Department of Pediatric Rheumatology, Dr. Sami Ulus Gynecology Obstetrics and Child Health and Diseases Training and Research Hospital, 06080, Ankara, Turkey.
Department of Pediatric Rheumatology, Dokuz Eylül University Medical School, Izmir, Turkey.
Clin Rheumatol. 2023 Oct;42(10):2855-2860. doi: 10.1007/s10067-023-06677-7. Epub 2023 Jun 28.
Morphea, also known as localized scleroderma, is an immune-mediated disease and the most common form of scleroderma in children. It is a localized sclerosing disease of the skin, but can also involve such adjacent tissues as the fascia, muscle, bone, and underlying tissues. This multicenter study aimed to evaluate Turkish pediatric morphea patients, regarding demographics, treatments, and response to treatment.
The study was performed by the Pediatric Rheumatology Academy and included pediatric morphea patients from 6 Turkish pediatric rheumatology centers who were followed up for ≥6 months. Demographic, clinical, and laboratory findings and treatment modalities were analyzed. The patients were divided into 3 groups according to treatment response, as follows: group 1: topical treatment response, group 2: methotrexate response, and group 3: methotrexate resistance. Clinical findings were compared between the 3 groups.
The study included 76 patients, of which 53 (69.7%) were female. Mean age at diagnosis of morphea was 9.7 ± 4.3 years and mean duration of follow-up was 3.2 ± 2.9 years. Linear morphea was the most common form, accounting for 43.4% (n = 33) of the patients. Extracutaneous features were noted in 17 patients (22.4%) and anti-nuclear antibody positivity was noted in 32 (42.1%). In all, 14.4% of the patients received topical treatment only, whereas 86.6% received both topical and systemic treatment. The methotrexate response rate was 76.9% in the patients that received systemic immunosuppressive therapy. The overall relapse rate while under treatment was 19.7%.
In this study, most of the pediatric morphea patients responded well to methotrexate. Bilateral lesions were more common in the methotrexate-resistant group. Multiple involvement, and bilateral lesions, were more common in relapsed patients than in non-relapsed patients. Key points • Most of the pediatric morphea patients respond well to MTX. • Multiple involvement, and bilateral involvement, were more common in relapsed patients than in non-relapsed patients. • Presence of extracutaneous findings in patients increased relapse rate 5.7 times.
硬斑病，又称局限性硬皮病，是一种免疫介导的疾病，也是儿童中最常见的硬皮病类型。它是一种皮肤局限性硬化性疾病，但也可累及筋膜、肌肉、骨骼和皮下组织等相邻组织。本多中心研究旨在评估土耳其儿科硬斑病患者的人口统计学、治疗方法和治疗反应。
该研究由儿科风湿病学会进行，纳入了来自土耳其 6 个儿科风湿病中心的至少随访 6 个月的儿科硬斑病患者。分析了患者的人口统计学、临床和实验室检查以及治疗方式。根据治疗反应将患者分为 3 组，如下：组 1：局部治疗反应；组 2：甲氨蝶呤反应；组 3：甲氨蝶呤耐药。比较了 3 组患者的临床发现。
该研究纳入了 76 例患者，其中 53 例（69.7%）为女性。硬斑病的平均诊断年龄为 9.7±4.3 岁，平均随访时间为 3.2±2.9 年。线状硬斑病是最常见的类型，占 43.4%（n=33）。17 例（22.4%）存在皮肤外表现，32 例（42.1%）抗核抗体阳性。14.4%的患者仅接受局部治疗，而 86.6%的患者接受局部和全身治疗。接受全身免疫抑制治疗的患者中，甲氨蝶呤的反应率为 76.9%。治疗期间的总体复发率为 19.7%。
在这项研究中，大多数儿科硬斑病患者对甲氨蝶呤反应良好。甲氨蝶呤耐药组双侧病变更为常见。复发患者比未复发患者更常出现多部位受累和双侧病变。关键点：·大多数儿科硬斑病患者对 MTX 反应良好。·复发患者比未复发患者更常出现多部位受累和双侧病变。·存在皮肤外表现的患者复发率增加 5.7 倍。
