Results 101 to 110 of about 29,559 (188)

Hyper-IgD and Periodic Fever Syndrome: a New MVK Mutation (p.R277G) Associated with a Severe Phenotype [PDF]

open access: yes, 2014
Hyperimmunoglobulinemia D and periodic fever syndrome (HIDS; MIM#260920) is a rare recessively-inherited autoinflammatory condition caused bymutations in the MVK gene, which encodes for mevalonate kinase, an essential enzyme in the isoprenoid pathway ...
Aróstegui, J   +4 more
core   +1 more source

NEMO‐NDAS: Case Report and Review of the Literature

open access: yesPediatric 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   +5 more
wiley   +1 more source

The Use of IL-1 Receptor Antagonist (Anakinra) in Idiopathic Recurrent Pericarditis: A Narrative Review

open access: yesCardiology Research and Practice, 2016
Recurrent pericarditis is a complication of acute pericarditis in 20–30% of the patients and is usually idiopathic in nature. The underlying pathogenesis of this condition remains unclear, although immune-mediated mechanisms seem likely.
Shankar Baskar   +2 more
doaj   +1 more source

Intravenous Anakinra for Macrophage Activation Syndrome May Hold Lessons for Treatment of Cytokine Storm in the Setting of Coronavirus Disease 2019

open access: yesACR Open Rheumatology, 2020
Macrophage activation syndrome (MAS) and hemophagocytic lymphohistiocytosis (HLH) are increasingly recognized as being on a continuum of cytokine storm syndromes, with different initiating pathways culminating in cytotoxic dysfunction and uncontrolled ...
Theresa L. Wampler Muskardin
doaj   +1 more source

Defining the optimal biological monotherapy in rheumatoid arthritis: a systematic review and meta-analysis of randomised trials [PDF]

open access: yes, 2016
Objectives To summarize and compare the benefits and harms of biological agents used as monotherapy for rheumatoid arthritis (RA) in order to inform decisions for patients who are intolerant to conventional DMARD therapy.
Bliddal, Henning   +12 more
core   +2 more sources

Biologic treatments in rheumatoid arthritis and juvenile idiopathic arthritis [PDF]

open access: yes, 2006
A number of biological approaches to the management of inflammtory arthropathies have been explored. These include the development of IL-1 receptor antagonists and TNF antagonists. Four biological agents are currently marketed in Europe.
Borg, Andrew A.
core  

The Role of Extracorporeal Membrane Oxygenation in Cardiopulmonary Dysfunction Due to Rheumatologic Diseases: A Systematic Review

open access: yesACR Open Rheumatology, Volume 8, Issue 4, April 2026.
Objective This systematic review documents the current status of extracorporeal membrane oxygenation (ECMO) use in patients with complications of rheumatologic diseases. Methods Following Preferred Reporting Items for Systematic Reviews and Meta Analyses 2020 guidelines, we conducted a systematic review of studies published from January 2019 to ...
Kari L. Falaas   +3 more
wiley   +1 more source

Development of an R script for simple lipidomic and metabolomic data analysis [PDF]

open access: yes, 2020
Background: Metabolomic and lipidomic studies generate vast quantities of data that are often analysed in a closed software environment with little to no access to the underlying algorithms.
Alshammari, Suad   +4 more
core   +1 more source

Effectiveness of Canakinumab for First‐Line Steroid‐Free Treatment in Systemic‐Onset Juvenile Idiopathic Arthritis and Juvenile Still Disease

open access: yesACR Open Rheumatology, Volume 8, Issue 4, April 2026.
Objective This prospective study assesses the response to canakinumab monotherapy administered as three monthly subcutaneous injections in glucocorticoid‐naïve patients with newly diagnosed systemic juvenile idiopathic arthritis (sJIA) and Still disease and evaluates the durability of drug‐free inactive disease for up to nine months after canakinumab ...
Gerd Horneff   +9 more
wiley   +1 more source

Interleukin 1 inhibition with anakinra in adult-onset Still disease: a meta-analysis of its efficacy and safety

open access: yesDrug Design, Development and Therapy, 2014
Dongsheng Hong,1 Zhihai Yang,1 Shuyin Han,1 Xingguang Liang,1 Kuifen Ma,1 Xingguo Zhang1,2 1Department of Pharmacy, the First Affiliated Hospital of College of Medicine, Zhejiang University, 2College of Pharmaceutical Science, Zhejiang Chinese Medical ...
Hong D   +5 more
doaj  

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