Efficacy of traditional and biologic agents in different clinical phenotypes of adult‐onset Still's disease

Abstract

Objective. To evaluate the efficacy of antiinflammatory agents, steroids, immunosuppressants, and biologic agents in patients with adult-onset Still's disease (AOSD) who have either chronic articular disease or nonchronic disease.Methods. Forty-five patients with AOSD were seen and followed up for at least 2 years at our institution, from 1991 to 2008. The majority of patients were treated with several therapeutic regimens; a total of 152 efficacy trials were administered. Data regarding the type of medication, the dosage used, and the outcome of these trials were collected and analyzed.Results. Our data showed that the efficacy of monotherapy with a nonsteroidal antiinflammatory drug was very low (16%) and confirmed good efficacy of steroid therapy (63%), particularly in patients without chronic articular disease (78%). Patients whose disease did not respond to steroid therapy at the time of disease onset were at risk of the subsequent development of chronic arthritis. Disease-modifying antirheumatic drug (DMARD) monotherapy was successful in controlling steroid-resistant or steroid-dependent disease in 60% of patients. Methotrexate and cyclosporine showed the best response rates. The combination of high-dose steroids and cyclosporine was administered to successfully control some acute life-threatening complications. Only 6 patients had disease that was both steroid resistant and DMARD resistant. Treatment with biologic agents eventually led to satisfactory control of disease manifestations in 5 (83%) of these 6 patients.Conclusion. Steroids were less effective in patients with chronic articular disease than in those with nonchronic disease. The administration of DMARDs early after disease onset could be beneficial in patients with steroid-resistant disease who are at risk of the development of chronic articular disease. Biologic agents proved to be highly effective in both steroidresistant and DMARD-resistant AOSD.Selecting the appropriate treatment of adultonset Still's disease (AOSD) relies primarily on information obtained from case reports or small case series; very few randomized clinical trials have been conducted. In particular, treatment using traditional immunosuppressant drugs in AOSD is marked by an absence of prospective trials, comparative studies, or large-scale retrospective studies (1).AOSD is characterized by a high variability in the possible clinical presentation (mild manifestations versus acute life-threatening complications) and in the disease course (self-limited versus intermittent versus chronic); nevertheless, adequate data for establishing a more tailored therapy strategy are largely lacking.In this study, we reviewed and analyzed our clinical experience with patients with AOSD and formulated some suggestions that could possibly prove to be useful when selecting the proper therapeutic regimen for a patient affected by this rare disease.

Adult-onset Still’s disease

“…Уста-новлено также, что у больных, не отвечающих на ГК, в начале болезни повышен риск развития хронического артрита (p<0,001). БПВП эффективны у 60% стероидо-резистентных больных, а ГИБП -у 83% больных, рези-стенных к ГК и БПВП [100]. Сообщение об успешном применении абатацепта при БСРВ, не отвечавшей на лечение БПВП, ингибиторами ФНО α и анакинрой, указывает на важную роль активации Т-клеток в рефра-ктерных случаях [101].…”

Médecine interne et réanimation — Maladie de Still de l’adulte

“…Près de 700 patients ont été décrits [3,4] mais les séries ne dépassent pas quelques dizaines de cas. La MSA débute à l'âge adulte avec deux pics de fréquence (15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25) ans et 36-46 ans) mais deux tiers des cas sont diagnostiqués avant 40 ans [3]. Elle peut aussi être la résurgence d'une maladie de Still de l'enfance qui se serait éteinte (10 à 15 % des cas).…”

“…Historiquement, l'aspirine à fortes doses a été utilisée. Les AINS ont été ensuite proposés en première intention mais ne sont efficaces que chez 10 à 20 % des patients [3,4,15]. Ils sont réservés aux formes modérées.…”

“…Leur efficacité est en moyenne de 40 à 60 %, conduisant parfois à en tester successivement plusieurs [4,15]. L'expé-rience la plus importante est celle avec le méthotrexate qui est particulièrement efficace dans les formes articulaires chroniques.…”

Steroid-sparing Effects of Etanercept in a Patient with Steroid-dependent Adult-onset Still's Disease

“…Nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids and disease-modifying antirheumatic drugs (DMARDs) are used to control the fever, arthritis and systemic manifestations of this disorder. Steroid monotherapy is used to achieve satisfactory control of the disease in more than three-fourths of patients with SD-type AOSD (1). In steroid-resistant or steroid-dependent patients, the initiation of immunosuppressant therapy is often sufficient to control the disease activity.…”