Results 111 to 120 of about 178,684 (248)

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

Schnitzler Syndrome as an Autoinflammatory Disease Driven by B‐Cell‐Specific Somatic MYD88 Mutation

Allergy, EarlyView.
Yuyi Zhou, Yoko Ueki, Naoya Iwata, Hiroshi Oue, Kentaro Kato, Mengyan Li, Kazushi Izawa, Tomoyasu Jo, Seishi Ogawa, Kenji Kabashima, Naotomo Kambe   +10 more
wiley   +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

Tocilizumab for treatment of cutaneous and systemic manifestations of vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome without myelodysplastic syndrome

JAAD Case Reports, 2022
Amrita Goyal, MD, Damodaran Narayanan, MBBS, PhD, Waihay Wong, MD, PhD, Alvaro C. Laga, MD, Nathan T. Connell, MD, MPH, Susan Y. Ritter, MD, PhD, Gabriela Cobos, MD   +6 more
doaj   +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

Interleukin-1 Blockers in Recurrent and Acute Pericarditis: State of the Art and Future Directions

Medicina
Diseases of the pericardium encompass a spectrum of conditions, including acute and recurrent pericarditis, where inflammation plays a pivotal role in the pathogenesis and clinical manifestations. Anti-inflammatory therapy indeed forms the cornerstone of
Antonella Gallo, Maria Grazia Massaro, Sara Camilli, Silvino Di Francesco, Laura Gerardino, Elena Verrecchia, Ludovico Luca Sicignano, Francesco Landi, Raffaele Manna, Massimo Montalto   +9 more
doaj   +1 more source

Age‐Dependent Differences in Canakinumab Safety: A Comprehensive Pharmacovigilance Analysis Using the FAERS Database

Pharmacology Research &Perspectives, Volume 14, Issue 3, June 2026.
ABSTRACT While the efficacy of canakinumab, an anti‐interleukin‐1β monoclonal antibody, is well‐established, its safety profile, particularly across different age groups, remains inadequately explored. Using the FDA Adverse Event Reporting System (FAERS) database, this study evaluated postmarketing safety by analyzing adverse event (AE) reports from ...
Youyang Wang, Ngasin Lam, Xiaoming Huang, Yang Jiao   +3 more
wiley   +1 more source

TREM‐1 Blockade Inhibits Inflammasome Activation and Pyroptosis: Novel Insights on the Role of TREM‐1 and Syk in Monosodium Urate Crystal‐Induced Inflammation

Immunology, Volume 178, Issue 2, Page 249-260, June 2026.
Schematic overview of the roles triggering receptor expressed on myeloid cells‐1 (TREM‐1) plays in monosodium urate (MSU) crystal‐induced inflammasome activation and IL‐1β secretion in gout arthritis. The present study provides novel data suggesting that blockade of TREM‐1 by LP17 attenuates MSU‐crystal‐induced inflammation through inhibition of ...
Yair Molad, Irina Lagovsky, Vitaly Kliminski   +2 more
wiley   +1 more source

Rediscovering parainfectious encephalopathy in the post-COVID-19 era

Frontiers in Immunology
The COVID-19 pandemic has unveiled the pivotal role of systemic inflammatory responses in neurological complications, particularly parainfectious encephalopathy.
Lin Bai, Chang Geng, Hongzhi Guan
doaj   +1 more source