MEDLINE/PubMed Journal Browser Search

Pubmed for Handhelds

PUBMED FOR HANDHELDS

Search MEDLINE/PubMed

Title: [Clinical characteristics, ultrasonic diagnosis, treatment and outcomes of eosinophilic fasciitis: a retrospective single-center analysis of 45 cases].
Author: Yang J, Wan Y, Zhang J, Zhu J.
Journal: Nan Fang Yi Ke Da Xue Xue Bao; 2023 Jan 20; 43(1):145-152. PubMed ID: 36856224.
Abstract:
OBJECTIVE: To evaluate the clinical features, laboratory and imaging results, treatment and outcomes of eosinophilic fasciitis (EF) and assess the value of ultrasound in the diagnosis of EF. METHODS: We retrospectively analyzed the clinical data of 45 patients with EF treated in our center from January 1, 2006 to February 28, 2022. The consistency between the diagnoses of EF based on ultrasound and MRI findings was assessed. RESULTS: In the 45 EF patients (male/female ratio 3.5:1), the age of onset ranged from 16 to 64 years with a mean disease course of 22.6 months. The average time from symptom onset to diagnosis was 16 months. The most common possible trigger of the disease was vigorous exercise (10/45), causing symmetrical lesions in the limbs, most commonly in the forearms (86.7%) and lower legs (80%). Clinical features of EF included subcutaneous swelling and induration (95.6%), arthralgia and arthritis (55.6%), groove sign (42.2%), hand joint contractures (42.2%), skin pigmentation (37.8%), and peau d'orange appearance (13.3%). Eosinophilia was found in 31 patients (68.9%). Hypergammaglobulinemia was seen in 23/44 (52.3%) and positive antinuclear antibodies in 9 (20%) of the patients. Twentyone of the patients were treated with high-dose methylprednisolone (≥200 mg daily for 3 to 5 consecutive days), and compared with the patients who did not receive this treatment, these patients more frequently experienced relapse before admission, had more extensive involvement, and had a higher rate of hypergammaglobulinemia without fever, but these differences were not statistically significant. Of the 31 patients (68.9%) with follow-up data (for a median of 3.2 years [range 0.2-15.9]), complete remission was achieved in 12 (38.7%) patients, and the accumulative complete remission rate was 44.1% at 5.5 years. No specific baseline characteristics or immunosuppressants were found to correlate with the treatment response. A total of 26 patients underwent both ultrasound and MRI examination, and the Kappa value of the diagnostic results between ultrasound and MRI was 0.91. CONCLUSION: EF is characterized by symmetrical subcutaneous swelling and induration in the limbs, accompanied by eosinophilia and hypergammaglobulinemia. Glucocorticoid is effective for treating EF. Ultrasound examination can identify thickening of subcutaneous fascia for an early diagnosis of EF. 目的: 回顾嗜酸性筋膜炎（EF）的临床症状、化验、检查、治疗及预后，并探讨超声在EF中的应用价值。方法: 回顾2006年1月1日~2022年2月28日在我中心就诊的EF患者的临床资料，并进行统计描述、分析，评估超声及磁共振成像（MRI）结果间的一致性。结果: 45例EF患者，男女比例3.5:1，发病年龄16~64岁，平均病程22.6个月，从出现症状到确诊的平均时间为16个月。最常见的诱因是剧烈运动诱发，占22%（10/45例）。EF以对称性四肢受累为主，前臂（86.7%）及小腿（80%）最常见。临床表现包括：皮下肿胀/紧硬（95.6%）、关节痛/炎（55.6%）、沟征（42.2%）、手关节屈曲挛缩（42.2%）、色素沉着（37.8%）、橘皮样皮肤病变（13.3%）等。31例（68.9%）患者嗜酸性粒细胞升高，52.3%（23/44例）的患者IgG升高，9例（20%）抗核抗体阳性。21例患者接受了≥200 mg/d（3~5 d）的大剂量激素治疗，与未接受大剂量激素治疗的患者相比，虽未达到统计学差异，但患者入院前曾复发的比例更高、受累部位更广、IgG升高的比例更多，且没有发热。共有31例（68.9%）患者完成了随访，12/31例（38.7%）患者完全缓解，随访的中位时间为3.2年（0.2~15.9年），5.5年时完全缓解的累积发生率为44.1%。未发现与治疗反应相关的特殊基线特征及免疫抑制剂种类。共26例患者同时进行了超声及MRI检查，超声与MRI在确定筋膜增厚/炎症方面一致性很好（Kappa=0.91）。结论: EF的临床特征为对称性四肢皮下肿胀、紧硬，伴嗜酸性粒细胞、IgG升高，激素治疗效果好。超声识别皮下筋膜增厚是诊断EF的一个早期最有效的工具。

[Abstract] [Full Text] [Related] [New Search]