| Objective:Adult-onset Still's disease (AOSD) is a syndrome with unknown aetiology, and may display non-specific, various symptoms, which is mainly manifested by hyperpyrexia, transient erythema, arthritis/arthrilgia and leukocytosis. Until now, there is no yet single index or uniform diagnostic criteria for identification of AOSD. Atypical manifestations more often occur in patients and a great lot of examinations are necessary in order to exclude infectious, neoplastic and autoimmune diseases. Nevertheless, misdiagnosis and missed diagnosis can not be avoided. Several sets of classification criteria have been published already, and Yamaguchi's criteria is the most commonly used. Treatment of AOSD remains empirical. The optimal treatment is difficult to determine because of the absence of large prospective studies. The correlative literature about prognosis is limited. So our study evaluated the value of clinical manifestations and laboratory findings in diagnosis of AOSD and further compared the efficiency of the different diagnostic criteria. Furthermore, we describe related-AOSD therapies and clinical course of patients with AOSD and analyze the prognostic factors associated with unfavorable outcomes in these patients.Methods:70 AOSD patients admitted at the Department of Rheumatology in Zhongshan Hospital affiliated to Fudan University from January 2003 to December 2009 were enrolled. The diagnosis of AOSD in the current study was based on the following steps:①The initial clinical assessment of patients presenting with clinical features (the typical symptoms and signs) of AOSD.②A careful history, physical examination and laboratory assessment for differential diagnosis:alternative diagnosis (e.g., infections, malignant tumor and other autoimmune disorders) was fully considered by the attending physicians and eliminated based on an extensive panel of relevant analysis such as bone marrow, imaging exams, pathologic finding, and microbiological test results. Antinuclear antibody (ANA), anti-dsDNA antibody, and antineutrophil cytoplasmic antibody were routinely investigated to exclude other autoimmune diseases. In cases of disagreement, the final classification decision was made by a senior team physician, either rheumatologist or internist. The control group included 140 patients with definite non-AOSD diseases during the same period, whose diagnosis was established on the basis of clinical, pathologic, or microbiologic data. Specifically, this group included patients with infectious, neoplastic and autoimmune diseases. Medical information of all patients were collected via a standardized questionnaire retrospectively, comprising basic clinical or biologic items frequently observed in the course of AOSD or "confusable" diseases. The different AOSD diagnostic criteria, including Yamaguchi's criteria (1992), American Rheumatology Association (ARA) criteria (1987), Cush's criteria (1987) and Calabro's criteria (1986) were used to all patients. The assessment index include sensitivity, specificity, positive predictive value, negative predictive value, positive likelihood ratio and negative likelihood ratio. The treatment and disease development of AOSD patients were also followed-up at clinics or by phones.Results:70 patients with AOSD (26 men,44 women ages 36.1±14.5, range 18～72 years) were identified. Of the 140 patients classified as controls (75 men,65 women ages 46.9±18.5, range 14～89 years),69 were affected with infectious diseases,19 with neoplasms and 52 with systemic autoimmune diseases. The clinical features such as rash, arthralgia, sore throat, myalgia, lymphadenectasis, hepatomegaly, splenomegaly, leukocytosis, neutrophil≥80% and serum ferritin≥5-fold the upper normal value have higher specificity (68.57～98.57%) with 2.00～5.00 of the positive likelihood ratio. Spiking fever≥39℃, ANA and RF negative have higher sensitivity (84.38～94.29%). Any combination of two or three items has higher positive likelihood ratio. The specificity of ARA criteria (1987) is highest with 99.29% and the accuracy is 83.33%, the sensitivity of Yamaguchi criteria (1992) is highest with 78.57% and the accuracy is 87.14%.61 patients with AOSD were followed-up,83.6% exhibited monocyclic systemic pattern,6.6% experienced disease replase at least once,4.9% exhibited chronic articular course and 4.9% died. There is no significant difference in gender, age, clinical features or therapeutic variance between patients with favorable (monocyclic pattern) and unfavorable (polycyclic pattern, chronic articular course or death) outcome.Conclusion:There is no single index for identification of AOSD. Rash, arthralgia, sore throat, myalgia, lymphadenectasis, hepatomegaly, splenomegaly, leukocytosis, neutrophil≥80% and serum ferritin≥5-fold the upper normal value are help for the diagnosis of AOSD. Different combination of items will elevate the diagnostic degree of certainty. The diagnostic criteria which are commonly used have the higher specificity in the study. Our results indicate that massive patients with AOSD have favorable outcome and we do not detect significant predictors for poor outcome. |
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