When my friend and colleague Lorenzo Emmi asked me to act as Editor for a book on Familial Mediterranean Fever in the context of a trilogy dedicated to autoinfl ammatory syndromes, I was really honoured but also a bit nervous. Indeed, the challenging idea to collect in a single book all the enormous amount of knowledge related to the “Big Mother” of all autoinfl ammatory diseases was really stimulating to me. On the other hand, I immediately realized that a relevant number of my colleagues, practically all the people involved in the present book and many others, had the right to act as the Editor of such a challenge much more than me. Indeed most of them literally wrote the history of this disease and in a sense of the whole expanding spectrum of autoinfl ammatory diseases.
Isabelle Touitou and Dan Kastner are the true pioneers in the identifi cation of genes involved in the rheumatic conditions transmitted in a Mendelian fashion. Indeed their identifi cation of MEFV gene (or marenostrin) in 1997 was the seminal step of the fascinating history of autoinfl ammatory disease. In her chapter, Isabelle Touitou and Guillaume Sarrabay describe all the secrets concerning the genetic aspects of this condition, whereas Dan Kaster, with the precious help of JJ Chae, gives us a brilliant and updated overview of all the advancements and the still unsolved questions related to the pathogenesis of the disease. Avi Livneh with his co-workers, Eldad Ben-Chetrit and Helen Lachmann share with us their outstanding clinical experience in the diagnosis and management of FMF from their perspective of adult rheumatologists and experts in amyloidosis. For the same reason, I am in debt with my dear colleagues, pediatric rheumatologists Seza Ozen, Tilo Kallinich and Huri Özdoğan, for their fantastic contribution in the description of the condition in children and in the illustration of the novel therapeutic approaches with biologics.
Finally, I am really grateful to Raffaele Manna and Erkan Demirkaya for their excellent contribution on epidemiology of FMF around the world and the description of available tools for the practical monitoring of disease activity and severity in everyday clinical practice. I am in debt to all these outstanding colleagues for their generous and extremely competent availability to be part of this project. This book defi nite is a tribute to their work on FMF during all these years.
