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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 PDFIntravenous Anakinra for Treating Macrophage Activation Syndrome in Adult-Onset Still's Disease.
Eur J Case Rep Intern Med
October 2024
Department of Internal Medicine, Division of Rheumatology, Baskent University, Ankara, Turkey.
Background: Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disease characterized by fever, rash, arthritis, and multi-organ involvement. Macrophage activation syndrome (MAS), a serious complication of AOSD, poses significant diagnostic and therapeutic challenges.
Case Presentation: A 32-year-old male was diagnosed with AOSD in 2020 after being hospitalized for a fever of unknown origin and elevated liver enzymes.
Adult-onset Still's disease masquerading as acute coronary syndrome: a case report and review of the literature.
J Med Case Rep
October 2024
Division of Infectious Diseases and Epidemiology, Department of Medicine, Penn State Health Milton S. Hershey Medical Center, 500 University Drive, Hershey, PA, 17033, USA.
Article Synopsis
- Adult-onset Still's disease is a rare inflammatory condition that can cause symptoms like fevers and myopericarditis, as seen in a young man who developed these after a respiratory infection.
- The patient had significant medical issues, including high fever, chest pain, and was eventually diagnosed after thorough testing; he responded well to steroids but experienced side effects before achieving remission with tocilizumab.
- Early recognition of this disease is essential for effective treatment, as steroids are the primary option despite their risks, while newer biologics like tocilizumab offer benefits but can be expensive.
Efficacy and safety of therapies for Still's disease and macrophage activation syndrome (MAS): a systematic review informing the EULAR/PReS guidelines for the management of Still's disease.
Ann Rheum Dis
November 2024
Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, ERN-RITA center, Roma, Italy.
Objectives: To analyse the efficacy and safety of treatments for Still's disease and macrophage activation syndrome (MAS).
Methods: Medline, Embase and Cochrane Library were searched for clinical trials (randomised, randomised controlled trial (RCT), controlled and clinical controlled trial (CCT)), observational studies (retrospective, longitudinal observational retrospective (LOR), prospective and longitudinal observational prospective (LOP)) and systematic reviews (SRs), in which the populations studied were patients with Still's disease and MAS. The intervention was any pharmacological treatment (approved or under evaluation) versus any comparator drug or placebo, and as outcomes, any relevant efficacy and safety event.
Anakinra in the management of adult-onset still's disease: a single-center experience.
Intern Emerg Med
September 2024
Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, Istanbul University-Cerrahpasa, 53 Kocamustafapasa Street, Fatih, 34098, Istanbul, Turkey.
Adult-onset Still's disease (AOSD) is a rare systemic autoinflammatory disorder of unknown etiology characterized by systemic inflammation, high fever, salmon-colored skin rash, arthralgia, and arthritis. Patients with AOSD may also present with elevated inflammatory markers, hyperferritinemia, anemia, leukocytosis, hepatosplenomegaly, and lymphadenopathy. Glucocorticoids and biological disease-modifying anti-rheumatic drugs, including the anti-interleukin-1 agent anakinra, are used in the management of AOSD.
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