Clinical Analysis Of42Cases With Adult-onset Still’s Disease

Objective:In order to have a better understanding of the etiology and clinical features of adult-onset Still’s disease (AOSD), and provide experience for clinical diagnosis and treatment to improve the prognosis, we reviewed the medical records of patients with AOSD diagnosed in our hospital. Some clinical and laboratory parameters were collected and analysed.Methods:A total of42patients diagnosed with AOSD, hospitalized in Sir Run Run Shaw Hospital of Zhejiang University from January2004to January2015were retrospectively evaluated. All of the patients fulfilled the Yamaguchi criteria. The disease course and treatment were recorded and compared to previously published studies. Data were collected from age of onset, clinical manifestation, laboratory workup, treatment and prognosis. Statistical analysis were performed to determine the correlation of clinical manifestation, laboratory results and the dosage of corticosteroid. And the serum ferritin of the patients in different conditions was analysed. Results:In our patients, the average age of onset was41.76±15.69years old. It was more common in women. Common clinical manifestations were fever (100%), rash (83.3%), sore throat (78.6%), arthralgia (73.8%), myalgia (50%), lymphadenopathy (50%) and splenomegaly (23.8%). Laboratory tests mainly showed the elevation of white blood cells (83.3%), serum ferritin (88.1%), ESR (92.9%), CRP (100%), GGT (49.3%), LDH (66.7%), ALP (100%), AST and (or) ALT (64.3%). Anemia was found in about45.2%of the patients and albumin decreased in87.5%. The negative rates of Antinuclear antibodies and RF were100%and92.9%. NSAIDS, corticosteroids, and DMARDs were effective for the patients. Most patients had good prognosis while a small number of patients, who were all females at the age of30to50years old, developed a chronic arthralgia. White blood cell count more than14*10^9/L was the risk factor for relapse(OR=6.231, P<0.05). White blood cell count, neutrophil count, LDH and superficial lymphadenopathy were correlated with the dose of corticosteroid (P<0.05). Patients were divided into3groups:onset, partial remission (PR) and complete remission (CR). Serum ferritin between onset and PR was not statistically significant (P>0.05), while between onset and CR, PR and CR were both statistically significant (P<0.05). The multiple regression analysis showed patients with high elevation of neutrophil count tended to need higher dose of corticosteroid.Conclusion:1) AOSD is more common in middle-aged females.2) Patients with significantly elevated white blood cell count, neutrophil count, LDH or with superficial lymphadenopathy need higher dose of corticosteroid.3) Patients with significantly elevated white blood cell count have the higher risk for relapse. 4) Serum ferritin is hysteretic as an evaluating factor for AOSD.5) Most patients have good prognosis, while females at the age of30to50years old may develop chronic arthralgia.