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津巴布韦的系统性硬化症:自身抗体生物标志物、临床和实验室相关性。

Systemic Sclerosis in Zimbabwe: Autoantibody Biomarkers, Clinical, and Laboratory Correlates.

机构信息

Department of Pathology, Faculty of Medicine, National University of Science and Technology, Bulawayo, Zimbabwe.

Asthma Allergy and Immunology Clinic, Harare, Zimbabwe.

出版信息

Front Immunol. 2021 Nov 9;12:679531. doi: 10.3389/fimmu.2021.679531. eCollection 2021.

Abstract

INTRODUCTION

Systemic sclerosis (SScl) is an autoimmune disease whose prevalence is rarely reported in Africa. Autoantibodies are the biomarkers of the condition, precede overt disease and determine disease phenotypes. SSc specific autoantibodies also vary between racial groupings. Objective: To investigate the clinical and laboratory characteristics of Zimbabwean patients who were reactive SSc specific autoantibodies.

MATERIALS AND METHOD

240 patients, 173 of them female with SSc specific autoantibodies were included. Autoantibodies were detected by indirect immunofluorescence microscopy and immunoblotting using a panel of 13 SScl (Euroimmun Ag., Germany). Demographic, clinical and laboratory parameters relevant to the monitoring of SScl were captured. These included pulmonary function tests, hematology, clinical chemistry, serology and thyroid function tests. Allergy skin prick tests (SPT) to inhalant and food allergen sources were conducted when indicated.

RESULTS

All the 240 patients (median age was 36 years) expressed SSc specific autoantibodies. 86% were Black, 11% White and 3% Asian and a fifth (20%) were younger than 16 years. Eleven (4.6%) fulfilled the ACR/EULAR classification of SSc. Clinically they had limited cutaneous (n=6), diffuse cutaneous (n=3) and SScl/inflammatory myopathy overlap (n=2). The most frequently detected antibodies anti-RNA polymerase III (RNAP) 55%, anti-Th/To (28%) anti-RNAP 11 (22%), anti-CENPB (18%) and anti-Scl-70/ATA (13%). Racial variations in the expression of these antibodies were apparent between Black, White and Asian patients. The majority (95%), who did not fulfil the ARA/EULAR criteria were symptomatic. Raynaud's Phenomenon was documented in 24%. Respiratory symptoms included coughing, dyspnea and wheezing. There was a restrictive ventilatory defect with increased FEV1/FVC ratio. Pruritus, urticaria and skin depigmentation were the main cutaneous features while constipation, bloating, Gastroesophageal reflux disease (GERD) and abdominal pain dominated GI symptoms. Mean blood pressure readings while normal varied with biomarkers. Haematology and biochemistry parameters were within normal reference ranges.

CONCLUSION

The expression of SSc specific autoantibodies is common and associated with known SSc symptoms. The types and frequency of autoantibodies varied with racial groupings. A fifth of the patients were children below the age of 16 years.

摘要

简介

系统性硬化症(SScl)是一种自身免疫性疾病,其在非洲的发病率很少有报道。自身抗体是该疾病的生物标志物,先于明显的疾病出现,并决定疾病表型。SSc 特异性自身抗体在不同的种族群体中也有所不同。目的:调查对津巴布韦患者反应性 SSc 特异性自身抗体的临床和实验室特征。

材料和方法

共纳入 240 例患者,其中 173 例为女性,具有 SSc 特异性自身抗体。使用 13 种 SScl(Euroimmun Ag.,德国)的面板通过间接免疫荧光显微镜和免疫印迹法检测自身抗体。捕获与 SScl 监测相关的人口统计学、临床和实验室参数。这些包括肺功能测试、血液学、临床化学、血清学和甲状腺功能测试。当需要时,进行过敏原皮肤点刺试验(SPT)以检测吸入性和食物过敏原来源。

结果

所有 240 例患者(中位年龄为 36 岁)均表达 SSc 特异性自身抗体。86%为黑人,11%为白人,3%为亚洲人,五分之一(20%)的患者年龄小于 16 岁。11 例(4.6%)符合 ACR/EULAR 的 SSc 分类标准。临床上他们有局限性皮肤(n=6)、弥漫性皮肤(n=3)和 SScl/炎症性肌病重叠(n=2)。最常检测到的抗体是抗 RNA 聚合酶 III(RNAP)55%、抗 Th/To(28%)抗 RNAP 11(22%)、抗 CENPB(18%)和抗 Scl-70/ATA(13%)。黑人、白人和亚洲患者之间这些抗体的表达存在明显的种族差异。大多数(95%)不符合 ARA/EULAR 标准的患者有症状。记录到 24%的雷诺现象。呼吸道症状包括咳嗽、呼吸困难和喘息。存在限制性通气缺陷,FEV1/FVC 比值增加。瘙痒、荨麻疹和皮肤色素脱失是主要的皮肤特征,而便秘、腹胀、胃食管反流病(GERD)和腹痛是胃肠道症状的主要表现。虽然正常血压读数变化,但平均血压读数因生物标志物而异。血液学和生化参数均在正常参考范围内。

结论

SSc 特异性自身抗体的表达很常见,并与已知的 SSc 症状相关。自身抗体的类型和频率因种族群体而异。五分之一的患者是年龄在 16 岁以下的儿童。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c145/8631108/9fd5ac21da3c/fimmu-12-679531-g001.jpg

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