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[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.
Breaking the storm: ruxolitinib as a lifeline in refractory hemophagocytic lymphohistiocytosis with macrophage activation syndrome.
Proc (Bayl Univ Med Cent)
July 2024
Department of Pathology, University of New Mexico School of Medicine, Albuquerque, New Mexico, USA.
This case report presents the management of a 25-year-old woman with refractory hemophagocytic lymphohistiocytosis (HLH) secondary to adult-onset Still's disease. Despite initial treatment with corticosteroids and biologic therapies, including anakinra and tocilizumab, her condition continued to deteriorate, necessitating intensive care unit admission. Following multidisciplinary consultation, ruxolitinib therapy, a Janus kinase (JAK) inhibitor targeting hyperinflammation, was initiated.
View Article and Find Full Text PDFCurrent landscape of monogenic autoinflammatory actinopathies: A literature review.
Autoimmun Rev
December 2024
Sorbonne University, Department of Internal Medicine, DMU3ID, ERN RITA, Hôpital Tenon, University, Assistance publique-hôpitaux de Paris (AP-HP), 4 rue de la Chine, 75020 Paris, France; Centre de référence des maladies autoinflammatoires et de l'amylose (CEREMAIA). Electronic address:
Autoinflammatory diseases (AID) are conditions leading to a hyperactivation of innate immunity without any underlying infection, and may be poly- (e.g. Still's disease) or monogenic.
View Article and Find Full Text PDFDelayed diagnosis of adult onset Still's disease in 2 cases: diagnostic dilemma in positive antinuclear antibody and tuberculosis endemic areas.
Postgrad Med
January 2025
Internal Medicine, Department of Internal Medicine, Faculty of Medicine, Universitas Brawijaya, Malang, Indonesia.
Article Synopsis
- Adult-onset Still's disease (AoSD) is a rare inflammatory condition affecting young adults, often misdiagnosed as other diseases.
- Two cases are outlined where AoSD was initially misidentified as tuberculous arthritis and systemic lupus erythematosus (SLE), leading to ineffective treatments.
- After further evaluation, both patients were correctly diagnosed with AoSD and treated with high-dose methylprednisolone and methotrexate, resulting in clinical improvement and reduced ferritin levels.
Lifetime clinical presentation of Still's disease in the Afro-descendant population of the French West Indies.
Joint Bone Spine
November 2024
EpiCliV Research Unit, University of the French West Indies, Martinique University Hospital, Fort-de France, Martinique; Department of Internal Medicine, Martinique University Hospital, Fort-de France, Martinique.
Introduction: The continuum in Still's disease has never been addressed in Afro-descendant (AD) populations. The aim of this study was to compare the features of Still's disease between children and adults in the AD population of French West Indies (FWI).
Methods: Retrospective longitudinal study from January 2000-2022.
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