Results 31 to 40 of about 10,179 (209)

Inflammasomes and dermatology [PDF]

Anais Brasileiros de Dermatologia, 2016
: Inflammasomes are intracellular multiprotein complexes that comprise part of the innate immune response. Since their definition, inflammasome disorders have been linked to an increasing number of diseases.
Daniel Coelho de Sá, Cyro Festa Neto
doaj   +1 more source

Muckle–Wells Syndrome in a Child With Recurrent Urticaria

Вопросы современной педиатрии, 2017
Cryopyrin associated periodic syndromes (CAPS) are rare monogenic autoinflammatory diseases from the group of hereditary periodic syndromes caused by a regulation defect of inflammatory cytokines, in particular interleukin 1β.
Elena M. Kamaltynova, Iulia P. Chasovskikh, Zinaida A. Maevskaia, Svetlana O. Salugina, Evgenii S. Fedorov, Inna E. Gerbek   +5 more
doaj   +1 more source

Insights into CANDLE Syndrome

Revista da Sociedade Portuguesa de Dermatologia e Venereologia, 2020
Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome is a recently described chronic autoinflammatory disease, pathophysiologically related to intracellular proteasome/immunoproteasome dysfunction.
Katarina Kieselová, Felicidade Santiago, Martinha Henrique   +2 more
doaj   +1 more source

Diagnosis and treatment of autoinflammatory diseases in adults: a clinical approach from rheumatologists

, 2019
utoinflammatory diseases are characterized by recurrent systemic inflammation due to dysfunction of the innate immune system, and they are originally the hereditary recurrent fever syndromes that develop in early childhood. Many cases are thus diagnosed
Koga, Tomohiro, Kawakami, Atsushi
core   +1 more source

Consensus proposal for taxonomy and definition of the autoinflammatory diseases (AIDs): a Delphi study [PDF]

, 2018
Autoinflammatory diseases (AIDs) are a relatively new family of disorders, defined about 19 years ago. Some of them are hereditary and some are not.
KASTNER, Daniel L., Ruperto, N, Paediatric Rheumatology International Trials Organisation (PRINT, Lachmann, Helen J., Gul, A, Kästner, Daniel, Ruperto, Nicolino, Lachmann, HJ, Touitou, I, Gattorno, M, Ben-Chetrit, Eldad, Gul, Ahmet, Ben-Chetrit, E, Lachmann, Helen, J., Gattorno, Marco, Lachmann, Helen,, Touitou, Isabelle, Kastner, DL   +17 more
core   +1 more source

A Brief History of IL-1 and IL-1 Ra in Rheumatology

Frontiers in Pharmacology, 2017
The history of what, in 1979, was called interleukin-1 (IL-1), orchestrator of leukocyte inter-communication, began many years before then, initially by the observation of fever induction via the endogenous pyrogen (EP) (1974) and then in rheumatology on
Jean-Michel Dayer, Francesca Oliviero, Leonardo Punzi   +2 more
doaj   +1 more source

Real-Life Indications of Interleukin-1 Blocking Agents in Hereditary Recurrent Fevers: Data From the JIRcohort and a Literature Review

Frontiers in Immunology, 2021
BackgroundInterleukin (IL)-1 inhibitors represent the main treatment in patients with colchicine-resistant/intolerant familial Mediterranean fever (crFMF), mevalonate kinase deficiency (MKD), and tumor necrosis factor receptor-associated periodic ...
Caroline Vinit, Caroline Vinit, Sophie Georgin-Lavialle, Sophie Georgin-Lavialle, Aikaterini Theodoropoulou, Aikaterini Theodoropoulou, Catherine Barbier, Alexandre Belot, Alexandre Belot, Manel Mejbri, Manel Mejbri, Pascal Pillet, Jana Pachlopnik, Sylvaine Poignant, Charlotte Rebelle, Andreas Woerner, Isabelle Koné-Paut, Isabelle Koné-Paut, Véronique Hentgen, Véronique Hentgen   +19 more
doaj   +1 more source

The expanding spectrum of rare monogenic autoinflammatory diseases. [PDF]

, 2013
International audience: Monogenic autoinflammatory diseases are a group of hereditary disorders characterized by a clinical and biological inflammatory syndrome in which there is little or no evidence of autoimmunity. The discovery of the first causative
Galeotti, Caroline, Véronique Hentgen, Piram, Maryam, Isabelle Koné-Paut, Rossi-Semerano, Linda, Hentgen, Véronique, Koné-Paut, Isabelle, Isabelle Touitou, null null, Caroline Galeotti, Touitou, Isabelle, Maryam Piram, Linda Rossi-Semerano   +12 more
core   +1 more source

Pustular Eruptions in Children as Manifestations of Autoinflammatory Diseases

Revista da Sociedade Portuguesa de Dermatologia e Venereologia, 2019
Nowadays, in clinical practice, when attending a child with a pustular eruption and systemic inflammation, it is mandatory to think of an autoinflammatory disease, once infectious causes have been ruled out.
Felicidade Santiago, Antonio Torrelo
doaj   +1 more source

Case report: JAK inhibition as promising treatment option of fatal RVCLS due to TREX1 mutation (pVAL235Glyfs*6)

Frontiers in Neurology, 2023
IntroductionAutosomal dominant mutations in the C-terminal part of TREX1 (pVAL235Glyfs*6) result in fatal retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCLS) without any treatment options.
Friederike Ufer, Susanne M. Ziegler, Marcus Altfeld, Manuel A. Friese   +3 more
doaj   +1 more source