Results 111 to 120 of about 157,940 (239)

Long‐term echocardiographic findings in patients with idiopathic recurrent pericarditis treated with anakinra

Internal Medicine Journal, EarlyView.
Abstract Aim Idiopathic recurrent pericarditis (IRP) is considered an autoinflammatory disease, and interleukin 1 inhibitors, such as anakinra, are used to treat resistant cases. Constrictive pericarditis, a feared complication, continues to be a critical concern. In the biologic era, evidence on long‐term transthoracic echocardiographic (TTE) findings
Zeynep Toker Dincer, Damla Raimoglou, Erkin Yilmaz, Ayten Dag, Melike Melikoglu, Serdal Ugurlu   +5 more
wiley   +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

OX40/OX40L modulation: A target for regulating T cells in cutaneous inflammatory disorders

Journal of the European Academy of Dermatology and Venereology, EarlyView.
OX40 and OX40L are a co‐stimulatory immune checkpoint pair. Modulation of this pair impacts multiple immune phenotypes and is an attractive target for immunotherapy in dermatological disorders. Trials are underway with the majority in atopic dermatitis and currently in phase 3 trials.
Aditya K. Gupta, Julia Steriopoulos, Vasiliki Economopoulos, Paradi Mirmirani, Vincent Piguet   +4 more
wiley   +1 more source

Is Still's Disease an Autoinflammatory Syndrome?

International Journal of Inflammation, 2012
Systemic juvenile idiopathic arthritis (sJIA), formerly called Still's disease, is officially classified as a subset of juvenile idiopathic arthritis (JIA).
Linda Rossi-Semerano, Isabelle Koné-Paut   +1 more
doaj   +1 more source

Development and Role in Therapy of Canakinumab in Adult-Onset Still's Disease [PDF]

, 2018
Adult-onset Still's disease (AOSD) is a rare inflammatory disease of unknown etiology typically characterized by episodes of spiking fever, evanescent rash, arthralgia, leukocytosis, and hyperferritinemia. The pivotal role of interleukin (IL)-1 and other
Baggio, Chiara, Bindoli, S, Galozzi, P, Oliviero, F, Sfriso, P   +4 more
core   +1 more source

Fatal Chronic Varicella‐Zoster Viral Infection in a Young Man With Chediak–Higashi Syndrome

Pediatric Dermatology, EarlyView.
ABSTRACT Chediak–Higashi syndrome (CHS) is a rare autosomal recessive primary immunodeficiency characterized by partial oculocutaneous albinism, neurologic involvement, and a predisposition to severe infections. Patients are particularly susceptible to developing hemophagocytic lymphohistiocytosis (HLH), which significantly worsens prognosis. We report
Albane Badet, Clément Pruvot, Zoé Manssens, Wadih Abou‐Chahla, Sébastien Buche   +4 more
wiley   +1 more source

A series of cutaneous manifestations in a patient with vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome

SAGE Open Medical Case Reports
Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic is a rare, autoinflammatory disorder caused by a somatic mutation of ubiquitin-activating enzyme 1.
Anne-Sophie Groleau, Alex Derstenfeld, Steffany Poupart, Isabelle Marcil   +3 more
doaj   +1 more source

Hidradenitis suppurativa with systemic autoinflammatory features in patients of Moroccan origin: case report and implications for personalized medicine

Frontiers in Immunology
Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease primarily affecting intertriginous regions, and emerging evidence suggests that systemic autoinflammation (“metainflammation”) contributes to its clinical heterogeneity.
Jérôme Lurel, Fadwa El Aissoug, Fatima-Zahra Hammoud, Dillon Mintoff, Farida Benhadou, Farida Benhadou   +5 more
doaj   +1 more source

Dermatologic Findings of RELA‐Associated Autoinflammatory Disease

Pediatric Dermatology, EarlyView.
ABSTRACT Variants in the gene RELA have been implicated in a monogenic, hereditary form of Behcet's‐like syndrome. This case series describes the dermatologic manifestations of three patients with identified RELA‐associated autoinflammatory disease.
Elizabeth Nourse, Vidya Sivaraman, Catherine G. Chung, Shoghik Akoghlanian, Esteban Fernandez Faith   +4 more
wiley   +1 more source

Comprehensive clinical phenotype, genotype and therapy in Yao syndrome

Frontiers in Immunology
ObjectiveYao syndrome (YAOS) is formerly called nucleotide-binding oligomerization domain containing 2 (NOD2)-associated autoinflammatory disease.We report a large cohort of YAOS.MethodsWe conducted a retrospective analysis of a cohort of adult patients ...
Hafsa Nomani, Song Wu, Ashmia Saif, Frank Hwang, Jane Metzger, Brianne Navetta-Modrov, Peter D. Gorevic, Ivona Aksentijevich, Qingping Yao   +8 more
doaj   +1 more source