Analysis of sarcoidosis in the Oporto region (Portugal)

A.V. Cardoso , P.C. Mota , N. Melo , S. Guimarães , C. Souto Moura , J.M. Jesus , R. Cunha , A. Morais
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引用次数: 10

Abstract

Background

Sarcoidosis is a systemic granulomatous disease of unknown etiology. Epidemiological studies of different populations are essential because clinical presentation, organ involvement, disease severity, and prognosis vary significantly according to region and population. The aim of this study was to assess epidemiological and clinical characteristics, staging factors, and clinical course in patients with sarcoidosis from a tertiary hospital in Oporto, Portugal.

Methods

A retrospective analysis of patients with sarcoidosis and at least 2 years of follow-up evaluated at the Centro Hospitalar de São João between 2000 and 2014.

Results

We identified 409 patients with sarcoidosis (females, 58.9%; mean age at diagnosis, 38.9 ± 13.4 years; smokers, 14.4%]. All the patients were diagnosed according to the ERS/ATS/WASOG consensus statement and 64.1% had evidence of noncaseating epithelioid cell granulomas in biopsy specimens. Bronchoalveolar lavage was performed as part of the diagnostic work-up in 289 patients and 90.2% had lymphocytosis (CD4/CD8 ratio ≥ 3.5 in 60.9% of cases). Exertion dyspnea, cough, and constitutional symptoms were the most common presenting symptoms; 10.1% of patients were asymptomatic, 22.8% had Löfgren syndrome, and 50.5% had extrathoracic involvement. Radiographic stages of disease according to the Scadding criteria were as follows: stage 0 (5.2%), stage I (33.7%), stage II (47.0%), stage III (8.4%), and stage IV (5.7%). Impaired respiratory function was observed in 45.6% patients and was mostly mild. Systemic treatment was administered in 58.6% of cases. Overall, 45.3% of patients experienced disease resolution.

Conclusion

The epidemiological and clinical characteristics of this cohort of patients with sarcoidosis from the Oporto region in northern Portugal revealed epidemiological and clinical characteristics that were generally similar to those described in other Western Europe populations and in the US ACCESS study. However, we found a higher proportion of patients who progressed to chronic forms.

葡萄牙波尔图地区结节病分析
背景:结节病是一种病因不明的系统性肉芽肿性疾病。不同人群的流行病学研究是必要的,因为临床表现、器官受累、疾病严重程度和预后因地区和人群而有显著差异。本研究的目的是评估葡萄牙波尔图一家三级医院结节病患者的流行病学和临床特征、分期因素和临床病程。方法回顾性分析2000年至2014年在奥约中心医院接受随访至少2年的结节病患者。结果409例结节病患者(女性,58.9%;平均诊断年龄38.9±13.4岁;烟民,14.4%)。所有患者的诊断均符合ERS/ATS/WASOG共识声明,64.1%的患者活检标本中有非干酪化上皮样细胞肉芽肿的证据。289例患者进行支气管肺泡灌洗作为诊断检查的一部分,90.2%的患者有淋巴细胞增多(60.9%的病例中CD4/CD8比值≥3.5)。用力呼吸困难、咳嗽和体质症状是最常见的症状;10.1%的患者无症状,22.8%有Löfgren综合征,50.5%有胸外受累。scadd标准的x线分期为:0期(5.2%)、I期(33.7%)、II期(47.0%)、III期(8.4%)和IV期(5.7%)。45.6%患者呼吸功能受损,且多为轻度。58.6%的病例接受了全身治疗。总体而言,45.3%的患者经历了疾病缓解。结论来自葡萄牙北部波尔图地区的结节病患者的流行病学和临床特征与其他西欧人群和美国ACCESS研究中描述的流行病学和临床特征大致相似。然而,我们发现进展为慢性形式的患者比例较高。
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