Rare Diseases of the Immune System

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.