A controlled study of the long-term prognosis of adult still's disease

Sampalis, John S.; Esdaile, John M.; Medsger, Thomas A.; Partridge, Alison J.; Yeadon, C; Sénécal, J; Myhal, D; Harth, Manfred; Gutkowski, Andrzej; Carette, Simon; Beaudet, F; Cush, John J.; Fries, James F.

doi:10.1016/s0002-9343(99)80318-0

Cited by 55 publications

(24 citation statements)

References 16 publications

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“…It affects young adults, with a higher prevalence in women [2–4] and most commonly presents with high daily spiking fever, arthritis and evanescent rash. Other clinical features include sore throat, elevated liver enzymes, lymphadenopathy, hepatosplenomegaly, and serositis [5–7].…”

Section: Introductionmentioning

confidence: 99%

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Adult-onset Still’s disease: evaluation of prognostic tools and validation of the systemic score by analysis of 100 cases from three centers

Ruscitti

Cipriani

Masedu

et al. 2016

BMC Med

143

110

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BackgroundAdult-onset Still’s disease (AOSD) is rare inflammatory disease of unknown etiology that usually affects young adults. The more common clinical manifestations are spiking fevers, arthritis, evanescent rash, elevated liver enzymes, lymphadenopathy, hepatosplenomegaly, and serositis. The multi-visceral involvement of the disease and the different complications, such as macrophage activation syndrome, may strongly decrease the life expectancy of AOSD patients.MethodsThis study aimed to identify the positive and negative features correlated with the outcome of patients. A retrospective analysis of AOSD patients prospectively admitted to three rheumatologic centers was performed to identify the clinical features present at the time of diagnosis and to predict the possible outcome. Furthermore, we investigated the as yet to be validated prognostic value of the systemic score previously proposed.ResultsOne hundred consecutive AOSD patients were enrolled. The mean systemic score showed that the majority of patients had a multi-organ involvement. Sixteen patients showed different complications, mainly the macrophage activation syndrome. A strong increase of inflammatory markers was observed. All patients received steroids at different dosages, 55 patients in association with immunosuppressive drugs and 32 in association with biologic agents. Sixteen patients died during the follow-up. Regression analysis showed that the higher values of the systemic score and the presence of AOSD-related complications, assessed at the time of diagnosis, were significantly correlated with patient mortality. A prognostic impact of the systemic score of ≥ 7.0 was reported.ConclusionsOur study showed that a higher systemic score and the presence of AOSD-related complications at the time of diagnosis were significantly associated with mortality. Of note, a cut-off at 7.0 of the systemic score showed a strong prognostic impact in identifying patients at risk of AOSD-related death.

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Section: Introductionmentioning

confidence: 99%

“…At present, only few studies have focused on the prognostic factors of the disease [2, 4–6]. Moreover, most studies were single center studies based on a limited number of patients.…”

Section: Introductionmentioning

confidence: 99%

Adult-onset Still’s disease: evaluation of prognostic tools and validation of the systemic score by analysis of 100 cases from three centers

Ruscitti

Cipriani

Masedu

et al. 2016

BMC Med

143

110

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“…A fever higher than 39.5 °C mainly occurs in systemic AOSD, whereas a leukocyte count higher than 30,000/mm 3 and systemic inflammation markers are correlated with relapses [12]. Increased serum ferritin levels are associated with disease activity and chronic or recurrent forms of AOSD [7, 12–14]. In 1991, from a study on 62 patients, Pouchot et al [5] proposed a ‘systemic score’ reaching up to 12 points, and assigning one point to each of the following manifestations: fever, skin rash, pleuritis, pneumonia, pericarditis, liver involvement, spleen involvement, lymphadenopathy, leucocytosis > 15,000/mm 3 , sore throat, myalgia, and abdominal pain.…”

Section: Introductionmentioning

confidence: 99%

About the complexity of adult onset Still’s disease… and advances still required for its management

Guilpain

Quellec

2017

BMC Med

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Adult onset Still’s disease (AOSD) is a rare inflammatory disorder that remains poorly understood. Its pathophysiology is yet to be completely elucidated, but is known to consist mainly on a cytokine cascade, responsible for the systemic manifestations. AOSD diagnosis is usually difficult and delayed, with physicians having to rule out several other conditions, including cancer or infectious diseases. Prognosis is heterogeneous and difficult to establish, ranging from benign outcome to chronic destructive polyarthritis and/or life-threatening events. In addition, treatment remains to be codified, especially considering the development of new drugs. In this commentary, we attempt to elucidate the complexity of AOSD and to highlight the need of working on prognostic tools for this disorder. We also discuss the numerous advances that would be useful for patients in the daily management of this disease.Please see related article: http://bmcmedicine.biomedcentral.com/articles/10.1186/s12916-016-0738-8.

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“…epidemiological studies, however, have been conducted [2][3][4] and no large-scale survey has been carried out. Therefore, w have only a little information on the prevalence or incidence o the disease 3).…”

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confidence: 99%

Estimated Prevalence and Incidence of Adult Still's Disease: Findings by a Nationwide Epidemiological Survey in Japan

Wakai

Ohta

Tamakoshi

et al. 1997

Journal of Epidemiology

145

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A controlled study of the long-term prognosis of adult still's disease

Cited by 55 publications

References 16 publications

Adult-onset Still’s disease: evaluation of prognostic tools and validation of the systemic score by analysis of 100 cases from three centers

Adult-onset Still’s disease: evaluation of prognostic tools and validation of the systemic score by analysis of 100 cases from three centers

About the complexity of adult onset Still’s disease… and advances still required for its management

Estimated Prevalence and Incidence of Adult Still's Disease: Findings by a Nationwide Epidemiological Survey in Japan

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