南印度一家三级中心系统性硬化症患者的临床和皮肤特征

Priya Kuryan, C. Peter, S. Pulimood, L. George, John Mathew
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摘要

背景与目的:系统性硬化症是一种自身免疫性疾病,其中皮肤硬化症是其主要特征。其他皮肤表现包括雷诺现象、指溃疡、坏疽、屈曲性挛缩和毛细血管扩张。已经注意到,不同种族的临床和抗体特征各不相同。本研究的目的是研究南印度一家三级中心系统性硬化症患者的临床特征,并与其他种族进行比较。材料和方法:在2014年2月至2015年9月的研究期间,招募了35名患者。主要研究者对疾病细节、全身表现、形态和皮肤表现的分布进行了记录和调查。皮肤硬化症的严重程度通过改良的Rodnan皮肤评分(mRSS)进行评估。结果:我们的研究在91%的患者中以弥漫型疾病的女性为主。常见的皮肤表现为硬化(n=35100%)、雷诺现象(n=2517.14%)和色素紊乱(n=33194.3%)。呼吸系统是最常见的受累系统,间质性肺病占78.1%(n=25)。mRSS的增加与间质性肺病之间存在显著相关性(P=0.026)。结论:色素紊乱在我们的人群中较高,而雷诺现象、指溃疡和毛细血管扩张在西方人群中较不常见。mRSS与间质性肺病之间存在显著相关性(P=0.026)。
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Clinical and cutaneous profile of patients with systemic sclerosis in a tertiary centre in South India
Background and Aims: Systemic sclerosis is an autoimmune disease, of which cutaneous sclerosis is a cardinal feature. Other skin manifestations include Raynaud's phenomenon, digital ulcers, gangrene, flexion contractures, and telangiectasias. It has been noted that the clinical and antibody profile varies within ethnic groups. The objective of the study was to study the clinical profile of patients with systemic sclerosis in a tertiary center in South India and to compare to other ethnic groups. Materials and Methods: Thirty-five patients were recruited in the study period between February 2014 and September 2015. A record of the disease details, systemic manifestations, morphology, and distribution of the cutaneous manifestations and investigations were done by the principal investigator. The severity of cutaneous sclerosis was assessed by modified Rodnan Skin Score (mRSS). Results: Our study had a female preponderance with diffuse type of disease in 91% of patients. The common cutaneous presenting complaints were sclerosis (n = 35, 100%), Raynaud's phenomenon (n = 25, 71.4%), and pigmentary disturbances (n = 33, 94.3%). Respiratory system was the most common system involved, with interstitial lung disease seen in 78.1% (n = 25). A significant association (P = 0.026) was noticed between the increase in mRSS and interstitial lung disease. Conclusion: Pigmentary disturbances were higher in our population, whereas Raynaud's phenomenon, digital ulcers, and telangiectasia were less common than in Western population. A significant association (P = 0.026) was found between mRSS and interstitial lung disease.
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