Aim: Adult-onset Still's disease (AOSD) is a rare disease. Very few cases have been reported from the South-Asian region so the aim of this study is to assess the clinical and laboratory aspects of 15 patients with AOSD in a tertiary referral hospital in Karachi.
Methods: Retrospective data was collected from all patients diagnosed using Yamaguchi criteria for AOSD between January 2004 and December 2010 at Jinnah Medical College Hospital, Karachi.
Results: Data of 15 patients with AOSD were analyzed. Their ages ranged from 17 to 55 years, the male-to-female ratio being 6:1. The most common clinical features were fever and articular symptoms (100%), sore throat (60%), rash (53.3%), weight loss (93.3%), lymphadenopathy (40%) and elevated erythrocyte sedimentation rate (86.7%). All patients had leukocytosis with counts>20,000/mm 3 were seen in 40%. Elevated liver enzymes were present in 80% of the case series and hyperferritinemia in 100% with a mean of 3,962 ng/mL (range 555-13,865). Ambiguity in presentation and lack of serologic markers make diagnosis of AOSD difficult as 40% of patients were receiving empirical anti-tuberculous therapy prior to final diagnosis.
Conclusion: It is necessary for physicians to have a high index of suspicion for AOSD in patients with high-grade fever, arthralgia and leukocytosis.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.1111/j.1756-185X.2012.01812.x | DOI Listing |
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.
Int Immunopharmacol
January 2025
Institute of Medicine, Chung Shan Medical University, Taichung 402, Taiwan; Department of Clinical Laboratory, Chung Shan Medical University Hospital, Taichung 402, Taiwan; Immunology Research Center, Chung Shan Medical University, Taichung 402, Taiwan. Electronic address:
Parvovirus B19 (B19V) is a human pathogen from the Parvoviridae family that primarily targets and replicates in erythroid progenitor cells (EPCs). While its symptoms are typically self-limiting in healthy individuals, B19V can cause or exacerbate autoimmune diseases in vulnerable patients. This review integrates the involvement of B19V in the development and worsening of several autoimmune diseases, including systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), juvenile idiopathic arthritis (JIA), hematological disorders (thalassemia, anemia, and thrombocytopenia), vasculitis, antiphospholipid syndrome (APS), dermatological disease (systemic sclerosis, psoriasis), autoimmune thyroid disease, myocarditis, and myasthenia gravis, and autoinflammatory disease of adult-onset Still's disease (AOSD).
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 PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!