Disseminierte intravasale Gerinnung, Perimyokarditis und bilaterales Pleuraempyem bei adultem Still-Syndrom

Krzossok, Stefan; Benck, Urs; Woude, Fokko J. van der; Braun, C.

doi:10.1055/s-2004-835297

Cited by 5 publications

(2 citation statements)

References 3 publications

(7 reference statements)

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“…The current treatment strategy for Still’s disease typically includes NSAIDs to relieve symptoms during the differential diagnostic process while reaching a final diagnosis. Glucocorticoids (GCs) are commonly used as first-line treatment once a diagnosis is made [13–15] and DMARDs are often considered in combination with GCs [16]. Biologic DMARDs (bDMARD) offer a more target-specific mechanism of action than regular DMARDs and have, for this reason, emerged as an important therapeutic alternative in patients with Still’s disease of all ages.…”

Section: Introductionmentioning

confidence: 99%

Anakinra in children and adults with Still’s disease

et al. 2019

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Systemic juvenile idiopathic arthritis and adult-onset Still’s disease are rare autoinflammatory disorders with common features, supporting the recognition of these being one disease—Still’s disease—with different ages of onset. Anakinra was recently approved by the European Medicines Agency for Still’s disease. In this review we discuss the reasoning for considering Still’s disease as one disease and present anakinra efficacy and safety based on the available literature. The analysis of 27 studies showed that response to anakinra in Still’s disease was remarkable, with clinically inactive disease or the equivalent reported for 23–100% of patients. Glucocorticoid reduction and/or stoppage was reported universally across the studies. In studies on paediatric patients where anakinra was used early or as first-line treatment, clinically inactive disease and successful anakinra tapering/stopping occurred in >50% of patients. Overall, current data support targeted therapy with anakinra in Still’s disease since it improves clinical outcome, especially if initiated early in the disease course.

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Section: Introductionmentioning

confidence: 99%

Anakinra in children and adults with Still’s disease

et al. 2019

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“… 1 , 2 The pathogenesis of AoSD is currently unclear, 3 and the therapeutic strategies of AoSD can be varied according to its clinical presentation. 4 – 6 Nonsteroidal anti-inflammatory drugs (NSAIDs) and corticosteroids are commonly used as first-line agents for AoSD, 7 , 8 and if first-line treatment is ineffective, disease-modifying antirheumatic drugs (DMARDs), such as methotrexate are added. 9 Corticosteroids can control the manifestations of AoSD in about 65% of patients, 10 and the response to corticosteroids is often quick, within a few hours.…”

Section: Introductionmentioning

confidence: 99%

Interleukin 1 inhibition with anakinra in adult-onset Still disease: a meta-analysis of its efficacy and safety

Hong¹,

Yang²,

Shu-yin³

et al. 2014

DDDT

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BackgroundAnakinra is the first interleukin-1 inhibitor to be used in clinical practice, and recent evidence showed that interleukin-1 plays a pivotal role in the pathogenesis of adult-onset Still disease (AoSD). However, data concerning efficacy with anakinra use in different clinical trials has not been evaluated, and the overall remission of AoSD with anakinra treatment has not been well defined.MethodsWe conducted a search on Embase, PubMed, and the Cochrane Library for relevant trials. Statistical analyses were conducted to calculate the overall remission rates, odds ratios (OR), and 95% confidence intervals (CI), by using either random effects or fixed effect models according to the heterogeneity.ResultsOf the 273 articles that were identified, 265 were excluded. Eight studies were eligible for inclusion. The overall remission rate and complete remission rate of anakinra in AoSD patients were 81.66% (95% CI: 69.51%–89.69%) and 66.75% (95% CI: 59.94%–75.3%), respectively. Compared with the controls, the use of anakinra was associated with a significant remission in AoSD, with an OR of 0.16 (95% CI: 0.06–0.44, P=0.0005). There were also significant reductions of the dosage of corticosteroid (mean difference =21.19) (95% CI: 13.2–29.18, P<0.0001) from anakinra onset to the latest follow up time. Clinical and laboratory parameters were all improved, and anakinra was well tolerated in patients with AoSD. No evidence of publication bias was observed.ConclusionOur study has shown that anakinra is effective in remitting the manifestations of AoSD, with reduction of the dose of corticosteroid in patients with AoSD. Further, anakinra therapy was not associated with increased risk of adverse events, and it was well tolerated in patients with AoSD. Further research is still recommended to investigate these findings.

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Adult-onset Still disease: a rare disorder with a potentially fatal outcome

Priori

Colafrancesco

Gattamelata

et al. 2010

Autoimmun Highlights

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Purpose The aim of this study was to assess the clinical and laboratory features of a cohort of Italian patients with adult-onset Still disease (AOSD) with particular attention on possible life-threatening complications. Methods The clinical charts of 41 consecutive Italian patients with AOSD referred to our rheumatological department over the last 10 years were retrospectively examined. Data regarding clinical manifestations, laboratory features and complications were collected and compared with those reported in literature. Results The most frequent manifestations were: fever (90.2%), arthralgias (80.4%), skin rash (75.6%), sore throat (53.6%), arthritis (51.2%), lymphadenopathy (48.7%), hepatosplenomegaly (41.4%), myalgia (21.9%), fatigue (12%), diarrhoea and vomiting (9.7%), pleural effusion (9.7%), pericardial effusion (4.8%) and abdominal pain (2.4%). In two patients whose cases are described in detail; the course of the disease was complicated by disseminated intravascular coagulopathy, in one patient with a fatal outcome. ESR, CRP and leucocyte count mean values were 69.41 mm/h, 69.05 mg/l and 18,798.5 cell/mm 3 (neutrophils 84.64%), respectively. Serum ferritin levels were increased in 48.7% of patients while transaminases were elevated in 42.6% of patients (71% considering only patients in an active phase of disease). Conclusion The results of this study are in line with those reported for other cohorts of patients. Even if the prognosis of AOSD is considered favourable, the present study indicates that the disease is a troubling condition needing prompt intervention. Occasionally, AOSD may rapidly worsen with life-threatening events.

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Disseminierte intravasale Gerinnung, Perimyokarditis und bilaterales Pleuraempyem bei adultem Still-Syndrom

Cited by 5 publications

References 3 publications

Anakinra in children and adults with Still’s disease

Anakinra in children and adults with Still’s disease

Interleukin 1 inhibition with anakinra in adult-onset Still disease: a meta-analysis of its efficacy and safety

Adult-onset Still disease: a rare disorder with a potentially fatal outcome

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Disseminierte intravasale Gerinnung, Perimyokarditis und bilaterales Pleuraempyem bei adultem Still-Syndrom

Cited by 5 publications

References 3 publications

Anakinra in children and adults with Still’s disease

Anakinra in children and adults with Still’s disease

Interleukin 1 inhibition with anakinra in adult-onset Still disease: a meta-analysis of&nbsp;its&nbsp;efficacy and safety

Adult-onset Still disease: a rare disorder with a potentially fatal outcome

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Interleukin 1 inhibition with anakinra in adult-onset Still disease: a meta-analysis of its efficacy and safety