Results 1 to 10 of about 148,918 (213)

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
doaj   +1 more source

Somatic Mutations and the Risk of Undifferentiated Autoinflammatory Disease in MDS: An Under-Recognized but Prognostically Important Complication

Frontiers in Immunology, 2021
Objectives: We theorized that myelodysplastic syndrome (MDS) with somatic mutations and karyotype abnormalities are associated with autoinflammation, and that the presence of autoinflammatory disease affected prognosis in MDS.Methods: One hundred thirty ...
Abdulla Watad, Abdulla Watad, Mark Kacar, Mark Kacar, Nicola Luigi Bragazzi, Qiao Zhou, Qiao Zhou, Miriam Jassam, Jan Taylor, Eve Roman, Alexandra Smith, Richard A. Jones, Howard Amital, Catherine Cargo, Dennis McGonagle, Sinisa Savic, Sinisa Savic   +16 more
doaj   +1 more source

The spectrum of clinical presentation in haploinsufficiency of A20; a case report of a novel mutation in TNFAIP3 gene

Frontiers in Pediatrics, 2023
Haploinsufficiency of A20 was first described in 2016 as a new autoinflammatory disease that clinically presents as early-onset Behcet's disease. After the publication of the first 16 cases, more patients were diagnosed and described in the literature ...
M. Debeljak, M. Debeljak, S. Blazina, J. Brecelj, J. Brecelj, T. Avčin, T. Avčin, N. Toplak, N. Toplak   +8 more
doaj   +1 more source

Long-Term Outcomes of Behçet’s Syndrome-Related Uveitis: A Monocentric Italian Experience

Mediators of Inflammation, 2020
Objectives. To examine demographic and clinical characteristics and long-term visual outcome in a cohort of Italian patients affected by Behçet’s uveitis (BU). Materials and Methods.
Jurgen Sota, Luca Cantarini, Antonio Vitale, Arianna Sgheri, Stefano Gentileschi, Valeria Caggiano, Viviana Gelardi, Bruno Frediani, Gian Marco Tosi, Claudia Fabiani   +9 more
doaj   +1 more source

Real-Life Data on the Efficacy of Canakinumab in Patients with Adult-Onset Still’s Disease

Mediators of Inflammation, 2020
Background. Interleukin-1 inhibition has revealed to be a successful treatment approach for patients with adult-onset Still’s disease (AOSD). However, real-life experience is focused on the use of anakinra, while data about canakinumab (CAN) are mainly ...
Antonio Vitale, Virginia Berlengiero, Jurgen Sota, Luisa Ciarcia, Nicola Ricco, Sara Barneschi, Mariam Mourabi, Giuseppe Lopalco, Chiara Marzo, Francesca Bellisai, Florenzo Iannone, Bruno Frediani, Luca Cantarini   +12 more
doaj   +1 more source

Interleukin‐18 signaling promotes activation of hepatic stellate cells in mouse liver fibrosis

Hepatology, EarlyView., 2022
Interleukin‐18 signaling promotes activation of hepatic stellate cells in mouse liver fibrosis. Abstract Background and Aims Nucleotide‐binding oligomerization domain‐like receptor‐family pyrin domain‐containing 3 (NLRP3) inflammasome activation has been shown to result in liver fibrosis.
Jana Knorr, Benedikt Kaufmann, Maria Eugenia Inzaugarat, Theresa Maria Holtmann, Lukas Geisler, Jana Hundertmark, Marlene Sophia Kohlhepp, Laela M. Boosheri, Daisy R. Chilin‐Fuentes, Amanda Birmingham, Kathleen M. Fisch, Joel D. Schilling, Sven H. Loosen, Christian Trautwein, Christoph Roderburg, Münevver Demir, Frank Tacke, Hal M. Hoffman, Ariel E. Feldstein, Alexander Wree   +19 more
wiley   +1 more source

Autoinflammatory Diseases in Childhood

Balkan Medical Journal, 2020
Autoinflammatory diseases are characterized by recurrent fevers and clinical findings of impaired natural immunity and can involve various organ systems. The concept of autoinflammatory disease emerged after the definition of familial Mediterranean fever
Mehmet Yıldız, Fatih Haşlak, Amra Adrovic, Kenan Barut, Özgür Kasapçopur   +4 more
doaj   +1 more source

Designation of Autoinflammatory Skin Manifestations With Specific Genetic Backgrounds

Frontiers in Immunology, 2020
“Autoinflammatory disease (AiD)” has first been introduced in 1999 when the responsible gene for the familial Hibernean fever or autosomal dominant-type familial Mediterranean fever-like periodic fever syndrome was reportedly identified as tumor necrosis
Nobuo Kanazawa
doaj   +1 more source

Autoinflammatory diseases: what’s behind them and what’s new. A review

Dermatology Reports, 2022
Autoinflammatory diseases are characterized by bouts of systemic or localized inflammation in the absence of an infection. While some autoinflammatory diseases are caused by a single gene mutation, others have been shown to be multifactorial, involving ...
Michele Maalouly, Serena Saade, Mazen Kurban   +2 more
doaj   +1 more source

Toward an Inclusive, Congruent, and Precise Definition of Autoinflammatory Diseases

Frontiers in Immunology, 2017
Autoinflammatory disease was introduced as a concept in 1999, demarcating an entirely new group of diseases in clinical, immunological, and conceptual terms.
Per Wekell, Per Wekell, Stefan Berg, Stefan Berg, Anna Karlsson, Anders Fasth, Anders Fasth   +6 more
doaj   +1 more source