Department of Rheumatology, Aristide Le Dantec Hospital, Dakar, Senegal.
Pediatr Rheumatol Online J. 2024 Feb 29;22(1):31. doi: 10.1186/s12969-024-00969-1.
Enthesitis/spondylitis-related arthritis (ERA) is a type of juvenile idiopathic arthritis (JIA) frequently associated with HLA-B27. In sub-Saharan Africa, HLA-B27-positive ERA hasn't been the subject of a specific study.
We aimed to describe the clinical features, disease activity, functional disability and treatment of HLA-B27-positive ERA at diagnosis in Senegal and compare the findings to other populations.
We conducted a retrospective study by reviewing the medical records of patients diagnosed with ERA with an age of symptom onset < 18 years according to the 2019 PRINTO provisional criteria for ERA from January 2012 to December 2022. We collected demographic, clinical, paraclinical and therapeutic data. Disease activity score was assessed by Ankylosing Spondylitis Disease Activity Score (ASDAS) and Bath Ankylosing Spondylitis Disease Activity Index (BASDAI). Functional disability was assessed using Bath Ankylosing Spondylitis Functional Index (BASFI).
A total of 31 patients with HLA-B27-positive ERA were included. Twenty of 31 (64.5%) were males. Twenty-seven (87%) were Fula (ethnicity). The median age at symptom onset and at diagnosis was 12 years and 19 years, respectively. Seven patients had a family history of Spondyloarthritis. Peripheral arthritis and enthesitis were the most common presenting features at disease onset. Peripheral arthritis was present in 29 (93.5%) and located in the lower limbs in 27/29 (93.1%) patients. Heel enthesitis was present in 26 (83.8%) patients. Axial involvement was present in 27 (87%) patients, dominated by low back pain and sacroiliac pain/ buttock pain in 24 (88.8%) and 22 (81.5%) patients, respectively. Seven (22.5%) patients had anterior uveitis. The ESR and CRP were elevated in 65.5% and 57.1% of cases, respectively. On imaging, sacroiliitis was found in 22 patients. The mean BASDAI was 5.5/10 (77.2% of patients had a high active disease; BASDAI ≥ 4/10). The mean ASDAS-ESR/CRP was 3.8. The mean BASFI was 5.4/10 (80% of patients had high functional disability; BASFI ≥ 4/10). Twenty-seven (87%) patients were treated with methotrexate and non-steroidal anti-inflammatory drugs. After 6 months of treatment, mean BASDAI was 3/10 and mean BASFI was 2.5/10.
In our study, HLA-B27-positive ERA was found in our Senegalese cohort mainly in adolescents of the Fula ethnic group. 22 (70.9%) patients developed ankylosing spondylitis at adulthood. The disease was very active at the time of diagnosis with significant functional disability. Treatment was mainly based on methotrexate and NAISDs.
附着点炎/脊柱炎相关关节炎(ERA)是一种常见于 HLA-B27 阳性的幼年特发性关节炎(JIA)。在撒哈拉以南非洲,HLA-B27 阳性的 ERA 尚未成为特定研究的主题。
我们旨在描述塞内加尔 HLA-B27 阳性 ERA 患者在诊断时的临床特征、疾病活动度、功能障碍和治疗情况,并与其他人群进行比较。
我们进行了一项回顾性研究,纳入了根据 2019 年 PRINTO 暂定 ERA 标准,2012 年 1 月至 2022 年 12 月期间发病年龄<18 岁的 ERA 患者的病历。我们收集了人口统计学、临床、实验室和治疗数据。采用强直性脊柱炎疾病活动评分(ASDAS)和 Bath 强直性脊柱炎疾病活动指数(BASDAI)评估疾病活动度。采用 Bath 强直性脊柱炎功能指数(BASFI)评估功能障碍。
共纳入 31 例 HLA-B27 阳性 ERA 患者。31 例患者中,男性 20 例(64.5%)。27 例(87%)为富拉尼族(族群)。症状发作的中位年龄和诊断时的中位年龄分别为 12 岁和 19 岁。7 例患者有强直性脊柱炎家族史。发病时最常见的表现为外周关节炎和附着点炎。29 例(93.5%)存在外周关节炎,27 例(93.1%)位于下肢。26 例(83.8%)存在足跟附着点炎。27 例(87%)存在轴性受累,24 例(88.8%)和 22 例(81.5%)患者分别表现为下腰痛和骶髂痛/臀部痛。7 例(22.5%)患者存在前葡萄膜炎。65.5%和 57.1%的患者红细胞沉降率和 C 反应蛋白升高。影像学检查发现 22 例存在骶髂关节炎。BASDAI 的平均评分为 5.5/10(77.2%的患者疾病高度活动;BASDAI≥4/10)。ASDAS-ESR/CRP 的平均评分为 3.8。BASFI 的平均评分为 5.4/10(80%的患者存在高度功能障碍;BASFI≥4/10)。27 例(87%)患者接受甲氨蝶呤和非甾体抗炎药治疗。治疗 6 个月后,BASDAI 平均评分为 3/10,BASFI 平均评分为 2.5/10。
在我们的研究中,HLA-B27 阳性 ERA 主要在塞内加尔富拉尼族青少年中发现。22 例(70.9%)患者在成年后发展为强直性脊柱炎。诊断时疾病活动度非常高,功能障碍明显。治疗主要基于甲氨蝶呤和非甾体抗炎药。