key points’. The first section deals with the epidemiology and the genetics involved in the pathogenesis of vasculitis. This section also has a chapter on classification of vasculitis including revised Chapel Hill (CHCC 2012) classification and paediatric classification of vasculitis. This second section focuses on the basic science of vasculitis and includes a detailed description on anti-neutrophil cytoplasmic antibodies (ANCAs) and the role of neutrophils in its pathogenesis. The understanding of vasculitis can never be complete without pathology; four chapters in the second section have been dedicated to the pathology of skin, lungs, kidney, and nerves and brain respectively. The pathological changes in different organs are described in detail with gross and microscopic pictures and will be of great help for both the clinicians as well as the pathologist.

The third section deals with imaging modalities having an important role in diagnosing the disease and assessment of disease activity including a chapter on role of positron emission tomography (PET) in vasculitis. The fourth section is dedicated to organ-specific clinical manifestation of vasculitis. These are covered in great detail and will be of interest to rheumatologists, specialists dealing with the management of these disorders namely dermatologists, ophthalmologists, cardiologists, pulmonologists, nephrologists, neurologists, gastroenterologists and rhino-otolaryngological specialists, as well as postgraduate students in these departments. The fifth section deals with the newer and future management of vasculitis, approach to vasculitis in paediatric and adult population, and the tools to assess the activity and damage of the disease. The final section has chapters on individual vasculitis syndromes.

Strengths of the book include case vignettes, exquisite clinical photographs of the protean manifestations, flow diagrams and summary panels for management. A key feature of the work-up of a potential vasculitis patient is the exclusion of mimics and secondary causes of vasculitis. Chapters on vasculitis mimics, secondary vasculitis, infection and malignancy-associated vasculitis are also included. A chapter on vasculitis and pregnancy gives insight into the pregnancy outcomes and concerns of vasculitis.

The book is written clearly with consistent and detailed coverage of the subject and is recommended as a desktop reference for all the specialties that deal with the management of these diseases. The only limitations in this otherwise excellent book are a rather lengthy focus on each of the topic with short focus on future advances. Issues related to functional genomics and pharmacogenomics of various vasculitides syndromes are important and should be incorporated in future editions.