Response rate of initial conventional treatments, disease course, and related factors of patients with adult-onset Still's disease: Data from a large multicenter cohort

Kalyoncu, Umut; Solmaz, Dilek; Emmüngil, Hakan; Yazıcı, Ayten; Kaşifoğlu, Timuçin; Kimyon, Gezmiş; Balkarlı, Ayşe; Beş, Cemal; Özmen, Mustafa; Alıbaz-Öner, Fatma; Erten, Şükran; Çağatay, Yonca; Çetin, Gözde Yıldırım; Yılmaz, Sedat; Yıldız, Fatih; Pamuk, Ömer Nurı; Küçükşahin, Orhan; Kılıç, Levent; Yazısız, Veli; Karadağ, Ömer; Koca, Süleyman Serdar; Hayran, Mutlu; Akar, Servet; Aksu, Kenan; Akkoç, Nurullah; Keser, Gökhan; Gönüllü, Emel; Kısacık, Bünyamin; Onat, Ahmet Mesut; Soy, Mehmet; İnanç, Nevsun; Direşkeneli, Haner; Sayarlıoğlu, Mehmet; Erken, Eren; Turgay, Murat; Çefle, Ayşe; Ertenli, İhsan; Pay, Salih

doi:10.1016/j.jaut.2016.02.010

Cited by 65 publications

(48 citation statements)

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“…As far as the clinical picture is concerned, all our patients experienced fever, frequently associated with both joint and skin involvement, mirroring data previously published [5, 6, 8, 27, 38–41]. In fact, it has been reported that fever occurs in 60–100% of AOSD patients, typically with the highest temperatures occurring in the evening.…”

Section: Discussionsupporting

confidence: 88%

“…Joint involvement is reported in 70–100% of these patients, mainly in wrists, knees, and ankles. At the onset of disease, arthritis may be mild and transient, sometimes evolving into a chronic destructive symmetrical polyarthritis with carpal ankylosis [5, 6, 8]. Further, 60–80% of AOSD patients experience a macular or maculopapular evanescent salmon-pink skin rash associated with the fever spiking [38–41]; some patients, those with a usually more severe outcome, may experience this skin involvement for many weeks [7, 42].…”

Section: Discussionmentioning

confidence: 99%

“…However, the favorable effects of an early diagnosis on prognosis have been underlined [8]. Basically, three different clinical patterns of AOSD have been identified: (1) monocyclic pattern, characterized by a systemic single episode; (2) polycyclic pattern, characterized by multiple flares lasting for a 1 year or longer, alternating with remissions; and (3) chronic pattern, related to a persistently active disease with associated polyarthritis [9].…”

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

138

107

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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: Discussionsupporting

confidence: 88%

Section: Discussionmentioning

confidence: 99%

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

138

107

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“…[5][6][7] Esta enfermedad puede tener curso monocíclico/autolimitado, recurrente o crónico y las formas recurrentes ocurren hasta en el 36% de los casos, presentando usualmente recaídas 18 meses después del episodio inicial. 8 El tratamiento de la ESA es empírico y proviene, principalmente, de información de series de casos retrospectivos. Las opciones terapéuticas incluyen: glucocorticoides (pilar fundamental de la terapia), antiinflamatorios no esteroideos, 9 ahorradores de esteroides (metotrexato, azatioprina) e inmunomoduladores -también llamados modificadores de la respuesta biológica-(anti-IL-1, anti-IL-6 y anti-TNF); el uso de estos últimos se ha descrito hasta en un 30% de los pacientes que no lograron remisión con fármacos de primera línea.…”

Section: Introductionunclassified

“…Las opciones terapéuticas incluyen: glucocorticoides (pilar fundamental de la terapia), antiinflamatorios no esteroideos, 9 ahorradores de esteroides (metotrexato, azatioprina) e inmunomoduladores -también llamados modificadores de la respuesta biológica-(anti-IL-1, anti-IL-6 y anti-TNF); el uso de estos últimos se ha descrito hasta en un 30% de los pacientes que no lograron remisión con fármacos de primera línea. 3,4,[8][9][10] El síndrome de activación de macrófago es la complicación más frecuente de la ESA (3.2-12.8%), seguido de miocarditis, pericarditis constrictiva, endocarditis, choque hemodinámico, síndrome de dificultad respiratoria, hemorragia alveolar, coagulación intravascular diseminada, microangiopatía trombótica y hepatitis fulminante. Respecto a los factores de riesgo para el desarrollo de estas complicaciones existe poca información.…”

Section: Introductionunclassified

Enfermedad de Still del adulto: características clínicas y pronóstico de una cohorte de pacientes colombianos

et al. 2020

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Introducción. La enfermedad de Still del adulto (ESA) es una condición médica poco frecuente que puede presentar complicaciones graves; sin embargo, aún no se conocen con claridad sus factores de riesgo, sus factores pronósticos, ni los aspectos asociados a las recaídas y a la refractariedad a esteroides en individuos con esta condición.Objetivos. Describir el comportamiento clínico de la ESA y determinar los factores asociados a la refractariedad a esteroides, a recaídas y a complicaciones en pacientes con esta enfermedad.Materiales y métodos. Estudio retrospectivo de cohortes que incluyó 45 pacientes diagnosticados con ESA entre enero de 2007 y enero de 2017 en 2 hospitales de referencia. Se presentan medidas de resumen. Se realizaron dos análisis de regresión logística, uno crudo y otro multivariado, para identificar posibles factores que expliquen la refractariedad a esteroides, las recaídas y el desarrollo de complicaciones en la población de estudio.Resultados. La edad promedio de los participantes fue 42.13±15.8 años. De los 45 pacientes incluidos, 23 (51.1%) eran mujeres, a 42 (93.3%) se le prescribieron esteroides, y, de estos, 13 (32%) se consideraron refractarios a esteroides. Se presentaron complicaciones en 12 (26.7%) individuos y su frecuencia fue mayor en pacientes con temperatura máxima >39°C. Finalmente, 33 (73.3%) pacientes tuvieron un seguimiento mayor a 1 año, de los cuales, 17 (37.8%) presentaron recaídas, las cuales fueron más frecuentes en individuos refractarios a esteroides o con esplenomegalia. Conclusión. Los pacientes que recibieron cualquier tipo de terapia biológica fueron más refractarios a esteroides; por su parte, la refractariedad a esteroides y la esplenomegalia se asociaron a un mayor número de recaídas, y la temperatura >39°C se asoció a al desarrollo de complicaciones.

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Morbus Still im Kindes- und Erwachsenenalter

2017

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Systemic juvenile idiopathic arthritis (sJIA) is characterized by fever, arthritis, and other signs of systemic inflammation. Historically, sJIA was named Still's disease after George Frederic Still, who first reported patients. Individuals who manifest after the 16 birthday are diagnosed with adult onset Still's disease (AOSD). The pathophysiology of sJIA and AOSD are incompletely understood. Increased activation of inflammasomes and the expression of proinflammatory cytokines play a central role. S100 proteins, which can activate Toll-like receptors, thus, maintaining positive feedback loops, have also been detected at increased levels in sera from sJIA patients. Reduced expression of the immune-modulatory cytokine IL-10 may further contribute to immune cell activation and the production of proinflammatory molecules. Here, we discuss the clinical picture, differential diagnoses, the current pathophysiological understanding, and treatment options in sJIA and AOSD.

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Response rate of initial conventional treatments, disease course, and related factors of patients with adult-onset Still's disease: Data from a large multicenter cohort

Cited by 65 publications

References 10 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

Enfermedad de Still del adulto: características clínicas y pronóstico de una cohorte de pacientes colombianos

Morbus Still im Kindes- und Erwachsenenalter

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