Clinical features and prognosis in adult-onset still's disease: a study of 104 cases

Abstract

The objective of this study is to evaluate the clinical features and prognosis of adult-onset Still's disease (AOSD). One hundred and four AOSD patients who were analyzed retrospectively were enrolled in this study. Medical charts were systematically reviewed for: demographic data, clinical features, laboratory findings, treatments, and outcomes. The major clinical features were: spiking fever 100%, evanescent maculopapular rash 95%, polyarthralgia 90%, sore throat 78%, lymphadenopathy 66%, hepatosplenomegaly 57%, hydrohymenitis 30%, neutrophilia 98%, liver disfunction 62%, increased erythrocyte sedimentation rate (ESR) 96%, and hyperferritinaemia 99%. Reactive hyperplasia was shown in all patients who underwent lymph node biopsy. Ninety-five percent and 63% of the patients were treated with glucocorticoid and immune suppressant, respectively. Those with prednisone or its equivalent dosage of > or =0.8 mg/kg/d achieved quicker remission and less relapse. Persistent fever, evanescent rash, arthritis, and sore throat were the most prevalent symptoms in patients with AOSD, with laboratory findings of leukocytosis, elevated liver enzymes, elevated ESR and serum ferritin. Glucocorticoid and immune suppressive drugs are effective for AOSD; however, the relapsing rate is relatively high. High levels of white blood cells, serum ferritin and ESR, as well as glucocorticoid dosage were related to relapse.

Adult-Onset Still's Disease and Cardiac Tamponade: A Rare Association

“…12,13,17 Adultonset Still's disease has a good prognosis, with low mortality rates despite a recurrence rate of 10% per year during followup. 4,5 Our report on this patient's case provides good evi dence that AOSD should be considered in the differen tial diagnosis of patients who present with pericarditis, with or without pericardial effusion. It also underscores the need to perform serial echocardiographic evalu ations in patients with this condition, to enable the timely diagnosis and management of cardiac sequelae that can, albeit rarely, include tamponade.…”

The cause of fever and pulmonary infiltrate: a difficult etiological diagnosis

“…Finally, even if Yamaguchi's criteria are not present, some authors report that high levels of serum ferritin are useful for indicating disease activity and abnormally high serum ferritin concentrations are present in over 90% of cases. 6 This protein assay is present in Fautrel's more recent criteria for diagnosing adult onset Still's disease (Table 2) Five or more criteria are required, of which two or more must be major. …”

Adult-onset Still’s disease

“…В целом ритуксимаб можно считать альтер-нативным препаратом при рефрак-терных случаях БСРВ [98]. Клиническая картина БСРВ за прошедшее столетие не измени-лась, недавно проведенный ретро-спективный анализ 104 больных показал, что основными симптома-ми остаются: лихорадка (100%), быстроисчезающая макулопапулез-ная сыпь (95%), полиартралгия (90%), боли в горле (78%), лимфо-аденопатия (66%), гепатоспленоме-галия (57%), нейтрофилез (98%), [99]. Уста-новлено также, что у больных, не отвечающих на ГК, в начале болезни повышен риск развития хронического артрита (p<0,001).…”