Results 81 to 90 of about 147,251 (203)

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

Paediatric‐onset autoimmune cytopenia: How can we reduce the long‐term mortality?

British Journal of Haematology, EarlyView.
Nathalie Aladjidi, Thierry Leblanc, Helder Fernandes, Capucine Picard, Jacinta Bustamante, Frédéric Rieux‐Laucat, Mathieu Fusaro, Maud Tusseau, Charlotte Durand‐Teyssier, Emma Cohendy, Jérôme Granel, Stéphane Ducassou, Agathe Escudier, Despina Moshous, Marc Michel, Bertrand Godeau, Sébastien Héritier, Mony Fahd, Brigitte Bader‐Meunier, Alain Fischer, Guy Leverger, All collaborators from the French Paediatric Haemato‐Immunologic CEREVANCE Network   +21 more
wiley   +1 more source

Periodontitis and Inflammatory Bowel Diseases: Mechanistic Evidence

Journal of Periodontal Research, EarlyView.
This review aims to provide a conceptual framework for understanding the mechanistic interplay between periodontitis and IBD, with a particular emphasis on the microbial and immunological crosstalk linking the oral cavity and the gastrointestinal tract.
Ana Paula V. Colombo, Adriana M. de Oliveira, Nobuhiko Kamada, Sho Kitamoto   +3 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

NEMO‐NDAS: Case Report and Review of the Literature

Pediatric Dermatology, EarlyView.
ABSTRACT NEMO‐deleted exon 5 autoinflammatory syndrome (NEMO‐NDAS) is the result of a gain‐of‐function IKBKG pathogenic variant leading to dysregulated NF‐κB signaling and systemic inflammation. We present a case of NEMO‐NDAS in a 2‐year‐old female presenting with recurrent fevers, subcutaneous nodules, lymphadenopathy, and splenomegaly.
Angela Yang, Kristie Mar, Kimberly A. Morishita, Kevin Meesters, Antonio Torrelo, Joseph M. Lam   +5 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

Sustained Remission of Pediatric Bowel‐Associated Dermatosis‐Arthritis Syndrome With Risankizumab

Pediatric Dermatology, EarlyView.
ABSTRACT Bowel‐associated dermatosis‐arthritis syndrome (BADAS) is a rare neutrophilic dermatosis characterized by recurrent fever, arthralgias, and skin eruptions, most commonly occurring in patients with inflammatory bowel disease. We report the case of a 17‐year‐old female with Crohn's disease who developed BADAS and achieved complete and durable ...
Kylie E. Peake, Harman Toor, Marie‐Christine Jean, Collin C. Barker, Joseph M. Lam   +4 more
wiley   +1 more source

Adjunctive Therapeutic Plasma Exchange in Refractory Adult‐Onset Still's Disease Complicated by Secondary Macrophage Activation Syndrome: A Single‐Center Experience

Therapeutic Apheresis and Dialysis, Volume 30, Issue 3, Page 389-396, June 2026.
ABSTRACT Introduction Adult‐onset Still's disease (AOSD) complicated by macrophage activation syndrome (MAS) carries substantial mortality. The role of therapeutic plasma exchange (TPE) remains uncertain. Methods We retrospectively analyzed patients with AOSD‐MAS treated with TPE at a single‐center.
Masataka Ueda, Tomohiro Saito, Masahide Mizobuchi, Tomoki Shimomura, Saki Kawamori, Mitsuru Kawanishi, Natsuko Hattori, Jo Sugiyama, Satoshi Tsubata, Hanami Horiguchi, Kazuki Abe, Nao Iida, Yuki Kajio, Nao Oguro, Ryo Yanai, Hirokazu Honda   +15 more
wiley   +1 more source