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Systemic autoinflammatory diseases
Journal of Autoimmunity, 2020 Systemic autoinflammatory diseases (SAIDs) are a growing group of disorders caused by a dysregulation of the innate immune system leading to episodes of systemic inflammation. In 1997, MEFV was the first gene identified as disease causing for Familial Mediterranean Fever, the most common hereditary SAID.
Krainer, Julie +2 more
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Autoinflammatory Bone Diseases [PDF]
Rheumatic Disease Clinics of North America, 2013 Autoinflammatory bone disease is a new branch of autoinflammatory diseases caused by seemingly unprovoked activation of the innate immune system leading to an osseous inflammatory process. The inflammatory bone lesions in these disorders are characterized by chronic inflammation that is typically culture negative with no demonstrable organism on ...
Sara M, Stern, Polly J, Ferguson
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Dermatologic and Dermatopathologic Features of Monogenic Autoinflammatory Diseases
Frontiers in Immunology, 2019 Autoinflammatory diseases include disorders with a monogenic cause and also complex conditions associated to polygenic or multifactorial factors. An increased number of both monogenic and polygenic autoinflammatory conditions have been identified during ...
Ignasi Figueras-Nart +3 more
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Macroglobulinemia and autoinflammatory disease [PDF]
Rheumatology and Immunology Research, 2021 Abstract Macroglobulinemia is associated with Schnitzler syndrome (SchS) and Waldenstrom macroglobulinemia (WM). The aim of this article was to review the above-mentioned two diseases from clinical aspects and their potential genetic links. We performed a PubMed search using the following keywords: “SchS,” “WM,” “autoinflammatory disease,
Navetta-Modrov, Brianne, Yao, Qingping
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Rare Autoinflammatory Diseases
Turkish Archives of Pediatrics, 2021 Systemic autoinflammatory diseases are disorders caused by dysregulation of the innate immune system leading to systemic inflammation. Since the first gene had been identified causing Familial Mediterranean Fever, the most common hereditary systemic autoinflammatory disease, advances in genomic techniques and awareness of the diseases have led to ...
Başaran, Özge +2 more
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Elucidation of the Pathogenesis of Autoinflammatory Diseases Using iPS Cells
Children, 2021 Autoinflammatory diseases are a disease entity caused by the dysregulation of innate immune cells. Typical autoinflammatory diseases are monogenic disorders and often very rare.
Megumu K. Saito
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Autoinflammatory keratinization diseases [PDF]
Journal of Allergy and Clinical Immunology, 2017 Inflammation caused by the hyperactivation of innate immunity due to genetic factors occasionally leads to inflammatory cutaneous keratinization diseases. Such inflammatory keratinization diseases with genetic autoinflammatory mechanisms are called “autoinflammatory keratinization diseases” (AiKDs). We proposed a four-part definition of AiKDs.
Akiyama, Masashi +3 more
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The Use of Interleukine-1 Inhibitors in Familial Mediterranean Fever Patients: A Narrative Review
Frontiers in Immunology, 2020 Purpose: Familial Mediterranean fever (FMF) is the most common monogenic auto-inflammatory disease characterized by recurrent attacks of fever and serositis.
Véronique Hentgen +3 more
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Bridging autoinflammatory and autoimmune diseases
The Egyptian Journal of Internal Medicine, 2021 Background Autoimmunity is used to cause by impairment of adaptive immunity alone, whereas autoinflammatory was originally defined as a consequence of unregulated innate immunity.
Emad M. El-Shebiny +3 more
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Monogenic autoinflammatory diseases [PDF]
Rheumatology, 2014 During the past 15 years, a growing number of monogenic inflammatory diseases have been described and their respective responsible genes identified. The proteins encoded by these genes are involved in the regulatory pathways of inflammation and are mostly expressed in cells of the innate immune system.
Ricardo A G, Russo, Paul A, Brogan
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