Department of Dermatology, Radboud University, Nijmegen, The Netherlands; Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands; Laboratory of Translational Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
Department of Rheumatology, Radboud University, Nijmegen, The Netherlands.
J Am Acad Dermatol. 2017 Sep;77(3):512-517.e5. doi: 10.1016/j.jaad.2017.05.018. Epub 2017 Jul 19.
Eosinophilic fasciitis (EF) is a connective tissue disease with an unknown long-term course.
To evaluate presence and determinants of residual disease damage in patients with EF after long-term follow-up.
Patients with biopsy-proven EF were included for this cross-sectional study. Outcome measures included the Physician's Global Assessment of Disease Activity, Physician's Global Assessment of Damage (PhysGA-D), skin pliability scores, passive range of motion, and health-related quality of Life (HRQoL) questionnaires.
In total, 35 patients (24 of whom were female [68.6%]) with a median age of 60 years participated. All patients had detectable residual damage. Impairment of HRQoL, assessed by the Dermatology Quality of Life Index and the 36-Item Short-Form Survey, correlated to the extent of residual damage. The PhysGA-D score at participation correlated to signs of severe disease at presentation, such as increased C-reactive protein level (Spearman's rho [r ] = 0.486, P = .006), involvement of the neck (r = 0.528, P = .001) and trunk (r = 0.483, P = .003), prolonged time to disease remission (r = 0.575, P = .003), and presence of concomitant morphea (r = 0.349, P = .040). Lastly, maximum methotrexate dose correlated negatively to PhysGA-D score at study participation (r = -0.393, P = .022).
Sample size.
All patients with EF had detectable residual damage. Impairment of HRQoL correlated to the extent of residual damage. Advanced age and signs of severe disease at presentation were associated with the severity of residual damage.
嗜酸性筋膜炎（EF）是一种结缔组织疾病，其长期病程尚不清楚。
评估长期随访后 EF 患者的疾病残留损害的存在和决定因素。
本横断面研究纳入了经活检证实的 EF 患者。结局指标包括医生整体疾病活动度评估、医生整体损害评估（PhysGA-D）、皮肤柔韧性评分、被动关节活动度和健康相关生活质量（HRQoL）问卷。
共纳入 35 例患者（24 例女性[68.6%]），中位年龄为 60 岁。所有患者均存在可检测到的残留损害。通过皮肤病生活质量指数和 36 项简短健康调查评估的 HRQoL 受损与残留损害的程度相关。参与研究时的 PhysGA-D 评分与疾病发作时的严重程度相关，如 C 反应蛋白水平升高（Spearman's rho[r] = 0.486，P =.006）、颈部受累（r = 0.528，P =.001）和躯干受累（r = 0.483，P =.003）、疾病缓解时间延长（r = 0.575，P =.003）和同时存在硬皮病（r = 0.349，P =.040）。最后，最大甲氨蝶呤剂量与研究参与时的 PhysGA-D 评分呈负相关（r = -0.393，P =.022）。
样本量。
所有 EF 患者均存在可检测到的残留损害。HRQoL 受损与残留损害的程度相关。发病时年龄较大和严重程度的疾病表现与残留损害的严重程度相关。
