Abstract
The systemic autoinflammatory disorders (SAIDs) or periodic fever syndromes are disorders of innate immunity, which can be inherited or acquired. They are almost all very rare and easily overlooked; typically, patients will have seen multiple specialities prior to diagnosis, so a high level of clinical suspicion is key. It is important to note that these are 'high-value' diagnoses as the majority of these syndromes can be very effectively controlled, dramatically improving quality of life and providing protection against the development of irreversible complications such as AA amyloidosis. In this article, we take an overview of SAIDs and look at the common features; in Part 2, we take a more in-depth look at the better recognized or more dermatologically relevant conditions.
© 2020 The Authors. Clinical and Experimental Dermatology published by John Wiley & Sons Ltd on behalf of British Association of Dermatologists.
MeSH terms
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Amyloidosis / etiology
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Amyloidosis / pathology
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Amyloidosis / prevention & control*
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Cryopyrin-Associated Periodic Syndromes / diagnosis
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Cryopyrin-Associated Periodic Syndromes / metabolism
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Cryopyrin-Associated Periodic Syndromes / pathology
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Dermatologists / statistics & numerical data*
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Diagnosis, Differential
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Familial Mediterranean Fever / diagnosis
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Familial Mediterranean Fever / metabolism
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Familial Mediterranean Fever / pathology
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Fever / diagnosis
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Fever / metabolism
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Fever / pathology
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Genetic Testing / standards
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Hereditary Autoinflammatory Diseases / diagnosis
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Hereditary Autoinflammatory Diseases / immunology*
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Hereditary Autoinflammatory Diseases / metabolism
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Hereditary Autoinflammatory Diseases / pathology
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Humans
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Immune System Diseases / complications
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Immune System Diseases / diagnosis
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Immune System Diseases / immunology*
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Immune System Diseases / pathology
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Immunity, Innate / immunology
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Inflammation / immunology
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Inflammation / metabolism
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Inflammation / pathology
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Interleukin-1beta / immunology
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Interleukin-1beta / metabolism
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Mevalonate Kinase Deficiency / diagnosis
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Mevalonate Kinase Deficiency / metabolism
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Mevalonate Kinase Deficiency / pathology
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Quality of Life
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Severity of Illness Index
Supplementary concepts
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Periodic fever, familial, autosomal dominant