Results 91 to 100 of about 2,214,075 (211)

Monogenic familial autoinflammatory Behçet-like syndrome/ haploinsufficiency A20 syndrome is a new form of autoinflammatory pathology. Literature review and description of cases

Научно-практическая ревматология
Monogenic familial autoinflammatory Behçet-like syndrome/haploinsufficiency A20 syndrome is a hereditary autoinflammatory disease from the group of ubiquitinopathies which are caused by a mutation of the TNFAIP3 gene encoding the A20 protein with an ...
Е. S. Fedorov, S. О. Salugina, Е. Yu. Zakharova, А. N. Shapovalenko, S. G. Radenska-Lopovok, V. G. Matkava, А. N. Arefieva   +6 more
doaj   +1 more source

A Real‐Life, Multicenter, Retrospective Study on Epidemiological and Clinical Features of Patients With Hidradenitis Suppurativa and Concomitant Down Syndrome

International Journal of Dermatology, EarlyView.
Summary of the clinical characteristics and 2‐year follow‐up of patients with HS and concomitant DS in a multicentre Italian cohort, highlighting earlier HS onset, clinical features, and improved disease severity, quality of life, and pain over follow‐up, supporting early screening and multidisciplinary care of this special population.
Chiara Moltrasio, Luisa Sarno, Giovanni Genovese, Federica Derlino, Dalma Malvaso, Valeria Gaspari, Marina Venturini, Federica Repetto, Gianluca Avallone, Simone Ribero, Elisa Molinelli, Oriana Simonetti, Alessandra Michelucci, Marco Romanelli, Valentina Dini, Marco Manfredini, Cristina Magnoni, Caterina Foti, Silvia Sanna, Laura Atzori, Federico Bardazzi, Riccardo Balestri, Giulia Rech, Paolo Gisondi, Raffaele Dante Caposiena Caro, Iris Zalaudek, Viviana Lora, Luca Fania, Elia Rosi, Stefano Buffon, Carlo Alberto Maronese, Alessandra Cartocci, Pietro Rubegni, Francesca Prignano, Angelo Valerio Marzano   +34 more
wiley   +1 more source

Emerging Roles of PSTPIP2 in Autoimmunity: From Mechanism to Therapeutic Implications

Immunology, EarlyView.
PSTPIP2 acts as a key brake on myeloid inflammation by suppressing IL‐1β‐driven signalling, macrophage dysregulation and inflammation‐associated bone remodelling. Its emerging roles in CRMO/CMO, rheumatoid arthritis, SAPHO syndrome and bullous pemphigoid support its potential as a biomarker and therapeutic target.
Erdong Zhang, Fengyu Liu, Yongle Wei, Guoqiang Li, Liujie Zheng, Lin Jin, Feng Luan, Yingze Zhang, Ling Wang, Zhiyong Hou   +9 more
wiley   +1 more source

How to identify a patient with autoinflammatory syndrome: Clinical and diagnostic algorithms

Современная ревматология, 2013
Autoinflammatory syndromes (AISs) are a group of predominantly hereditary diseases associated with the spontaneous uncontrolled production of proinflammatory cytokines. Most diseases are known to have molecular mechanisms and an inheritance pattern.
Mikhail Mikhailovich Kostik, L S Snegireva, M F Dubko, V V Masalova, T S Likhacheva, O V Kalashnikova, V G Chasnyk   +6 more
doaj   +1 more source

Genomic and proteomic insights into hidradenitis suppurativa

Journal of the European Academy of Dermatology and Venereology, EarlyView.
A dual pathogenic model of HS involving both epithelial dysfunction and systemic inflammation is supported. The HLA‐DRA association hints at autoimmune overlap, but the proteomic signature which is dominated by innate immune mediators favours an autoinflammatory classification, which may guide future therapeutic strategies and patient stratification ...
Maria Argyropoulou, Emmanouil Stylianakis, Isis Ricaño‐Ponce, Nick Keur, Theodora Kanni, Dimitra Stergianou, Mihai G. Netea, Vinod Kumar, Evangelos J. Giamarellos‐Bourboulis   +8 more
wiley   +1 more source

Autoinflammatory diseases in children.

, 2010
Autoinflammatory diseases encompass a growing number of multisystem clinical entities with genetic or acquired defects in the innate immune system. Distinct conditions can be identified in this expanding sphere: familiar Mediterranean fever, mevalonate ...
Rigante, Donato
core  

Management of chronic urticaria: Current status and future prospect

Journal of the European Academy of Dermatology and Venereology, EarlyView.
Chronic urticaria is driven by mast cell activation through autoimmune, inflammatory, and neuroimmune pathways. A structured approach combining diagnosis, patient‐reported outcomes, stepwise therapy, treatment optimization, monitoring and emerging targeted agents may improve disease control and enable more personalized management.
Andaç Salman, Mona Al‐Ahmad, Maryam Al‐Nesf, Chia‐Yu Chu, Luis Felipe Ensina, Ana M. Gimenez‐Arnau, Margarida Gonçalo, Clive Grattan, Michihiro Hide, Pavel Kolkhir, Martin Metz, Iman Nasr, Romi Saini, Simon Francis Thomsen, Torsten Zuberbier, Emek Kocaturk   +15 more
wiley   +1 more source

Autoinflammatory Disorders

, 2012
Originally, autoinflammatory diseases were defined as conditions characterized by seemingly unprovoked episodes of inflammation, without high titres of autoantibodies or antigen-specific T-cells [1].
Berg, Stefan,, Stefan Berg, Wekell, Per,, Per Wekell, Anders Fasth, Fasth, Anders,   +5 more
core   +1 more source

First report from the Czech national registry of inborn errors of immunity (2012–2025)

Frontiers in Immunology
IntroductionCongenital immune system defects represent an ever-growing group of diseases characterized by increased susceptibility to infections and association with autoimmune, autoinflammatory, allergic and malignant complications. Here, we provide the
Zita Chovancova, Zita Chovancova, Eva Hlavackova, Eva Hlavackova, Roman Hakl, Roman Hakl, Tomas Milota, Pavlina Kralickova, Pavlina Kralickova, Ivana Malkusova, Beata Hutyrova, Michaela Safarova, Jana Vydlakova, Dalibor Jilek, Jiri Novak, Helena Schneiderova, Helena Schneiderova, Petra Kralova, Alena Zimulova, Vitezslav Novak, Jaromir Bystron, Dita Zaveska, Vendula Latalova, Frantisek Kopriva, Milan Kasl, Vladimir Kracik, Renata Formankova, Petr Sedlacek, Karolina Vytiskova, Michal Svoboda, Hana Novakova, Jiri Litzman, Jiri Litzman   +32 more
doaj   +1 more source

European S2k guidelines for hidradenitis suppurativa/acne inversa Part 1. Epidemiology, diagnosis and clinical assessment

Journal of the European Academy of Dermatology and Venereology, EarlyView.
Updated S2k hidradenitis suppurativa (HS) guidelines provide expert consensus on diagnosis, assessment and comorbidities. Expanded substantially since the 2015 version, they reflect rapid growth in HS literature and aim to improve generalist‐level patient care through evidence‐informed, consensus‐based guidance.
G. B. E. Jemec, B. Villumsen, K. R. van Straalen, H. H. van der Zee, S. Valiukevičienė, T. Tzellos, J. C. Szepietowski, A. Szegedi, D. M. L. Saunte, M. Romanelli, H. Raynal, F. Prignano, E. P. Prens, M. Podda, A. Nassif, A. Molina‐Leyva, Ł. Matusiak, A. V. Marzano, B. M. McGrath, A. I. Liakou, B. Kirby, L. Kemény, E. Just, D. Ioannidis, J. R. Ingram, R. E. Hunger, B. Horváth, P. Guillem, Ø. Grimstad, E. J. Giamarellos‐Bourboulis, M. Dolenc‐Voljč, V. Del Marmol, Z. Bukvić Mokos, V. Bettoli, F. Benhadou, F. G. Bechara, C. C. Zouboulis   +36 more
wiley   +1 more source