Nashel Jennifer, Steen Virginia
Beth Israel Deaconess Hospital, Boston, MA, USA.
Clin Rheumatol. 2015 Aug;34(8):1481-4. doi: 10.1007/s10067-014-2777-x. Epub 2014 Sep 24.
Eosinophilic fasciitis (EF) is a rare localized fibrosing disorder of the fascia whose diagnosis is often suspected based on clinical findings and laboratory values. These lab abnormalities can be transient in early disease and may not always be present. We have reviewed a case series of patients to assess the utility of the various laboratory abnormalities in diagnosing EF. We performed a retrospective review of EF patients seen at Georgetown University Hospital in the Division of Rheumatology during 2009 and 2013. This review included 15 adult patients with EF with a mean age at diagnosis of 45 years (range 18 to 77 years). The majority of patients 13/15 had classic skin thickening documented on all four extremities Only eight patients had peripheral eosinophilia ranging between 8 and 38 %. In these patients, the peripheral eosinophilia was an early but transient finding. Inflammatory markers including the erythrocyte sedimentation rate (ESR) was elevated in 5/14 and C-reactive Protein (CRP) was elevated in 7/11. At disease presentation, only one of eleven patients checked had an elevated creatine phosphokinase (CPK). Aldolase levels were available for 12 of the 15 patients, and they were increased in 11 out of 12 patients. We have found that in this case series, aldolase was more likely to be abnormal than peripheral eosinophilia, hypergammaglobulinemia, and ESR particularly after starting treatment. Aldolase should be measured in all patients suspected of having EF, and may also play a useful role in following disease activity.
嗜酸性筋膜炎(EF)是一种罕见的局限性筋膜纤维化疾病,其诊断通常基于临床症状和实验室检查结果来怀疑。这些实验室异常在疾病早期可能是暂时的,并不总是存在。我们回顾了一系列病例,以评估各种实验室异常在诊断EF中的作用。我们对2009年至2013年期间在乔治敦大学医院风湿病科就诊的EF患者进行了回顾性研究。该研究包括15例成年EF患者,诊断时的平均年龄为45岁(范围为18至77岁)。大多数患者(13/15)四肢均有典型的皮肤增厚记录。只有8例患者外周血嗜酸性粒细胞增多,范围在8%至38%之间。在这些患者中,外周血嗜酸性粒细胞增多是一个早期但短暂的表现。炎症指标包括红细胞沉降率(ESR)在14例中有5例升高,C反应蛋白(CRP)在11例中有7例升高。在疾病初发时,11例接受检查的患者中只有1例肌酸磷酸激酶(CPK)升高。15例患者中有12例可检测醛缩酶水平,其中12例中有11例升高。我们发现在这个病例系列中,醛缩酶比外周血嗜酸性粒细胞增多、高球蛋白血症和ESR更易出现异常,尤其是在开始治疗后。所有疑似患有EF的患者均应检测醛缩酶,其在监测疾病活动方面也可能发挥有用的作用。