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NLRC4-associated autoinflammatory disease: insights from mouse models and somatic variants.
J Allergy Clin Immunol
December 2024
Department of Microbiology, Immunology and Transplantation, Allergy and Clinical Immunology Research Group, KU Leuven, Leuven, Belgium; Department of General Internal Medicine, Division of Allergy and Clinical Immunology, University Hospitals Leuven, Leuven, Belgium.
Long-Term Safety and Effectiveness of Canakinumab in Patients with MKD/HIDS: Interim Analysis of the RELIANCE Registry.
Rheumatol Ther
December 2024
Division of Paediatric Rheumatology and Autoinflammation Reference Centre Tübingen, Department of Paediatrics, University Hospital Tübingen; Member of ERN-RITA, Tübingen, Germany.
Introduction: Interim analysis of the long-term safety and effectiveness of canakinumab, at a patient level, in the mevalonate kinase deficiency/hyperimmunoglobulin-D syndrome (MKD/HIDS) cohort of the RELIANCE registry.
Methods: From June 2018, the RELIANCE registry enrolled paediatric (aged ≥ 2 years) and adult patients (aged ≥ 18 years) with MKD/HIDS who were receiving canakinumab as part of their routine medical care. Safety, physician- and patient-reported measures of disease activity and dosing patterns were evaluated at baseline and every 6 months until end-of-study visit.
Pediatric uveitis.
J Family Med Prim Care
November 2024
Flinders University College of Medicine and Public Health, Adelaide, Australia.
Up to 10% of uveitis cases occur in children, with notable implications due to the risk of chronicity and vision loss. It can result from infections, autoimmune and autoinflammatory diseases, trauma, or masquerade syndromes. Primary care providers are vital in early detection, symptom management, and timely specialist referral.
View Article and Find Full Text PDF[VEXAS-like auto inflammatory syndrome: 2 cases].
Rev Med Interne
December 2024
Service de médecine interne, CHI Poissy-St Germain, 10, rue du Champs Gaillard, 78300 Poissy, France.
Introduction: VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic), recently described, due to a somatic mutation of the UBA1 gene and often associated with hemopathy, is characterized by systemic symptoms close to those described in Still's disease or relapsing polychondritis. There are also patients with hemopathy, presenting inflammatory symptoms reminiscent of those of VEXAS syndrome but without mutation of the UBA1 gene.
Case/discussion: Two male patients consulted for general signs, dermatological symptoms, arthralgia, chondritis and venous thrombosis, like patients in the French cohort suffering from VEXAS syndrome.
Persistent urticarial eruption preceding systemic features of adult-onset Still's disease: A case report.
Australas J Dermatol
December 2024
Department of Dermatology, St Vincent's Hospital Melbourne, Fitzroy, Victoria, Australia.
Adult-onset Still's disease (AOSD) is a rare autoinflammatory systemic disorder classically characterised by inflammatory polyarthritis, daily fevers and a transient asymptomatic salmon-pink maculopapular rash that typically arises with the onset of fevers. We report a case of AOSD presenting with a severely pruritic urticarial eruption starting 6 weeks prior to the onset of fever and arthritis and complicated by macrophage activation syndrome. This case highlights the importance of early recognition of diverse cutaneous manifestations of AOSD to facilitate timely diagnosis and treatment to improve disease outcomes.
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