Farrington M L, Haas J E, Nazar-Stewart V, Mellins E D
Department of Pediatrics, University of Washington, Seattle 98195.
J Rheumatol. 1993 Jan;20(1):128-32.
We evaluated longterm outcome in 21 pediatric patients with biopsy proven eosinophilic fasciitis (EF), 4 followed in our rheumatology clinic and 17 reported in the literature. Two-thirds of these patients developed residual cutaneous fibrosis, while one-third enjoyed complete resolution of disease. Children under age 7 years were twice as likely as those over age 7 years to experience disease progression to cutaneous fibrosis [relative risk = 2.0 (95% confidence intervals 1.2, 3.4)]. Fourteen of 17 patients with extensive disease at diagnosis (involvement of 3-4 extremities +/- trunk) progressed to cutaneous fibrosis whereas all 4 patients with minimal disease (involvement of 1-2 extremities) at onset resolved completely. We detected no association between progression to cutaneous fibrosis and sex of patient, duration of symptoms prior to therapy, type of therapy, history of prior physical stress, or laboratory variables at diagnosis.
我们评估了21例经活检证实为嗜酸性筋膜炎（EF）的儿科患者的长期预后，其中4例在我们的风湿病诊所接受随访，17例在文献中报道。这些患者中有三分之二出现了残留的皮肤纤维化，而三分之一的患者疾病完全缓解。7岁以下儿童疾病进展为皮肤纤维化的可能性是7岁以上儿童的两倍[相对风险=2.0（95%置信区间1.2, 3.4）]。诊断时患有广泛性疾病（累及3 - 4个肢体+/-躯干）的17例患者中有14例进展为皮肤纤维化，而发病时患有轻微疾病（累及1 - 2个肢体）的所有4例患者均完全缓解。我们未发现进展为皮肤纤维化与患者性别、治疗前症状持续时间、治疗类型、既往身体应激史或诊断时的实验室变量之间存在关联。
