西非塞内加尔 HLA-B27 阳性关节炎/脊柱炎相关关节炎的概况。

IF 2.8 3区 医学 Q1 PEDIATRICS
Mounib M Sabounji, Aïssatou Ndiaye, Saïdou Diallo
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引用次数: 0

摘要

背景:切肌炎/脊柱炎相关关节炎(ERA)是幼年特发性关节炎(JIA)的一种类型,经常与 HLA-B27 相关。在撒哈拉以南非洲地区,HLA-B27阳性的ERA尚未成为专门研究的对象:我们旨在描述塞内加尔确诊的 HLA-B27 阳性 ERA 的临床特征、疾病活动性、功能障碍和治疗情况,并将研究结果与其他人群进行比较:方法:我们对确诊为ERA的患者的病历进行了回顾性研究,并注明了发病年龄:共纳入 31 名 HLA-B27 阳性的ERA 患者。31 人中有 20 人(64.5%)为男性。27人(87%)为富拉族(Fula)。发病年龄和确诊年龄的中位数分别为12岁和19岁。七名患者有脊柱关节炎家族史。外周关节炎和腱鞘炎是发病时最常见的表现特征。29例(93.5%)患者出现外周关节炎,27/29例(93.1%)患者的关节位于下肢。26名患者(83.8%)出现足跟关节炎。27名(87%)患者出现轴向受累,主要表现为腰背痛,24名(88.8%)和22名(81.5%)患者出现骶髂关节痛/臀部疼痛。7名患者(22.5%)患有前葡萄膜炎。分别有 65.5% 和 57.1% 的病例血沉和 CRP 升高。影像学检查发现,22 名患者患有骶髂关节炎。平均 BASDAI 为 5.5/10(77.2% 的患者病情高度活跃;BASDAI ≥ 4/10)。ASDAS-ESR/CRP 平均值为 3.8。平均 BASFI 为 5.4/10(80% 的患者有高度功能障碍;BASFI ≥ 4/10)。27名患者(87%)接受了甲氨蝶呤和非甾体抗炎药治疗。治疗 6 个月后,平均 BASDAI 为 3/10,平均 BASFI 为 2.5/10:在我们的研究中,HLA-B27 阳性的 ERA 主要出现在塞内加尔的富拉族青少年中。22名(70.9%)患者在成年后发展为强直性脊柱炎。确诊时病情非常活跃,并伴有严重的功能障碍。治疗主要以甲氨蝶呤和非那西汀为主。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Profile of HLA-B27-positive enthesitis/spondylitis-related arthritis in Senegal, West Africa.

Background: 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.

Objectives: 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.

Methods: 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).

Results: 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.

Conclusion: 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.

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来源期刊
Pediatric Rheumatology
Pediatric Rheumatology PEDIATRICS-RHEUMATOLOGY
CiteScore
4.10
自引率
8.00%
发文量
95
审稿时长
>12 weeks
期刊介绍: Pediatric Rheumatology is an open access, peer-reviewed, online journal encompassing all aspects of clinical and basic research related to pediatric rheumatology and allied subjects. The journal’s scope of diseases and syndromes include musculoskeletal pain syndromes, rheumatic fever and post-streptococcal syndromes, juvenile idiopathic arthritis, systemic lupus erythematosus, juvenile dermatomyositis, local and systemic scleroderma, Kawasaki disease, Henoch-Schonlein purpura and other vasculitides, sarcoidosis, inherited musculoskeletal syndromes, autoinflammatory syndromes, and others.
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