Hereditary systemic autoinflammatory diseases and Schnitzler’s syndrome

Author:

Kacar Mark12,Pathak Shelly1,Savic Sinisa12ORCID

Affiliation:

1. National Institute for Health Research–Leeds Biomedical Research Centre and Leeds Institute of Rheumatic and Musculoskeletal Medicine, Leeds, UK

2. Department of Clinical Immunology and Allergy, St James’s University Hospital, Leeds, UK

Abstract

Abstract The systemic autoinflammatory diseases are disorders of the innate immune system distinguished by severe inflammation resulting from dysregulation of the innate immune system. Hereditary fever syndromes, such as FMF, TNF receptor-associated periodic syndrome, cryopyrin-associated periodic syndromes and mevalonate kinase deficiency, were the first group of systemic autoinflammatory diseases for which a genetic basis was established, between 1999 and 2001. Currently according to the latest report of the international union of immunological societies, 37 separate monogenic disorders were classified as autoinflammatory. In addition to the abovementioned monogenic conditions, we describe Schnitzler’s syndrome, a well-defined, acquired autoinflammatory condition without a clear genetic basis. For the purposes of this review, we discuss several conditions defined by the latest consensus process as systemic autoinflammatory diseases. We focus on those disorders where recent studies have contributed to further phenotypic characterization or had an impact on clinical management.

Funder

Sobi

Publisher

Oxford University Press (OUP)

Subject

Pharmacology (medical),Rheumatology

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