About 8 results

Case Report: VEXAS Syndrome: From Mild Symptoms to Life-Threatening Macrophage Activation Syndrome
Frontiers in Immunology; Staels F, Betrains A et. al.

Recently, a novel disorder coined VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome was identified in patients with adult-onset inflammatory syndromes, often accompanied by myelodysplastic syndrome1. All patients had myeloid lineage-restricted somatic mutations in UBA1 affecting the Met41 residue of the protein and resulting in decreased cellular ubiquitylation activity ...

Still Disease
StatPearls; Juhi Bhargava, Sreelakshmi Panginikkod

Jul 10th, 2021 - Adult Onset Still's Disease, also known as systemic onset juvenile idiopathic arthritis, is a rare systemic inflammatory disorder characterized by inflammatory polyarthritis, daily fever, and a transient salmon-pink maculopapular rash. A serum ferritin level of more than 1000 ng/ml is common in this condition. This activity highlights the role of the interprofessional team in caring for patient...

New Guideline Addresses Childhood SLE

Jul 3rd, 2017 - Action Points Note that these guidelines for the diagnosis and management of childhood-onset SLE were developed by the Single Hub and Access Point for Pediatric Rheumatology in Europe (SHARE) project. Broadly, the diagnostic criteria are similar to those used for adult SLE. Evidence-based recommendations on the comprehensive management of childhood-onset systemic lupus erythematosus (cSLE) have...