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嗜酸性粒细胞增多性肌痛综合征:205例患者发病2年后的状况及治疗结果

The eosinophilia-myalgia syndrome: status of 205 patients and results of treatment 2 years after onset.

作者信息

Hertzman P A, Clauw D J, Kaufman L D, Varga J, Silver R M, Thacker H L, Mease P, Espinoza L R, Pincus T

机构信息

Los Alamos Medical Center, New Mexico, USA.

出版信息

Ann Intern Med. 1995 Jun 1;122(11):851-5. doi: 10.7326/0003-4819-122-11-199506010-00008.

Abstract

OBJECTIVE

To describe the course of the eosinophilia-myalgia syndrome during a 2-year period.

DESIGN

15 physicians completed a structured review form to describe symptoms, physical findings, laboratory data, and responses to treatments in 205 patients with the eosinophilia-myalgia syndrome at the onset of illness and after 18 to 24 months of follow-up.

SETTING

15 university and private clinical practice settings.

PATIENTS

205 patients for whom follow-up data were available and who met four criteria at diagnosis: eosinophil count of 1000 cells/mm3 or greater; presence of fasciitis, peripheral neuropathy, polyradiculopathy, interstitial pulmonary disease, pulmonary hypertension, or myocardial involvement; history of L-tryptophan consumption; and absence of other conditions that could account for these findings.

INTERVENTION

Empirical interventions by the physicians.

MEASUREMENTS

Symptoms, physical findings, laboratory test results, biopsy findings, radiographic reports, therapeutic interventions, and responses to these interventions.

RESULTS

After 18 to 24 months, all symptoms except cognitive changes were reported to have improved in most patients. Nearly all physical findings were also reported to have improved or resolved in most patients; only peripheral neuropathy was unchanged. No evidence of ongoing inflammatory disease was reported. Prednisone was reported to be helpful in 79% of patients who received it during the acute phase of the syndrome. No other treatment was reported to be consistently beneficial.

CONCLUSIONS

18 to 24 months after the onset of illness, most symptoms and physical findings in most patients with the eosinophilia-myalgia syndrome resolved or improved. Cognitive changes were reported to be worse in 32% of patients. Prednisone was helpful in the acute phase of illness. No treatment was clearly valuable in management of the later phase of the syndrome.

摘要

目的

描述嗜酸性粒细胞增多性肌痛综合征在两年期间的病程。

设计

15名医生填写了一份结构化回顾表,以描述205例嗜酸性粒细胞增多性肌痛综合征患者在疾病发作时以及随访18至24个月后的症状、体格检查结果、实验室数据及治疗反应。

地点

15所大学及私人临床实践机构。

患者

205例有随访数据且在诊断时符合以下四项标准的患者:嗜酸性粒细胞计数为每立方毫米1000个细胞或更高;存在筋膜炎、周围神经病变、多神经根病、间质性肺病、肺动脉高压或心肌受累;有服用L-色氨酸的病史;且不存在可解释这些发现的其他病症。

干预措施

医生的经验性干预。

测量指标

症状、体格检查结果、实验室检查结果、活检结果、影像学报告、治疗性干预措施以及对这些干预措施的反应。

结果

18至24个月后,多数患者报告除认知改变外的所有症状均有改善。多数患者的几乎所有体格检查结果也报告有改善或已消失;仅周围神经病变无变化。未报告有持续炎症性疾病的证据。据报告,泼尼松对在综合征急性期接受该药治疗的79%的患者有帮助。未报告其他治疗有持续的益处。

结论

疾病发作18至24个月后,多数嗜酸性粒细胞增多性肌痛综合征患者的多数症状和体格检查结果缓解或改善。据报告,32%的患者认知改变更严重。泼尼松在疾病急性期有帮助。在综合征后期的管理中,没有哪种治疗有明确的价值。

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