| Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. It is characterized by fever, skin rash, polyarthralgias or polyarthritis, sore throat, hepatosplenomegaly, lymphadenopathy,leukocytosis, liver enzyme elevation, and high serum level of ferritin. The age at disease onset ranged from 14 to 83 years with a mean of 29. 85 years, and the vast majority of our patients (96%) had disease onset between the ages of 16 and 35 years. It's generally believed that AOSD happends with the factors of infection, genetic and immunological abnormalities. Importantly, the disease course was benign, probably as an outcome of heightened awareness and less diagnostic delay than in the past. Still's disease, although uncommon, has characteristic constellation of clinical and laboratory features and should be considered in the differential diagnosis of fever of unknown origin. To investigate the clinical features, diagnosis, treatment and outcome of patients with adult onset Still's disease (AOSD), we analyzed the clinical data of 48 patients with AOSD during the period 2005.1-2009.1. Methods: Clinical data of 48 patients with AOSD were analyzed. Results: Fever(100%), arthritis(89.6%) and rash(88.2%) were the most common symptoms seen in AOSD. All the 48 cases of patients were fever, and 40 (83.3%) of these patients'temperature were above 39℃. 43 patients(89.6%) had arthritis, the joints most frequently affected are the knees(79.6%), wrists(79.6%), ankles(58.3%), elbow (45.8%), shoulder(41.6%), proximal interphalangeal joint(39.4%), metacarpophalangeal joint(33.3%) and hip(15.4%), metatarso- phalangeal joints, distal interphalangeal joint and chest clavicular joint, such as were less involved. 42 patients(88.2%) had rashes, the classic rash is an evanescent, salmon-pink, maculopapular eruption, which frequently appears during febrile attacks and is predominantly found on the proximal limbs and trunk. The rash can be mildly pruritic or may be associated with burning. 23 cases(47.9%) of these patients with AOSD had the appearance of lymphadenopathy, the neck lymphadenopathy in 20 cases(41.7%), inguinal lymphadenopathy in 7 cases (14.6%), axillary lymphadenopathy in 4 (8.3%);lymphadenopathy around aortic arch in 1 case(2.1%), lymphadenopathy around porta lienis in 1 case(2.1%). Splenomegaly in 25 cases(52.1%), all of them were mildly. Hepatomegaly in 8 cases(16.7%). 41 cases (85.4%) were accompanied by pharyngodynia, 37 cases(77.1%) were accompanied by myalgia. In addition, serositis and serous effusions in 10 cases(20.8%), onset Still's disease associated with peripheral neuritis in 1case(2.1%), associated with brain damage in 1 case (2.1%). The results of selected laboratory tests were as follows: leukocytosis in 43 cases(89.6%), neutrophilia in 45 cases(93.8%) patients, anemia with Hb<11g/dL in 19 cases(39.6%), thrombocytosis with elevated platelets >300×109/L in 34cases(71.3%), An elevated erythrocyte sedimentation rate (ESR) in 48 cases(100%), C-reactive protein (CRP) in 33 cases(33/36,91.7%), increased AST in 30 cases(62.5%), increased ALT in 28 cases(58.3%), increased ALP in 20 cases(41.7%), increased GGT in 27 cases(58.3%), increased lactic dehydrogenase in 43 cases(89.6%), and decreased ALB in 35 cases(72.9%). Elevated serum ferritin levels in 44 cases(91.65%) and ferritin levels×5 normal in 3 cases(6.25%). 48 cases of patients with AOSD, only three cases (6.25%) simple application of non-steroidal anti-inflammatory drugs can control the disease, and the rest will require the application of glucocorticoids, including 36 (75%) patients with glucocorticoid alone can control the disease, seven cases of (12.5%) in the application of glucocorticoid required to increase the basis of one kinds of immunosuppressive agents used to control the disease. There are one cases of glucocorticoid in the application based on the need to add two kinds of immunosuppressive agents used to control the disease. For the application of glucocorticoids, the glucocorticoid dose in accordance with sub-study found that patients with the highest temperature in the course of disease, the high-dose groups were significantly different (P <0.05), and to 39.5℃for the cut-off point, in two distribution between groups were significantly different (P <0.05), CRP to 55mg/L for the critical point in the distribution between the two groups were significantly different (P <0.05), WBC to 22×109/ L for critical point in the distribution between the two groups were significantly different (P <0.05). AOSD sensitive response to treatment: WBC and ESR in the treatment of 1 week after the marked decline, CRP in the treatment began to decline after two weeks. PLT in the treatment continues to rise after 1 week, 2 weeks after the beginning of a gradual slow decline. AOSD is a relatively benign disease if diagnosed early and treated appropriately. 48 cases of AOSD patients, one patient died of septic shock, 6 cases (13%) recurrent and re-admitted to hospital, while the others all sustained remission. Conclusion: 1. Fever, arthritis and rash were the most common symptoms seen in AOSD. Other common symptoms were in accordance with the incidence of pharyngodynia, myalgia, lymphadenopathy, splenomegaly, hepatomegaly. Serositis and serous effusions can also occur, sometimes involving the nervous system. 2. AOSD laboratory indicators change in a wide range, accompanied by indicators such as acute reaction response, SF and enzymes also increased, in which LDH increased significantly than the other. 3. AOSD has sensitive response to treatment: WBC and ESR significantly decreased in the treatment of the firsr week, CRP begin to decline in the treatment of the first two weeks. PLT continue to rise in the treatment of the first weeks, when the treatment of the first two weeks PLT began to decline slowly. 4. special types AOSD have complex and diverse clinical manifestations, the disease taking control need the use of high doses of hormones or at least one kinds of combined immunosuppressants.
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