Results 21 to 30 of about 94,301 (231)

004 Systemic autoinflammatory diseases: a multicenter cohort study

Rheumatology, 2022
Background Systemic autoinflammatory diseases (SAIDs) are a group of hereditary diseases characterized by inflammatory attacks accompanied by clinical findings such as fever, skin rash, abdominal pain, and musculoskeletal symptoms and with high acute ...
A. Tanatar, Özlem Akgün, Ş. Çağlayan, S. Özdel, G. O. Yener, M. Çakan, Kübra Öztürk, H. E. Sönmez, B. Sözeri, N. Ayaz   +9 more
semanticscholar   +1 more source

POS1365 THE FMF&AID SURVEY - A PATIENT ORGANIZATION DRIVEN SURVEY FOR AUTOINFLAMMATORY DISEASES

Annals of the Rheumatic Diseases, 2022
Autoinflammatory diseases (also referred to as hereditary periodic fever syndromes) are caused by defects in the innate immune system. Many autoinflammatory syndromes arise from inherited genetic mutations which begin in childhood and persist throughout ...
J. Rech, K. Taşçilar, A. Tufan, M. Gattorno, J. Kuemmerle-Deschner, S. Ozen, T. Krickau, E. Cohen, L. Oliveira Mendonca, A. Kontzias, M. Vetterli   +10 more
semanticscholar   +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

Long-Term Follow-Up and Optimization of Interleukin-1 Inhibitors in the Management of Monogenic Autoinflammatory Diseases: Real-Life Data from the JIR Cohort

Frontiers in Pharmacology, 2021
Objectives: The major role of interleukin (IL)-1 in the pathogenesis of hereditary recurrent fever syndromes favored the employment of targeted therapies modulating IL-1 signaling. However the best use of IL1 inhibitors in terms of dosage is difficult to
V. Hentgen, I. Koné-Paut, A. Belot, C. Galeotti, G. Grateau, A. Carbasse, A. Pagnier, P. Pillet, M. Delord, M. Hofer, S. Georgin-lavialle   +10 more
semanticscholar   +1 more source

Joint together: The etiology and pathogenesis of ankylosing spondylitis

Frontiers in Immunology, 2022
Spondyloarthritis (SpA) refers to a group of diseases with inflammation in joints and spines. In this family, ankylosing spondylitis (AS) is a rare but classic form that mainly involves the spine and sacroiliac joint, leading to the loss of flexibility ...
Yuehan Xiong, Menghua Cai, Yi Xu, Peng Dong, Hui Chen, Hui Chen, Wei He, Wei He, Jianmin Zhang, Jianmin Zhang   +9 more
doaj   +1 more source

Autoinflammatory diseases: a possible cause of thrombosis? [PDF]

, 2015
Autoinflammatory diseases are a group of disorders due to acquired or hereditary disfunction of innate immune system and characterized by systemic or localized manifestations. The prototype is Familial Mediterranean Fever, a monogenic hereditary disorder,
Francesco Orlandini, Micaela La Regina, Raffaele Manna   +2 more
core   +2 more sources

Clinical and functional characterisation of a novel TNFRSF1A c.605T > A/V173D cleavage site mutation associated with tumour necrosis factor receptor-associated periodic fever syndrome (TRAPS), cardiovascular complications and excellent response to etanercept treatment. [PDF]

, 2008
Objectives: To study the clinical outcome, treatment response, T-cell subsets and functional consequences of a novel tumour necrosis factor (TNF) receptor type 1 (TNFRSF1A) mutation affecting the receptor cleavage site.
B H Belohradsky, C Dejaco, C Duftner, K Huss, M Herold, M Schirmer, P Lohse, S Stojanov   +7 more
core   +1 more source

Development of the doctrine of auto-inflammatory diseases in the XXI century

Научно-практическая ревматология, 2019
The article presents the development of the doctrine of autoinflammatory diseases (AID) in the last decade. Data on interleukin 1, inflammasomes and their role in the development of AID are presented.
E. S. Fedorov, S. O. Salugina, N. N. Kuzmina   +2 more
doaj   +1 more source

An Up-to-date Approach to a Patient with a Suspected Autoinflammatory Disease

Rambam Maimonides Medical Journal, 2017
Autoinflammatory diseases (AID) are characterized by seemingly unprovoked self-limited attacks of fever and systemic inflammation potentially leading to amyloidosis. Familial Mediterranean fever (FMF) is the most common AID and therefore the most studied.
Merav Lidar, Eitan Giat
doaj   +1 more source

Development of the autoinflammatory disease damage index (ADDI) [PDF]

, 2016
OBJECTIVES: Autoinflammatory diseases cause systemic inflammation that can result in damage to multiple organs. A validated instrument is essential to quantify damage in individual patients and to compare disease outcomes in clinical studies.
Al-Mayouf, SM, Amaryan, G, Annink, KV, Anton, J, Barron, KS, Benseler, SM, Brogan, PA, Cantarini, L, Cattalini, M, Cochino, AV, De Benedetti, F, De Jesus, AA, Dedeoglu, F, Della Casa Alberighi, O, Demirkaya, E, Dolezalova, P, Durrant, KL, Fabio, G, Frenkel, J, Gallizzi, R, Gattorno, M, Goldbach-Mansky, R, Hachulla, E, Hentgen, V, Herlin, T, Hofer, M, Hoffman, HM, Insalaco, A, Jansson, AF, Kallinich, T, Koné-Paut, I, Kozlova, A, Kuemmerle-Deschner, JB, Lachmann, HJ, Laxer, RM, Martini, A, Nielsen, S, Nikishina, I, Ombrello, AK, Ozen, S, Papadopoulou-Alataki, E, Quartier, P, Ravelli, A, Rigante, D, Russo, R, Simon, A, Ter Haar, NM, Trachana, M, Uziel, Y   +48 more
core   +2 more sources