Results 21 to 30 of about 27,529 (230)

GSDMD is critical for autoinflammatory pathology in a mouse model of Familial Mediterranean Fever [PDF]

, 2018
Pyroptosis is an inflammasome-induced lytic cell death mode, the physiological role of which in chronic inflammatory diseases is unknown. Familial Mediterranean Fever (FMF) is the most common monogenic autoinflammatory disease worldwide, affecting an ...
Chi, Hongbo, Kambara, Hiroto, Kanneganti, Apurva, Lamkanfi, Mohamed, Luo, Hongbo R, Malireddi, RK Subbarao, Tillman, Heather, Van Gorp, Hanne, Vande Walle, Lieselotte, Viana Saavedra, Pedro Henrique, Vogel, Peter, Xavier, Ramnik J   +11 more
core   +1 more source

Hematopoietic Cell Transplantation in Patients With Primary Immune Regulatory Disorders (PIRD): A Primary Immune Deficiency Treatment Consortium (PIDTC) Survey. [PDF]

, 2020
Primary Immune Regulatory Disorders (PIRD) are an expanding group of diseases caused by gene defects in several different immune pathways, such as regulatory T cell function.
Allenspach, Eric J, Andolina, Jeffrey R, Aquino, Victor M, Bacchetta, Rosa, Bednarski, Jeffrey J, Bhatia, Monica, Buckley, Rebecca H, Burroughs, Lauri M, Caywood, Emi, Chan, Alice Y, Chandrakasan, Shanmuganathan, Chellapandian, Deepak, Chen, Karin, Chong, Hey, Cooper, Megan A, Cowan, Morton J, Craddock, John A, Cuvelier, Geoffrey DE, Davila Saldana, Blachy J, de la Morena, Maria Teresa, Dorsey, Morna J, Dvorak, Christopher C, Forbes, Lisa R, Gennery, Andrew R, Goldman, Frederick D, Griffith, Linda M, Haddad, Elie, Heimall, Jennifer, Jordan, Michael B, Kapoor, Neena, Kohn, Donald B, Leiding, Jennifer W, Liu, Xuerong, Logan, Brent R, Marsh, Rebecca A, Martinez, Caridad, Meyts, Isabelle, Miller, Holly K, Notarangelo, Luigi D, Oshrine, Benjamin R, Pai, Sung-Yun, Parikh, Suhag, Prockop, Susan E, Puck, Jennifer M, Pulsipher, Michael A, Seidel, Markus G, Seroogy, Christine M, Shenoy, Shalini, Shereck, Evan, Shyr, David C, Skoda-Smith, Suzanne, Slatter, Mary, Smith, Angela R, Sullivan, Kathleen E, Torgerson, Troy R, Uzel, Gulbu, Vander Lugt, Mark T, Walter, Jolan E, Weinacht, Katja G, Wright, Nicola   +59 more
core   +1 more source

NAIP/NLRC4 inflammasome activation in MRP8+ cells is sufficient to cause systemic inflammatory disease. [PDF]

, 2017
Inflammasomes are cytosolic multiprotein complexes that initiate protective immunity in response to infection, and can also drive auto-inflammatory diseases, but the cell types and signalling pathways that cause these diseases remain poorly understood ...
Nichols, Randilea D, Vance, Russell E, von Moltke, Jakob   +2 more
core   +2 more sources

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

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

State of care for patients with systemic autoinflammatory diseases – Results of a tertiary care survey

World Allergy Organization Journal, 2019
Background: Systemic autoinflammatory diseases (SAIDs) are rare debilitating disorders of which there is limited awareness and a significant delay in diagnosis.
Mati Chuamanochan, Karsten Weller, Eugen Feist, Tilmann Kallinich, Marcus Maurer, Jasmin Kümmerle-Deschner, Karoline Krause   +6 more
doaj   +1 more source

Erythematous nodes, urticarial rash and arthralgias in a large pedigree with NLRC4-related autoinflammatory disease, expansion of the phenotype [PDF]

, 2016
Autoinflammatory disorders (AID) are a heterogeneous group of diseases, characterized by an unprovoked innate immune response, resulting in recurrent or ongoing systemic inflammation and fever1-3.
Blokx, W. A. M., Boes, M., de Koning, H. D., de Kovel, C., Frenkel, J., Giltay, J., Oberndorff, K., Schalkwijk, J., Simon, A., van Gijn, M. E., van Kempen, T. S., van Steensel, M. A. M., van Well, G. T. J., Volker-Touw, C. M. L.   +13 more
core   +2 more sources

Autoinflammation: translating mechanism to therapy [PDF]

Journal of Leukocyte Biology, 2011
ABSTRACTAutoinflammatory syndromes are a clinically heterogeneous collection of diseases characterized by dysregulation of the innate immune system. The hereditary recurrent fever disorders were the first to be defined as autoinflammatory. Several of the responsible genes are now known to encode proteins forming multimeric complexes called ...
Taylor A, Doherty, Susannah D, Brydges, Hal M, Hoffman   +2 more
openaire   +2 more sources

Editorial: tertiary lymphoid organs (TLOs): powerhouses of disease immunity [PDF]

, 2017
No abstract ...
Habenicht, Andreas J.R., Maffia, Pasquale, Mohanta, Sarajo, Yin, Changjun   +3 more
core   +2 more sources

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