罕见的系统性狼疮伴FS - Jaccoud关节病1例报告并文献复习。

IF 2.4 3区 医学 Q2 ORTHOPEDICS
Shao-Yu Wang, Si-Peng Wang, Liang Kang, Zhi-Hui Pang
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引用次数: 0

摘要

背景:franois - sigismond - Jaccoud关节病(FSJA)是一种罕见的继发性关节疾病,其特征是关节畸形,无骨侵蚀,具有“可复位”模式。这一领域存在大量知识;然而,FSJA的发病机制仍不明确,缺乏明确的诊断标准。此外,还需要采取预防措施和专门的治疗干预措施,以促进FSJA的发展。因此,在这个新颖的报告中,我们提出了一个24岁男性患者继发性FSJA的病例,其特征是病程延长,早期非典型症状,以及原发性系统性红斑狼疮(SLE)诊断不明确。我们的目的是为SLE继发的FSJA的发病机制、诊断和治疗提供进一步的指导。病例介绍:患者有9年多关节痛病史。此外,下肢全身性皮疹和手部畸形持续时间超过6个月,症状在一周内恶化。体格检查发现面部蝴蝶状红斑,手部畸形,关节痛。x线摄影显示关节畸形,无骨侵蚀。实验室结果显示蛋白尿,抗核抗体阳性,补体成分3和4水平降低。根据2019年欧洲抗风湿病联盟/美国风湿病学会的分类标准,诊断SLE继发的狼疮肾炎(LN)和FSJA。患者的SLE LN和关节症状在静脉输注贝利单抗一年后得到改善;除了口服甲基强的松龙,羟氯喹和霉酚酸酯。此外,他的手部畸形并没有明显恶化。结论:SLE继发的FSJA较为罕见;尽管如此,还需要进一步关注和研究这一情况。临床上应常规进行FSJA的鉴别诊断,以避免误诊。SLE患者的早期关节症状表现为无关节变形,需要定期检查关节。因此,可以确保FSJA的早期诊断和及时治疗,减少对其生活质量的影响。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Rare case of systemic lupus with manifestation of FS Jaccoud arthropathy: a case report and literature review.

Background: François-Sigismond Jaccoud arthropathy (FSJA) is a rare, secondary joint disease characterised by joint deformities without bone erosions, with a "reducible" pattern. Substantial knowledge exists in this area; however, the pathogenesis of FSJA remains indefinite, and clear diagnostic criteria are lacking. Moreover, preventive measures and specialised treatment interventions for the development of FSJA are needed. Thus, in this novel report, we present the case of a 24-year-old male patient with secondary FSJA, characterised by a prolonged disease course, early atypical symptoms, and an unclear primary systemic lupus erythematosus (SLE) diagnosis. Our objective was to provide further guidance regarding the pathogenesis, diagnosis, and treatment of FSJA secondary to SLE.

Case presentation: The patient presented with a 9-year medical history of polyarthralgia. Furthermore, a generalised rash of the lower limbs and hand deformities manifested over a 6-month duration, with symptoms worsening over a week. Physical examination revealed butterfly-shaped facial erythema, hand deformities, and arthralgia. Radiography revealed joint deformities without bone erosions. Laboratory findings indicated proteinuria, positive antinuclear antibodies, and decreased levels of complement components 3 and 4. Based on the 2019 European League Against Rheumatism/American College of Rheumatology classification criteria, the diagnoses of lupus nephritis (LN) and FSJA, secondary to SLE were made. The patient's SLE LN and joint symptoms improved after a year of intravenous belimumab infusion; in addition to oral methylprednisolone, hydroxychloroquine, and mycophenolate mofetil. Additionally, his hand deformities did not considerably worsen.

Conclusions: FSJA secondary to SLE is rare; nonetheless, further attention and studies regarding this condition are required. Clinically, the differential diagnosis of FSJA should routinely be performed to avoid a misdiagnosis. The manifestation of early joint symptoms without joint deformation requires regular examinations of the joints, in patients with SLE. The early diagnosis and prompt management of FSJA will thus be ensured, reducing the impact on their quality of life.

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来源期刊
BMC Musculoskeletal Disorders
BMC Musculoskeletal Disorders 医学-风湿病学
CiteScore
3.80
自引率
8.70%
发文量
1017
审稿时长
3-6 weeks
期刊介绍: BMC Musculoskeletal Disorders is an open access, peer-reviewed journal that considers articles on all aspects of the prevention, diagnosis and management of musculoskeletal disorders, as well as related molecular genetics, pathophysiology, and epidemiology. The scope of the Journal covers research into rheumatic diseases where the primary focus relates specifically to a component(s) of the musculoskeletal system.
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