Marie I, Hachulla E, Hatron P Y, Hellot M F, Levesque H, Devulder B, Courtois H
Department of Internal Medicine, Centre Hospitalier Universitaire de Rouen-Boisguillaume, Rouen, France.
J Rheumatol. 2001 Oct;28(10):2230-7.
To assess short term and longterm outcome of polymyositis (PM) and dermatomyositis (DM), and predictive variables of PM/DM course.
The medical records of 77 consecutive patients with PM/DM were reviewed. The criteria for PM/DM diagnosis were based upon Bohan and Peter criteria.
Thirty-one patients (40%) achieved remission of PM/DM, whereas 33 (43%) improved and 13 (17%) worsened their clinical status. Short term recurrences of PM/DM (during tapering of therapy) occurred in 36 patients and longterm recurrences (after discontinuation of therapy) in 9 patients. PM/DM were associated with both decreased functional status and quality of life at longterm followup: (1) only 52% of patients considered to be in remission experienced a return to previous normal activities; and (2) 45% of the other patients with nonremitting PM/DM still had a marked reduction of activities (as shown by the disability scale of the Health Assessment Questionnaire). Overall mortality was as high as 22%, and the main causes of death were cancer and lung complications. Factors associated with PM/DM remission were younger age and shorter duration of clinical manifestations prior to therapy initiation. Variables associated with poor outcome of PM/DM were older age, pulmonary and esophageal involvement, and cancer.
Our series shows both high morbidity and mortality related to PM/DM, emphasizing that management of PM/DM patients at an early stage is required. Lung complications (i.e., aspiration pneumonia due to PM/DM related esophageal dysfunction and ventilatory insufficiency) were one of the main causes of death in our series, indicating that investigating for subclinical esophageal and lung impairment should become an integral part of initial PM/DM evaluation. The presence of poor prognostic factors should prompt both close followup and aggressive therapy in patients with PM/DM.
评估多发性肌炎（PM）和皮肌炎（DM）的短期和长期预后，以及PM/DM病程的预测变量。
回顾了77例连续的PM/DM患者的病历。PM/DM的诊断标准基于博汉和彼得标准。
31例患者（40%）实现了PM/DM缓解，而33例（43%）病情改善，13例（17%）临床状态恶化。36例患者出现PM/DM短期复发（在治疗减量期间），9例出现长期复发（在治疗中断后）。在长期随访中，PM/DM与功能状态下降和生活质量下降均相关：（1）仅52%被认为处于缓解期的患者恢复到了之前的正常活动；（2）其他未缓解的PM/DM患者中有45%的活动仍有显著减少（如健康评估问卷的残疾量表所示）。总死亡率高达22%，主要死亡原因是癌症和肺部并发症。与PM/DM缓解相关的因素是年龄较小和治疗开始前临床表现的持续时间较短。与PM/DM预后不良相关的变量是年龄较大、肺部和食管受累以及癌症。
我们的系列研究表明PM/DM的发病率和死亡率均较高，强调需要对PM/DM患者进行早期管理。肺部并发症（即由于PM/DM相关食管功能障碍和通气不足导致的吸入性肺炎）是我们系列研究中的主要死亡原因之一，表明对亚临床食管和肺部损害进行检查应成为初始PM/DM评估的一个组成部分。存在不良预后因素应促使对PM/DM患者进行密切随访和积极治疗。
