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This volume aims at delivering the state-of-the-art scientific and clinical know-how for the management of patients with Connective Tissue Diseases (CTDs). With contributions from leading specialists from different disciplines, it presents the latest research findings on many aspects of the diseases in a clear, comprehensive way, and describes the most recent trends in treatment, such as the “treat to target” approach. Both basic science and clinical medicine are addressed, with emphasis on the most promising clinical and laboratory-based studies. The coverage is comprehensive in scope, and includes epidemiology, pathogenesis, autoantibodies and biomarkers, disease manifestations, involvement of different organs or systems, the relationships with other disorders, and biological therapy. Readers will find this second volume to be an excellent source of information on the current understanding of CTDs, and clinical approach to CTD, clearly conveying the progress made in recent years. Connective Tissue Disease – A Comprehensive Guide Vol.2 will be an invaluable source of up-to-date information for all practitioners involved in the care of patients with these complex diseases.

In this book, detailed information on the nosology, pathology, pathogenesis, clinical presentation, diagnosis and treatment of large- and medium-sized vessel and single-organ vasculitis is provided and critically discussed by the most expert physicians and researchers in the field. Among the conditions considered are giant cell arteritis, Takayasu arteritis, polyarteritis nodosa, primary central nervous system vasculitis, isolated aortitis, isolated gastrointestinal vasculitis, cutaneous vasculitis and isolated genitourinary vasculitis. The role of histopathology in the diagnosis and prognosis of these vasculitis is evaluated, along with the part played by imaging studies in diagnosing and monitoring these diseases, while indications and limitations of the available imaging modalities are discussed as well. The expanding role of biological agents for the treatment of the large vessel vasculitis is addressed, as well as the current approaches to these diseases. This book will be a valuable companion in decision-making for medical practitioners, internists, specialists, researchers and postgraduate students interested in the intriguing fields of vasculitis and rare diseases.

Systemic and nervous system vasculitides are a heterogeneous group of related disorders, each characterized by vascular inflammation such that they have the potential to cause serious morbidity and mortality if unrecognized and therefore untreated. Systemic vasculitis affect all populations and every nationality and walk-of-life, from childhood to older age. The first edition of The Vasculitides, published in 2014, met the urgent need for a clear, concise, and reliable textbook regarding the epidemiology, pathogenesis, clinical presentation, laboratory evaluation and management of these disorders, assembling participants of the 16th International Vasculitis and ANCA Workshop in Paris, France. Five years later, two subsequent meetings have taken place in London and Tokyo. Participants of the 19th International Vasculitis and ANCA Workshop will gather in April 2019 at the University of Pennsylvania for an exceptional venue to share translational scientific discoveries, data from clinical trials, and advances in the clinical assessment, pathophysiology, genetic biomarkers, standard-of-care, and novel therapies of vasculitis. The Workshop participants regathered to update the The Vasculitides, producing an even more cutting-edge two-volume set of books. This is the first book in the set, covering general considerations and systemic vasculitis.

This book provides detailed information on the nosology, pathology, pathogenesis, clinical presentation, diagnosis and treatment of fibro-inflammatory disorders, rare diseases that often display systemic organ involvement. Among the conditions considered are IgG4-related disease, sclerosing cholangitis, Hashimoto's and Riedel's thyroiditis, retroperitoneal fibrosis/chronic periaortitis, mediastinal fibrosis, Erdheim-Chester disease, gadolinium-induced fibrosis, and sclerosing mesenteritis. This group of entities is still poorly defined and is characterized by the common denominator of chronic inflammatory infiltrate admixed with abundant fibrosis. IgG4-related disease is the prototypical example. Systemic Fibroinflammatory Disorders is the first book to draw together information on these conditions. As these diseases often require an interdisciplinary approach, the book is addressed to specialists of different disciplines, especially internists, rheumatologists, nephrologists, clinical immunologists, and hematologists.

Guest edited by Jonathan Kay, this issue of Rheumatic Disease Clinics will cover the latest research and evidence surrounding the diagnosis, treatment and management of rarely seen rheumatic diseases.