Results 81 to 90 of about 161,044 (231)

Predictive factors for therapeutic response and cluster analysis in syndrome of undifferentiated recurrent fever (SURF)

open access: yesRMD Open
Introduction: Syndrome of undifferentiated recurrent fever (SURF) refers to a group of recurrent fevers without a clear monogenic cause. Clinical spectrum, treatment response predictors and management strategies remain unclear. Objective: This study aims
Marco Gattorno   +15 more
doaj   +1 more source

Current Therapeutic Options for the Main Monogenic Autoinflammatory Diseases and PFAPA Syndrome: Evidence-Based Approach and Proposal of a Practical Guide

open access: yesFrontiers in Immunology, 2020
Monogenic autoinflammatory diseases are rare conditions caused by genetic abnormalities affecting the innate immunity. Previous therapeutic strategies had been mainly based on results from retrospective studies and physicians' experience. However, during
Alessandra Soriano   +6 more
doaj   +1 more source

Juvenile Gangrenous Vasculitis of the Scrotum or Scrotum Lipschütz Ulcers

open access: yesJEADV Clinical Practice, EarlyView.
ABSTRACT Lipschütz genital ulcers are traditionally described in adolescent females and often linked to acute viral infections. Their male counterpart remains poorly characterised. We report a 59‐year‐old man with sudden onset of unilateral necrotic ulcers on the scrotum and inguinal region following a brief febrile episode and mild pharyngitis ...
Francesco Bellinato   +2 more
wiley   +1 more source

The International Guideline for the Definition, Classification, Diagnosis and Management of Urticaria

open access: yesAllergy, EarlyView.
ABSTRACT This update and revision of the international guideline for urticaria was developed in accordance with the methods recommended by Cochrane and the Grading of Recommendations Assessment, Development and Evaluation (GRADE) working group. It is an initiative of the Global Allergy and Asthma Excellence Network (GA2LEN) and its Urticaria and ...
T. Zuberbier   +221 more
wiley   +1 more source

Autosomal Dominant Hyper‐IgE Syndrome Patients Retain IL10‐Producing preTh17‐Cells That Are Activated by Opportunistic Pathogens and Support IgE Production

open access: yesAllergy, EarlyView.
IL‐10 producing CCR6+Th‐cells are central memory T‐cells that express ROR‐γt and differentiate to Th17‐cells via an autocrine loop of STAT3‐activating cytokines (preTh17). STAT3‐deficient AD‐HIES patients lack Th17‐ and Tfh17‐cells but retain preTh17‐ and Th1/17‐cells.
Giorgia Moschetti   +18 more
wiley   +1 more source

Autoinflammatory diseases: An immunologist’s view

open access: yesСовременная ревматология, 2015
Autoinflammatory syndromes (AISs) are a heterogeneous group of genetically determined diseases, whose basis is the dysregulated mechanisms of inflammation.
A. Yu. Shcherbina
doaj   +1 more source

Development of the autoinflammatory diseases damage index (ADDI) [PDF]

open access: yes, 2016
OBJECTIVES: Autoinflammatory diseases cause systemic inflammation that can result in damage to multiple organs. A validated instrument is essential to quantify damage in individual patients and to compare disease outcomes in clinical studies. Currently,
Ravelli, A   +102 more
core   +1 more source

Haemostatic changes and bleeding with anti‐IL‐6 directed therapy in autoimmune diseases

open access: yesBritish Journal of Pharmacology, EarlyView.
Abstract Anti‐IL‐6 directed therapy, especially tocilizumab (TCZ), is widely used for the treatment of autoimmune diseases such as rheumatoid arthritis, giant cell arteritis and systemic juvenile idiopathic arthritis. Next to being a master regulator of inflammation, IL‐6 also is an important regulator of haemostasis. Although generally well tolerated,
Charlotte D. C. C. van der Heijden   +3 more
wiley   +1 more source

Dysfunctional immunoproteasomes in autoinflammatory diseases [PDF]

open access: yes, 2016
Recent progress in DNA sequencing technology has made it possible to identify specific genetic mutations in familial disorders. For example, autoinflammatory syndromes are caused by mutations in gene coding for immunoproteasomes.
有持, 秀喜   +22 more
core  

An Atypical Skin Presentation of Adult‐Onset Still's Disease With Histological Description: A Case Report

open access: yesJournal of Cutaneous Pathology, EarlyView.
ABSTRACT Adult‐onset Still's disease (AOSD) is an auto inflammatory disorder with a variable clinical presentation, and without a pathognomonic diagnostic test, characterized by high spiking fever, arthralgia/arthritis, a suggestive skin rash, elevated white blood cell count 10 G/L (> 80% neutrophils), elevated ferritin, with glycosylated ferritin < 20%
M. Nordmann   +7 more
wiley   +1 more source

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