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嗜酸性筋膜炎:临床特征及对甲氨蝶呤的反应

Eosinophilic fasciitis: clinical characteristics and response to methotrexate.

作者信息

Berianu Florentina, Cohen Marc D, Abril Andy, Ginsburg William W

机构信息

Department of Rheumatology, Mayo Clinic, Jacksonville, Florida, USA.

出版信息

Int J Rheum Dis. 2015 Jan;18(1):91-8. doi: 10.1111/1756-185X.12499. Epub 2014 Dec 20.

Abstract

AIM

To describe our experience with 16 patients with eosinophilic fasciitis (EF) treated in our clinic over 14 years.

METHODS

We retrospectively reviewed the charts of all patients with biopsy-proven EF. We collected data regarding demographics, clinical presentations, possible triggers, labs, imaging, treatment and response to therapy on follow-up.

RESULTS

Eight women and eight men with a mean age of 52 years were included in the study. Three patients related the onset to prior strenuous exercise and one was exposed to vibratory machinery. Fourteen patients had a gradual onset and presented with induration of the skin. Two other patients presented with acute-onset and significant edema and weight gain. All patients required immunosuppressive therapy. Methotrexate (MTX) was used in all of our patients. The rate of complete remission was ~60%. Although the recurrence rate after stopping MTX was 70%, these patients responded well to re-treatment with MTX.

CONCLUSION

We believe that MTX represents an effective treatment option for EF. The rarity of this disease would make a double-blind controlled trial study difficult to perform.

摘要

目的

描述我们诊所14年来治疗的16例嗜酸性筋膜炎(EF)患者的经验。

方法

我们回顾性分析了所有经活检证实为EF患者的病历。我们收集了有关人口统计学、临床表现、可能的诱发因素、实验室检查、影像学检查、治疗及随访时治疗反应的数据。

结果

研究纳入了8名女性和8名男性,平均年龄52岁。3例患者将发病与之前的剧烈运动相关联,1例接触过振动机械。14例患者起病缓慢,表现为皮肤硬结。另外2例患者急性起病,伴有明显水肿和体重增加。所有患者均需要免疫抑制治疗。我们所有患者均使用了甲氨蝶呤(MTX)。完全缓解率约为60%。虽然停用MTX后的复发率为70%,但这些患者再次使用MTX治疗反应良好。

结论

我们认为MTX是治疗EF的一种有效选择。这种疾病的罕见性使得进行双盲对照试验研究很困难。

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