Pattern and diagnostic evaluation of systemic autoinflammatory diseases other than familial Mediterranean fever among Arab children: a multicenter study from the Pediatric Rheumatology Arab Group (PRAG)
Sign Up to like & getrecommendations! Published in 2019 at "Rheumatology International"
DOI: 10.1007/s00296-019-04478-3
Abstract: To define the spectrum and phenotypic characteristics of systemic autoinflammatory diseases (SAIDs) other than familial Mediterranean fever (FMF) in Arab children and to delineate diagnostic evaluation. Data retrospectively collected on patients with clinical and/or genetically… read more here.
Keywords: diagnostic evaluation; familial mediterranean; systemic autoinflammatory; autoinflammatory diseases ... See more keywords
Comparison of the clinical diagnostic criteria and the results of the next-generation sequence gene panel in patients with monogenic systemic autoinflammatory diseases
Sign Up to like & getrecommendations! Published in 2020 at "Clinical Rheumatology"
DOI: 10.1007/s10067-020-05492-8
Abstract: The clinicians initially prefer to define patients with the systemic autoinflammatory disease (SAID)’s based on recommended clinical classification criteria; then, they confirm the diagnosis with genetic testing. We aimed to compare the initial phenotypic diagnoses… read more here.
Keywords: diagnosis; autoinflammatory diseases; clinical diagnostic; panel ... See more keywords
The monogenic autoinflammatory diseases define new pathways in human innate immunity and inflammation
Sign Up to like & getrecommendations! Published in 2017 at "Nature Immunology"
DOI: 10.1038/ni.3777
Abstract: Autoinflammatory diseases were first recognized nearly 20 years ago as distinct clinical and immunological entities caused by dysregulation in the innate immune system. Since then, advances in genomic techniques have led to the identification of… read more here.
Keywords: diseases define; monogenic autoinflammatory; autoinflammatory diseases; new pathways ... See more keywords
TNF receptor signalling in autoinflammatory diseases.
Sign Up to like & getrecommendations! Published in 2019 at "International immunology"
DOI: 10.1093/intimm/dxz024
Abstract: Autoinflammatory syndromes are a group of disorders characterised by recurring episodes of inflammation as a result of specific defects in the innate immune system. Patients with autoinflammatory disease present with recurrent outbreaks of chronic systemic… read more here.
Keywords: receptor signalling; signalling autoinflammatory; tnf receptor; autoinflammatory diseases ... See more keywords
Diagnostic and therapeutic algorithms for monogenic autoinflammatory diseases presenting with recurrent fevers among adults.
Sign Up to like & getrecommendations! Published in 2022 at "Rheumatology"
DOI: 10.1093/rheumatology/keac712
Abstract: Autoinflammatory diseases (AIDs) are defined as disorders of innate immunity. They were initially defined in contrast to autoimmune diseases because of the lack of involvement of the adaptive immune system and circulating autoantibodies. The four… read more here.
Keywords: algorithms monogenic; autoinflammatory diseases; monogenic aids; diagnostic therapeutic ... See more keywords
Vasculitis in the autoinflammatory diseases
Sign Up to like & getrecommendations! Published in 2017 at "Current Opinion in Rheumatology"
DOI: 10.1097/bor.0000000000000347
Abstract: Purpose of review This article addresses the prevalence and relationship between autoinflammatory diseases and vasculitis. Recent findings Autoimmune diseases (AIDs) are a group of syndromes characterized by episodes of unprovoked inflammation due to dysregulation of… read more here.
Keywords: vasculitis; autoinflammatory diseases; diseases vasculitis; vasculitis autoinflammatory ... See more keywords
Diagnostic value of skin biopsy in autoinflammatory diseases for patients with recurrent fever and urticarial eruption
Sign Up to like & getrecommendations! Published in 2020 at "Clinical and Experimental Dermatology"
DOI: 10.1111/ced.14508
Abstract: Recurrent fever is defined by two episodes of fever separated by a free interval of at least two weeks.1 Diseases causing recurrent fever may be classified into three categories: infections, neoplasia, and non-infectious inflammations. Recurrent… read more here.
Keywords: recurrent fever; urticarial eruption; diagnostic value; autoinflammatory diseases ... See more keywords
Autoimmune and autoinflammatory diseases with mucocutaneous manifestations: A pediatric rheumatology perspective
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DOI: 10.1111/ijd.16352
Abstract: The presence of mucocutaneous manifestations has clinical significance, as it may be a part of the initial presentation or activation stage of both autoimmune and autoinflammatory rheumatic diseases. The cutaneous signs may display a particular… read more here.
Keywords: mucocutaneous manifestations; rheumatology; rheumatic diseases; dermatology ... See more keywords
Novel use of Autoinflammatory Diseases Activity Index (AIDAI) captures skin and extracutaneous features to help manage pediatric DITRA: A case report and a proposal for a modified disease activity index in autoinflammatory keratinization disorders
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DOI: 10.1111/pde.14155
Abstract: Generalized pustular psoriasis (GPP) is a severe form of psoriasis, which is rare in pediatric and adult patients. It is characterized by sterile pustular lesions that appear on erythematous skin, associated with systemic features. A… read more here.
Keywords: activity index; disease activity; activity; autoinflammatory diseases ... See more keywords
Consensus proposal for taxonomy and definition of the autoinflammatory diseases (AIDs): a Delphi study
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DOI: 10.1136/annrheumdis-2017-212515
Abstract: Autoinflammatory diseases (AIDs) are a relatively new family of disorders, defined about 19 years ago. Some of them are hereditary and some are not. The names given to these diseases do not follow any systematic… read more here.
Keywords: definition; study; autoinflammatory diseases; aids delphi ... See more keywords
Clinical and multi-omics cross-phenotyping of patients with autoimmune and autoinflammatory diseases: the observational TRANSIMMUNOM protocol
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DOI: 10.1136/bmjopen-2017-021037
Abstract: Introduction Autoimmune and autoinflammatory diseases (AIDs) represent a socioeconomic burden as the second cause of chronic illness in Western countries. In this context, the TRANSIMMUNOM clinical protocol is designed to revisit the nosology of AIDs… read more here.
Keywords: transimmunom; clinical multi; autoimmune autoinflammatory; autoinflammatory diseases ... See more keywords