By Marco Gattorno
This ebook, written by means of rather well recognized opinion leaders within the box, covers all elements of familial Mediterranean fever, the commonest monogenic autoinflammatory ailment. the outlet chapters clarify the genetic foundation of the illness and supply insights into the pathogenesis derived from fresh experimental reports. a wide a part of the publication is then dedicated to an in depth description of the common and odd medical shows, the sickness direction, and strength problems in either pediatric and grownup sufferers. assistance is supplied at the size of sickness severity and the administration of sufferers in day-by-day perform. the recommendation relating to therapy is predicated at the top at the moment on hand facts and a focus can be paid to special rising treatments.
The ebook is a part of Springer’s sequence Rare illnesses of the Immune System, which provides lately obtained wisdom on pathogenesis, analysis, and remedy with the purpose of selling a extra holistic method of those stipulations. Monogenic autoinflammatory ailments are hereditary problems which are as a result of single-gene defects in innate immune regulatory pathways and are characterised through a scientific and organic inflammatory syndrome within which there's restricted, if any, facts of autoimmunity. Familial Mediterranean fever itself is because of a mutation within the MEFV gene, which codes for the protein pyrin; it's characterised via periodic fever and episodes of painful irritation within the stomach, chest, and joints. Familial Mediterranean Fever might be a useful resource of updated info for all practitioners fascinated by the care of sufferers with the disease.