- High-titer anti-interferon-γ neutralizing autoantibodies linked to opportunistic infections in patients with adult-onset still's disease. [Journal Article]Front Med (Lausanne). 2022; 9:1097514.FM
- CONCLUSIONS: AOSD patients have a high positive rate and titers of anti-IFN-γ autoantibodies. The remarkable blockade effect of high-titer autoantibodies on IFN-γ-mediated STAT1-phosphorylation and chemokines could make these patients susceptible to OIs.
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- Adult-onset still's disease and budd-chiari syndrome: A case report. [Case Reports]
- Adult onset still's disease (AOSD) is a rare autoinflammatory disease displaying with a wide range of non-specific symptoms and budd-chiari syndrome (BCS) is an uncommon disorder characterized by obstruction of hepatic venous outflow. We present the case of a young patient who presented with persistent fever, sore throat, elbow, hand fingers and knees arthralgia with abdominal pain. The patient's…
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- Proposal of a new diagnostic algorithm for adult-onset Still's disease. [Journal Article]Clin Rheumatol. 2023 Jan 25 [Online ahead of print]CR
- CONCLUSIONS: The proposed new algorithm could be a good diagnostic tool for adult-onset Still's disease in clinical practice and research. Key Points • A diagnostic algorithm was performed to help the physician in the diagnostic approach of AOSD. • The points-based score included in this algorithm had a high sensitivity and accuracy. • This diagnostic algorithm can be useful in the clinical research.
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- Development and validation of the AF score for diagnosis of adult-onset Still's disease in fever of unknown origin. [Journal Article]
- CONCLUSIONS: We developed and validated a new score which can identify AOSD in FUO with higher classification accuracy than Yamaguchi's criteria. Future multi-centric prospective studies need to be designed to confirm the diagnosis value of AF score.
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- Refractory adult-onset Still's disease complicated with monoclonal gammopathy of undetermined significance: A case report. [Case Reports]
- CONCLUSIONS: The disease activity of AOSD is exacerbated by multiple factors, including comorbidities or infections. Clinicians need to consider that monoclonal gammopathy of undetermined significance complications might become AOSD refractory by an elevation of the inflammatory cytokines. Moreover, further prospective studies are required to confirm this result.
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- Intravenous anakinra to curb cytokine storm in adult-onset Still's disease and in macrophage activation syndrome: A case series. [Case Reports]Joint Bone Spine. 2023 Jan 06; 90(2):105524.JB
- CONCLUSIONS: We found that administration of anakinra in patients with newly-diagnosed AOSD and/or upcoming MAS reduced hyperinflammation and prevented life-threatening complications. The IV route appears to be preferable in the hospital setting, where comorbidities such as coagulopathies and thrombocytopenia can complicate the use of other routes of administration.
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- Differential diagnostic performance of PET/CT in adult-onset still's disease and lymphoma: a retrospective pilot study. [Journal Article]
- CONCLUSIONS: Our scoring model showed good diagnosis performance in distinguishing AOSD from lymphoma.
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- Canakinumab as first-line biological therapy in Still's disease and differences between the systemic and the chronic-articular courses: Real-life experience from the international AIDA registry. [Journal Article]
- CONCLUSIONS: Canakinumab used for Still's disease has been effective in controlling both clinical and laboratory manifestations disregarding the type of disease course when used as first-line biotechnological agent. These excellent results might have been further enhanced by the early start of IL-1 inhibition.
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- Differences and similarities in cytokine profiles of macrophage activation syndrome in systemic lupus erythematosus and adult-onset Still's disease. [Journal Article]Clin Exp Med. 2023 Jan 07 [Online ahead of print]CE
- To clarify the differences and similarities in the cytokine profiles of macrophage activating syndrome (MAS) between systemic lupus erythematosus (SLE) and adult-onset Still's disease (AOSD). The study participants included 9 patients with MAS-SLE, 22 with non-MAS-SLE, 9 with MAS-AOSD, and 13 with non-MAS-AOSD. Serum cytokine levels were measured using a multiplex bead assay. Cytokine levels were…
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- mTORC1 implicated in Still's disease and MAS. [Journal Article]Nat Rev Rheumatol. 2023 Feb; 19(2):64.NR
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- Ferritin-induced NETs lead to cytokine storm in AOSD. [Journal Article]Nat Rev Rheumatol. 2023 Feb; 19(2):61.NR
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- The Expanding Spectrum of Autoinflammatory Diseases. [Journal Article]
- Autoinflammatory diseases are systemic disorders caused by genetic or acquired abnormalities in certain signaling pathways of the innate immune system. Dysregulated activation of the inflammasome, i.e. molecular platforms responsible for the activation of caspase-1 and production of interleukin-1β, causes autoinflammation. Familial Mediterranean fever (FMF), the most common genetic autoinflammato…
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- Comprehensive description of adult-onset Still's disease after COVID-19 vaccination. [Journal Article]
- Cases of adult-onset Still's disease (AOSD) have been reported after COVID-19 vaccination. Here we provide a comprehensive description and analysis of all cases of AOSD reported in the literature and in pharmacovigilance databases through April 2022. Disproportionality analyses of pharmacovigilance data were performed in order to further explore the association between vaccination and AOSD. We in…
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- Response letter to "Latent class analysis of 216 patients with adult-onset Still's disease" by Sugiyama et al. [Letter]
- Sugiyama et al. recently described in "Latent class analysis of 216 patients with adult-onset Still's disease," baseline characteristics, laboratory tests, treatment, relapse, and death of adult-onset Still's disease (AOSD) patients from a Japanese hospital. They identified two subgroups: Class 1 (n=155) with a younger age and typical symptoms of AOSD and Class 2 (n=61) with older patients and fe…
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- First report of a patient meeting criteria for both multisystem inflammatory syndrome in children and adult onset Still's disease. [Journal Article]
- CONCLUSIONS: MAS is a life-threatening rheumatological emergency, and physicians must be able to identify diseases, like MIS-C and AOSD, that may be complicated by MAS. Our patient's distinguishing feature on presentation was symmetrical polyarticular arthralgia/arthritis, which has not been associated with MIS-C. Simultaneously, AOSD-which is associated with polyarticular arthralgia/arthritis-is only now being recognized as a possible post-infectious entity in the aftermath of COVID-19 infection. In patients like our own, who meet criteria for both MIS-C and AOSD, administering first line treatment for both diseases may be best practice.
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- Rare But Fatal: Hemophagocytic Lymphohistiocytosis (HLH) With Acute Acalculous Cholecystitis. [Case Reports]
- Acalculous cholecystitis is an acute inflammatory disease of the gall bladder with high morbidity and mortality rate. It can be seen in trauma, burns, sepsis, total parenteral nutrition, prolonged fasting, and autoimmune diseases. However, there are very few reports of acalculous cholecystitis with macrophage activation syndrome (MAS) and hemophagocytic lymphohistiocytosis (HLH) in patients with …
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- Bilateral dacryoadenitis in adult-onset Still's disease: A case report. [Case Reports]
- We present an unusual case of bilateral dacryoadenitis in a middle-aged patient with adult-onset Still's disease (AOSD). We reviewed relevant clinical studies addressing the association between lacrimal lesions and AOSD. A 50-year-old Chinese woman with a 4 year history of recurrent fever and rashes was admitted to the hospital. She had also developed nodules on both eyelids 10 months before admi…
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- Immune-Mediated Diseases Following COVID-19 Vaccination: Report of a Teaching Hospital-Based Case-Series. [Journal Article]
- The occurrence and course of immune-mediated diseases (IMDs) following COVID-19 vaccination has been little explored so far. We retrieved, among adult patients hospitalized at the Internal Department of a French university hospital up to May 2022, all those who had developed, or relapsed to, an IMD less than 3 weeks following COVID-19 vaccination, without other triggers. Twenty-seven (24 new-onse…
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- Diagnosis and treatment of adult-onset Still's disease: a concise summary of the German society of rheumatology S2 guideline. [Review]Z Rheumatol. 2022 Dec 15 [Online ahead of print]ZR
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- Ruxolitinib rescued the macrophage activation syndrome in adult-onset Still's disease with delayed hypersensitivity reaction to tocilizumab. [Journal Article]Rheumatology (Oxford). 2022 Dec 15 [Online ahead of print]R
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- [Adult-onset Still's disease : Diagnosis and treatment according to the new S2e guidelines of the German Society of Rheumatology (DGRh)]. [Journal Article]Z Rheumatol. 2022 Dec 14 [Online ahead of print]ZR
- Adult-onset Still's disease (AOSD) is a rare autoinflammatory disease characterized by intermittent fever and a combination of symptoms, such as an evanescent rash synchronous with fever, arthralgia/arthritis, lymphadenopathy and hepatosplenomegaly. The diagnosis is based on a characteristic constellation of symptoms and the exclusion of infections, hemato-oncological diseases, infectious disease…
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- A Streamlined Diagnostic Process Improved the Outcomes of Patients with Adult-Onset Still's Disease: A Single-Center Retrospective Observational Study. [Journal Article]
- CONCLUSIONS: Implementing an SDP for expediting diagnosis could improve outcomes for AOSD patients. This diagnostic process increased the early diagnosis rate and led to a higher disease remission rate. However, the beneficial effects of SDP implementation need further external validation.
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- Clinical features and serum cytokine profiles of elderly-onset adult-onset Still's disease. [Journal Article]
- Recent studies have suggested that the clinical features of elderly-onset adult-onset Still's disease (AOSD) differ from those of young and middle-aged-onset patients, whereas the details remain unclear, and cytokine profiles of elderly-onset AOSD have not been reported. To clarify the clinical features and cytokine profiles of elderly-onset AOSD, we examined patients with AOSD who developed the …
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- Adult-onset Still's disease presenting with "pencil sketch-like" arrangement of papules and geographic pigmentation. [Letter]J Cosmet Dermatol. 2022 Dec 02 [Online ahead of print]JC
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- mTORC1 links pathology in experimental models of Still's disease and macrophage activation syndrome. [Journal Article]
- Still's disease is a severe inflammatory syndrome characterized by fever, skin rash and arthritis affecting children and adults. Patients with Still's disease may also develop macrophage activation syndrome, a potentially fatal complication of immune dysregulation resulting in cytokine storm. Here we show that mTORC1 (mechanistic target of rapamycin complex 1) underpins the pathology of Still's d…
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- Systematic review on the use of biologics in adult-onset still's disease. [Review]Semin Arthritis Rheum. 2023 02; 58:152139.SA
- This systematic review (SR) describes the efficacy and safety of biologic disease modifying anti-rheumatic drugs (bDMARDs) for patients with adult-onset Still's disease (AOSD). Three randomised controlled trials (RCTs), one retrospective case series of multiple interventions, and 17 case series of single interventions met the inclusion criteria for this SR. Comparisons of biologic therapy in AOSD…
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- The Spectrum of Still's Disease: A Comparative Analysis of Phenotypic Forms in a Cohort of 238 Patients. [Journal Article]
- Still's disease (SD) is a heterogeneous autoinflammatory disorder for which several phenotypes have been described. We conducted a retrospective study to re-evaluate the dichotomous view of the disease, to compare the juvenile and adult forms, and to look for prognostic factors. We collected data from ten French centers, seeking patients with a diagnosis of adult-onset SD (AOSD) or systemic juven…
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- Adult-onset Still's disease after ChAdOx1 nCoV-19 vaccine: a possible association. [Case Reports]
- With emergent Sars-Cov-2, a highly transmissive virus that caused millions of deaths worldwide, the development of vaccines became urgent to combat COVID-19. Although rare, important adverse effects had been described in a hypothetical scenario of immune system overstimulation or overreaction. Still's disease is a rare inflammatory syndrome of unknown etiology. It manifests as a cytokine storm, m…
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- Derivation and validation of adult Still Activity Score (SAS). [Multicenter Study]
- CONCLUSIONS: SAS has shown a good test performance to distinguish active AOSD patients from others. SAS may be a useful method for evaluating the disease activity of AOSD patients in daily practice.
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