This clinical case series describes our experience with the use of Janus kinase 1/2 inhibitor baricitinib in two patients suffering from refractory adult-onset Still's disease (AOSD) as well as in one case suffering from AOSD-like autoinflammatory disease in the context of myelodysplastic syndrome. All patients suffered from disease non-responsive to conventional Disease-modifying antirheumatic drugs (DMARDs) as well as biological therapies including interleukin (IL)-1 and IL-6 blockade, relying instead on high daily doses of prednisolone. We also report the first case of infection following baricitinib use.
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http://dx.doi.org/10.1136/rmdopen-2020-001246 | DOI Listing |
Rheumatology (Oxford)
January 2025
Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy.
Objectives: To assess the lung involvement in patients with Still's disease, an inflammatory disease assessing both children and adults. To exploit possible associated factors for parenchymal lung involvement in these patients.
Methods: A multicentre observational study was arranged assessing consecutive patients with Still's disease characterized by the lung involvement among those included in the AIDA (AutoInflammatory Disease Alliance) Network Still's Disease Registry.
J Coll Physicians Surg Pak
January 2025
Department of Rheumatology and Immunology, The Second Affiliated Hospital of Anhui Medical University, Anhui, China.
Objective: To investigate the characteristics of Adult-onset Still's disease (AOSD) patients with macrophage activation syndrome (MAS) and explore the risk factors for the development of MAS.
Study Design: A case-control study. Place and Duration of the Study: Department of Rheumatology and Immunology, the Second Hospital of Anhui Medical University, Anhui, China, from January 2008 to June 2024.
Clin Case Rep
January 2025
Department of Rheumatology, Institute of Medicine Tribhuvan University Maharajgunj Kathmandu Nepal.
Hemophagocytic lymphohistiocytosis (HLH), is a fatal systemic hyperinflammatory syndrome. HLH may be due to immunosuppression, infections, cancer, or autoimmune diseases with fever and cytopenia. HLH which occurs in adult-onset Stills disease (AOSD) is called secondary HLH, also known as macrophage activation syndrome (MAS).
View Article and Find Full Text PDFJ Clin Med
January 2025
Department of Pediatric Rheumatology, Institut de Recerca Sant Joan de Déu, Hospital Sant Joan de Déu, Universitat de Barcelona, 08950 Barcelona, Spain.
To investigate the prevalence and clinical spectrum of atypical or non-classical complications in adult-onset Still's disease (AOSD) beyond macrophage activation syndrome (MAS) and to identify factors linked to their occurrence. Multicenter cross-sectional study of AODS cases included in the Spanish registry on Still's disease. This study included 107 patients (67% women), of whom 64 (59.
View Article and Find Full Text PDFDiagnostics (Basel)
December 2024
Divison of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Bursa Uludag University, 16059 Bursa, Turkey.
Background/objectives: The objective of this study was to evaluate the diagnostic accuracy of the lactate dehydrogenase-to-albumin ratio (LAR) in adult-onset Still's disease (AOSD) and compare it with other inflammatory indices, using patients with fever of unknown origin (FUO) as a control group due to their overlapping clinical features with AOSD. The study also compared LAR's diagnostic performance with other inflammatory indices like the serum immune-inflammatory index (SII), ferritin/erythrocyte sedimentation rate (FER), CRP/albumin ratio (CAR), platelet/lymphocyte ratio (PLR), and neutrophil/lymphocyte ratio (NLR), as well as its combinations with FER, PLR, and ferritin (LAR + FER, LAR + PLR, LAR + ferritin).
Methods: A retrospective evaluation was conducted on 70 patients with fever of unknown cause and 78 patients with AOSD, admitted between January 2000 and December 2023 in a tertiary care hospital.
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