Focal and Segmental Glomerulosclerosis (FSGS) is a severe glomerulopathy that frequently leads to end stage renal disease. Only a subset of patients responds to current therapies, making it important to identify alternative therapeutic options.
Michael Boehm +6 more
doaj +1 more source
Objective The objective of this study was to report the benefit of a therapeutic approach consisting of intravenous (IV) continuous anakinra (recombinant human interleukin‐1 receptor antagonist) infusions in treating severely ill adult patients with ...
Luke Adam Monteagudo +2 more
doaj +1 more source
Successful Treatment of Severe Purpura Fulminans With Anakinra
ABSTRACT Purpura fulminans (PF) is a rare, often fatal pediatric condition characterized by intravascular thrombosis and hemorrhagic infarction of the skin. A timely diagnosis and treatment are paramount to prevent the involvement of internal organs, causing disseminated intravascular coagulation and gangrene of the extremities.
Francesco Zulian +4 more
wiley +1 more source
Background. Revascularization of infarcted myocardium results in release of inflammatory cytokines mediating myocardial reperfusion injury and heart failure. Blockage of inflammatory pathways dampens myocardial injury and reduces infarct size.
Christina Grothusen +6 more
doaj +1 more source
International Retrospective Chart Review of Treatment Patterns in Severe Familial Mediterranean Fever, Tumor Necrosis Factor Receptor–Associated Periodic Syndrome, and Mevalonate Kinase Deficiency/Hyperimmunoglobulinemia D Syndrome [PDF]
Objective: Periodic fever syndrome (PFS) conditions are characterized by recurrent attacks of fever and localized inflammation. This study examined the diagnostic pathway and treatments at tertiary centers for familial Mediterranean fever (FMF), tumor ...
Cantarini, L +19 more
core +2 more sources
ABSTRACT Erythrokeratodermia cardiomyopathy (EKC) syndrome is a rare autosomal dominant disorder characterized by generalized erythrokeratoderma and progressive dilated cardiomyopathy, caused by pathogenic variants in the SR6 domain of desmoplakin (DSP).
Sepideh Hamzehlou +7 more
wiley +1 more source
Acrodermatitis continua of Hallopeau: clinical perspectives. [PDF]
Acrodermatitis continua of Hallopeau (ACH) is a rare, sterile pustular eruption of one or more digits. The condition presents with tender pustules and underlying erythema on the tip of a digit, more frequently arising on a finger than a toe.
Beck, Kristen M +5 more
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Managing Adult-onset Still's disease: The effectiveness of high-dosage of corticosteroids as first-line treatment in inducing the clinical remission. Results from an observational study [PDF]
To assess the effectiveness of the treatment with high dosage of corticosteroids (CCSs), as first-line therapy, in inducing remission in naïve Adult-onset Still's disease (AOSD) patients compared with low dosage of CCSs, after 6 months.
Berardicurti O. +7 more
core +1 more source
Dermatologic Findings of RELA‐Associated Autoinflammatory Disease
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 +4 more
wiley +1 more source
A large pericardial effusion and bilateral pleural effusions as the initial manifestations of Familial Mediterranean Fever [PDF]
Familial Mediterranean Fever (FMF) is a condition characterized by recurrent febrile poly-serositis. Typical presentations of the disease include episodes of fever, abdominal pain and joint pains. Chest pain is a less common presentation.
Cassar Demarco, Daniela +4 more
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