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108,67 €Preface.- 1 Genetics.- 2 Pathogenesis.- 3 Clinical Picture in Childhood.- 4 Clinical Picture in Adulthood and Unusual Clinical Features.- 5 FMF in Western Countries.- 6 Long Term Complications in FMF.- 7 Recent advances in quantitative assessment of FMF.- 8 How to Manage FMF Patients in Daily Practice.- 9 New Emerging Treatments.- Subject Index.
- Covers all aspects of familial Mediterranean fever
- Designed to meet the increasing interest in inherited
autoinflammatory disease and the needs of rheumatologists, clinical
immunologists, pediatricians and dermatologists
- Written by very well known opinion leaders in the field
This book, written by very well known opinion leaders in the field, covers all aspects of
familial Mediterranean fever, the most common monogenic autoinflammatory disease.
The opening chapters explain the genetic basis of the disease and provide insights into
the pathogenesis derived from recent experimental studies. A large part of the book is
then devoted to a detailed description of the typical and atypical clinical presentations,
the disease course, and potential complications in both pediatric and adult patients.
Guidance is provided on the measurement of disease severity and the management of
patients in daily practice. The advice regarding treatment is based on the best currently
available evidence and attention is also paid to important emerging treatments.
The book is part of Springer’s series Rare Diseases of the Immune System, which presents
recently acquired knowledge on pathogenesis, diagnosis, and therapy with the aim of
promoting a more holistic approach to these conditions. Monogenic autoinflammatory
diseases are hereditary disorders that are caused by single-gene defects in innate immune
regulatory pathways and are characterized by a clinical and biological inflammatory
syndrome in which there is limited, if any, evidence of autoimmunity. Familial
Mediterranean fever itself is due to a mutation in the MEFV gene, which codes for the
protein pyrin; it is characterized by periodic fever and episodes of painful inflammation in
the abdomen, chest, and joints. Familial Mediterranean Fever will be an invaluable source
of up-to-date information for all practitioners involved in the care of patients with the
disease.
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