Pouchot J, Sampalis J S, Beaudet F, Carette S, Décary F, Salusinsky-Sternbach M, Hill R O, Gutkowski A, Harth M, Myhal D
Service de Médecine Interne, Hôpital Louis Mourier, Colombes, France.
Medicine (Baltimore). 1991 Mar;70(2):118-36.
Clinical and laboratory manifestations, disease course, outcome, and HLA associations were studied in an inception cohort of 62 subjects with adult Still's disease (ASD) from 5 Canadian universities. Twenty-eight patients (45%) were female and the median age at disease onset was 24 years. In general, the clinical features observed in our patients were identical to those in other published series. However, significantly higher frequencies of sore throat (92%), weight loss (76%), lymphadenopathy (74%), pleuritis (53%), pneumonitis (27%), and abdominal pain (48%) were noted in our patients compared to those in a recent literature review. Liver involvement with hepatomegaly (44%) or abnormal liver function tests (LFTs) (76%) was common and was responsible for the 2 deaths attributed to Still's disease in our series. Severe liver failure always occurred in conjunction with aspirin or NSAID therapy. Therefore, whether or not aspirin or other NSAIDs are used, we recommend close monitoring of LFTs in patients with ASD, especially early in the disease course. Laboratory manifestations were similar to those already reported. Leukocytosis (greater than or equal to 15,000/mm3) was present in 50 patients (81%), a normochromic, normocytic anemia (hemoglobin less than or equal to 10 g/dl) in 42 (68%), and an elevated ESR in all. The mean follow-up of the 62 patients was 70 months (range, 2-163). Twenty-one patients (34%) had a self-limited disease course, 15 (24%) an intermittent course, and 22 (36%) a chronic disease course. Four patients (6%) died, and 2 of these deaths were attributed to Still's disease. For those patients who experienced a recurrence of ASD, the flares were usually of shorter duration and milder in severity than the initial episode. No initiating factor for disease exacerbation was identified in our patients. Although 22 of 62 patients (36%) had a chronic disease course, 52 (90%) were in ARA Functional Class I, and only 4 and 2 patients were in ARA Functional Class II and III, respectively. Patients with Still's disease had higher scores than the controls on the Pain (P less than 0.01) and Physical Disability (P less than 0.05) subscales of Arthritis Impact Measurement Scales health status questionnaire. Joint radiographs performed at the follow-up evaluation disclosed typical carpometacarpal and intercarpal involvement in 16 of 39 patients. In our series, HLA-B17, B18, B35, and DR2 were significantly associated with ASD. Three significant predictors of an unfavorable outcome, either a chronic disease course or a longer time to clinical remission, were identified.(ABSTRACT TRUNCATED AT 400 WORDS)
对来自加拿大5所大学的62例成人斯蒂尔病（ASD）患者的起始队列进行了临床和实验室表现、病程、结局及HLA相关性研究。28例患者（45%）为女性，发病时的中位年龄为24岁。总体而言，我们患者中观察到的临床特征与其他已发表系列中的特征相同。然而，与最近一篇文献综述中的患者相比，我们的患者中咽痛（92%）、体重减轻（76%）、淋巴结病（74%）、胸膜炎（53%）、肺炎（27%）和腹痛（48%）的发生率显著更高。肝脏受累伴肝肿大（44%）或肝功能检查异常（LFTs）（76%）很常见，且是我们系列中2例斯蒂尔病相关死亡的原因。严重肝衰竭总是与阿司匹林或非甾体抗炎药治疗同时发生。因此，无论是否使用阿司匹林或其他非甾体抗炎药，我们建议对ASD患者密切监测LFTs，尤其是在病程早期。实验室表现与已报道的相似。50例患者（81%）存在白细胞增多（≥15,000/mm³），42例（68%）存在正细胞正色素性贫血（血红蛋白≤10 g/dl），所有患者的血沉均升高。62例患者的平均随访时间为70个月（范围2 - 163个月）。21例患者（34%）病程自限，15例（24%）病程间歇，22例（36%）病程慢性。4例患者（6%）死亡，其中2例死亡归因于斯蒂尔病。对于那些经历ASD复发的患者，病情发作通常比初始发作持续时间短且严重程度轻。我们的患者中未发现疾病加重的起始因素。虽然62例患者中有22例（36%）病程慢性，但52例（90%）处于美国风湿病学会（ARA）功能I级，分别只有4例和2例患者处于ARA功能II级和III级。斯蒂尔病患者在关节炎影响测量量表健康状况问卷的疼痛（P < 0.01）和身体残疾（P < 0.05）子量表上的得分高于对照组。随访评估时进行的关节X线片显示，39例患者中有16例存在典型的掌指关节和腕骨间关节受累。在我们的系列中，HLA - B17、B18、B35和DR2与ASD显著相关。确定了三个不良结局的显著预测因素，即慢性病程或临床缓解时间较长。（摘要截短至400字）
