We report the seventh published case of thrombotic thrombocytopenic purpura (TTP) associated to a Still's disease. We confirmed the secondary character of TTP using the measurement of ADAMTS 13 protease. Because of clinical and biological improvement, daily plasmapheresis was stopped after eight days of ICU treatment. Unfortunately, early (24 hours) relapse occurred resulting in daily plasmapheresis resumption for 11 more days. Main therapeutic goals and aggressive treatment duration of PTT associated with a Still's disease remain to be determined.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.1016/j.annfar.2006.01.014 | 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.
J 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 PDFSci Rep
December 2024
International Collaboration On Repair Discoveries, School of Biomedical Engineering, University of British Columbia, Vancouver, BC, Canada.
Cytokine storm syndromes such as hemophagocytic lymphohistiocytosis (HLH), Adult-onset Still's disease (AOSD), and COVID-19 cytokine storm (CCS) are characterized by markedly elevated inflammatory cytokines. However clinical measurement of serum cytokines is not widely available. This study examined the clinical utility of C-reactive protein (CRP) and ferritin, two inexpensive and widely available inflammatory markers, for distinguishing HLH from AOSD and CCS.
View Article and Find Full Text PDFRev 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.
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!