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塞内加尔一家三级医院的儿童发病型类风湿关节炎。

Childhood-onset rheumatoid arthritis at a tertiary hospital in Senegal, West Africa.

机构信息

Department of Rheumatology, Aristide Le Dantec Hospital, Dakar, Senegal.

Department of Rheumatology, COUD Hospital, UCAD, Dakar, Senegal.

出版信息

Pediatr Rheumatol Online J. 2023 Sep 12;21(1):98. doi: 10.1186/s12969-023-00889-6.

Abstract

BACKGROUND

Childhood-onset rheumatoid arthritis (CORA), known as rheumatoid factor (RF)-positive juvenile idiopathic arthritis is a type of juvenile idiopathic arthritis that shares the same genetic factors and clinical features as adult-onset rheumatoid arthritis. In Africa, CORA hasn't been the subject of a specific study.

OBJECTIVES

The aim of this study is to describe the clinical features, disease activity, functional disability, and treatment of CORA at diagnosis in Senegal and compare the findings to other CORA populations.

METHODS

We conducted a mixed cohort study by reviewing the medical records of patients diagnosed with CORA with an age of symptom onset < 18 years according to the 2019 PRINTO provisional criteria for RF-positive JIA from January 2020 to December 2022 at rheumatology department of Aristide Le Dantec Hospital in Dakar, Senegal. We collected demographic, clinical, paraclinical and therapeutic data. Disease activity score was assessed by DAS28-ESR and DAS28-CRP. Functional disability was assessed using Health Assessment Questionnaire (HAQ) or Childhood HAQ.

RESULTS

A total of 21 patients were included. Eighteen (85.7%) were Females. The mean age at symptom onset was 13.0 ± 3.0 years, and at diagnosis was 16.4 ± 4.2 years. Morning stiffness, joint swelling, and joint deformities were found in 20, 18 and 13 patients respectively. Four patients had a family history of rheumatoid arthritis. Five patients had extra-articular involvement such as rheumatoid nodules. Two patients had interstitial lung disease. The biological inflammatory syndrome was found in 90% of cases. 16 of 21 (76.2%) patients had positive RF, and 18 of 20 (90%) patients had positive Anti-CCP. Seven of 12 (58.3%) patients had positive anti-nuclear antibodies. The mean DAS28-ESR was 5.7 ± 1.0. Fifteen (71.4%) patients had high disease activity (DAS28-ESR > 5.1). The mean DAS28-CRP was 5.4 ± 1.1. The median HAQ was 2.12 with a mean HAQ of 1.9. Nineteen (90.5%) patients were treated with methotrexate, while 17 (81%) had a combination of methotrexate and hydroxychloroquine. Oral prednisone was used in 17 (81%) cases. Non-steroidal anti-inflammatory drugs were used in 4 cases (19%). After 6 months of treatment, mean DAS28-CRP was 2.9.

CONCLUSION

In our study, CORA mainly affects 13-year-old girls, characterised by high disease activity with joint deformity and significant functional impairment. Treatment is mainly based on methotrexate, prednisone and hydroxychloroquine. Further studies are needed to determine the exact clinical phenotype of this disease.

摘要

背景

儿童发病的类风湿关节炎(CORA),又称类风湿因子(RF)阳性的青少年特发性关节炎,是一种与成人发病的类风湿关节炎具有相同遗传因素和临床特征的青少年特发性关节炎。在非洲,CORA 尚未成为一项专门研究的课题。

目的

本研究旨在描述塞内加尔 CORA 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 CORA 人群进行比较。

方法

我们对 2020 年 1 月至 2022 年 12 月在塞内加尔达喀尔阿里斯蒂德·勒丹泰克医院风湿科就诊的符合 2019 年 PRINTO 暂定 RF 阳性 JIA 标准、发病年龄<18 岁的 CORA 患者的病历进行了混合队列研究。我们收集了人口统计学、临床、实验室和治疗数据。疾病活动度评分采用 DAS28-ESR 和 DAS28-CRP 评估。使用健康评估问卷(HAQ)或儿童 HAQ 评估功能障碍。

结果

共纳入 21 例患者。18 例(85.7%)为女性。症状起始年龄的平均值为 13.0±3.0 岁,诊断时为 16.4±4.2 岁。20 例患者出现晨僵,18 例患者出现关节肿胀,13 例患者出现关节畸形。4 例患者有类风湿关节炎家族史。5 例患者有类风湿结节等关节外表现。2 例患者患有间质性肺病。90%的病例存在生物炎症综合征。21 例患者中有 16 例(76.2%)RF 阳性,20 例中有 18 例(90%)患者抗环瓜氨酸肽(Anti-CCP)阳性。12 例中有 7 例(58.3%)患者抗核抗体阳性。DAS28-ESR 的平均值为 5.7±1.0。15 例(71.4%)患者疾病活动度高(DAS28-ESR>5.1)。DAS28-CRP 的平均值为 5.4±1.1。HAQ 的中位数为 2.12,平均为 1.9。19 例(90.5%)患者接受甲氨蝶呤治疗,17 例(81%)患者接受甲氨蝶呤联合羟氯喹治疗。17 例(81%)患者使用了口服泼尼松。4 例(19%)患者使用了非甾体抗炎药。治疗 6 个月后,DAS28-CRP 的平均值为 2.9。

结论

在我们的研究中,CORA 主要影响 13 岁的女孩,其特征是疾病活动度高,伴有关节畸形和明显的功能障碍。治疗主要基于甲氨蝶呤、泼尼松和羟氯喹。需要进一步研究以确定该疾病的确切临床表型。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9209/10496368/7a370c3f4790/12969_2023_889_Fig1_HTML.jpg

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