Results 21 to 30 of about 27,174 (202)

A purine metabolic checkpoint that prevents autoimmunity and autoinflammation. [PDF]

, 2021
Still's disease, the paradigm of autoinflammation-cum-autoimmunity, predisposes for a cytokine storm with excessive T lymphocyte activation upon viral infection. Loss of function of the purine nucleoside enzyme FAMIN is the sole known cause for monogenic

core   +3 more sources

Living with autoinflammatory diseases: identifying unmet needs of children, adolescents and adults

Pediatric Rheumatology Online Journal, 2018
Background Autoinflammatory diseases (AIDs) illnesses of the innate immunity resulting in clinical signs and symptoms of systemic inflammation and loss of organ functions.
Gabriele Erbis, Kirstin Schmidt, Sandra Hansmann, Tetiana Sergiichuk, Christine Michler, Jasmin B. Kuemmerle-Deschner, Susanne M. Benseler   +6 more
doaj   +1 more source

Modern concept of autoimmunity in rheumatology

Научно-практическая ревматология, 2023
Two fundamental pathologic processes are central to the spectrum of chronic inflammation mechanisms: autoimmunity and autoinflammation. Autoimmunity and autoinflammation are mutually potent pathologic processes; their development is considered within the
E. L. Nasonov
doaj   +1 more source

Still's Disease and Autoinflammation: Positioning an Inflammatory Syndrome on the Autoinflammation-Autoimmunity Spectrum. [PDF]

Curr Rheumatol Rep
Abstract Purpose of this Review Still’s disease exemplifies systemic inflammatory disorders existing on a continuum between autoinflammation and autoimmunity. This review examines Still’s disease through this spectrum lens, integrating recent advances in pathogenesis, clinical heterogeneity ...
Pietsch D, Savic S.
europepmc   +3 more sources

Imaging of Joints and Bones in Autoinflammation [PDF]

Journal of Clinical Medicine, 2020
Autoinflammatory disorders are commonly characterized by seemingly unprovoked systemic inflammation mainly driven by cells and cytokines of the innate immune system. In many disorders on this spectrum, joint and bone involvement may be observed and imaging of these manifestations can provide essential diagnostic information.
Katharina Ziegeler, Iris Eshed, Torsten Diekhoff, Kay Geert Hermann   +3 more
openaire   +2 more sources

Successful treatment of periodic disease and secondary AA amyloidosis in a colchicine-resistant patient

Alʹmanah Kliničeskoj Mediciny, 2018
The paper demonstrates current opportunities of effective management of periodic disease as an autoinflammatory disorder based on a clinical case of colchicine-resistant Mediterranean fever. We present modern approaches to the autoinflammation hypothesis
V. V. Rameev, M. V. Bogdanova, L. V. Kozlovskaya, N. A. Mukhin   +3 more
doaj   +1 more source

Janus kinase inhibition for autoinflammation in DNASE2 deficiency [PDF]

, 2019
We characterise the immunopathogenesis of autoinflammation, HLH, and early onset intestinal inflammation caused by homozygous mutation in DNASE2, and provide the first description of favourable therapeutic response to JAK1/JAK2 blockade in this ...
Bader-Meunier, B, Booth, C, Sebire, N, Bullock, AN, Nanthapisal, S, Yasuda, T, Capitani, M, Pandey, S, Gilmour, K, Uhlig, HH, Cavounidis, A, Brogan, P, Hong, Y, Murphy, C, Omoyinmi, E, McCreary, D, Takeshita, H, Eleftheriou, D, Rao, A, Shah, N, Klein, N   +20 more
core   +2 more sources

Lung Functioning and Inflammation in a Mouse Model of Systemic Juvenile Idiopathic Arthritis

Frontiers in Immunology, 2021
Systemic juvenile idiopathic arthritis (sJIA) is an immune disorder characterized by fever, skin rash, arthritis and splenomegaly. Recently, increasing number of sJIA patients were reported having lung disease.
Bert Malengier-Devlies, Tatjana Decaesteker, Kaat Dekoster, Arno Vanstapel, Kourosh Ahmadzadeh, Fariba Poosti, Tania Mitera, Laura Seldeslachts, Erik Verbeken, Carine Wouters, Carine Wouters, Carine Wouters, Greetje Vande Velde, Jeroen Vanoirbeek, Patrick Matthys   +14 more
doaj   +1 more source

Consensus protocols for the diagnosis and management of the hereditary autoinflammatory syndromes CAPS, TRAPS and MKD/HIDS: a German PRO-KIND initiative

Pediatric Rheumatology Online Journal, 2020
Background Rare autoinflammatory diseases (AIDs) including Cryopyrin-Associated Periodic Syndrome (CAPS), Tumor Necrosis Receptor-Associated Periodic Syndrome (TRAPS) and Mevalonate Kinase Deficiency Syndrome (MKD)/ Hyper-IgD Syndrome (HIDS) are ...
Sandra Hansmann, Elke Lainka, Gerd Horneff, Dirk Holzinger, Nikolaus Rieber, Annette F. Jansson, Angela Rösen-Wolff, Gabi Erbis, Martina Prelog, Juergen Brunner, Susanne M. Benseler, Jasmin B. Kuemmerle-Deschner   +11 more
doaj   +1 more source

Gene hunting in autoinflammation

Clinical and Translational Allergy, 2013
AbstractSteady progress in our understanding of the genetic basis of autoinflammatory diseases has been made over the past 16 years. Since the discovery of the familial Me diterranean f ev er gene MEFV (also known as marenostrin) in 1997, 18 other genes responsible for monogenic autoinflammatory diseases have been identified to date.
Standing, Ariane, Omoyinmi, Ebun, Brogan, Paul   +2 more
openaire   +2 more sources