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荷兰新诊断的类肉瘤病患者的器官受累:首个大型欧洲多中心前瞻性研究。

Organ involvement in newly diagnosed sarcoidosis patients in the Netherlands: The first large European multicentre prospective study.

机构信息

Department of Respiratory Medicine, Zuyderland Medical Centre, Heerlen/Sittard, the Netherlands.

Department of Respiratory Medicine, OLVG, Amsterdam, the Netherlands.

出版信息

Respir Med. 2024 May;226:107608. doi: 10.1016/j.rmed.2024.107608. Epub 2024 Apr 4.

Abstract

BACKGROUND

Clinical presentation and prevalence of organ involvement is highly variable in sarcoidosis and depends on ethnic, genetic and geographical factors. These data are not extensively studied in a Dutch population.

AIM

To determine the prevalence of organ involvement and the indication for systemic immunosuppressive therapy in newly diagnosed sarcoidosis patients in the Netherlands.

METHODS

Two large Dutch teaching hospitals participated in this prospective cohort study. All adult patients with newly diagnosed sarcoidosis were prospectively included and a standardized work-up was performed. Organ involvement was defined using the WASOG instrument.

RESULTS

Between 2015 and 2020, a total of 330 patients were included, 55% were male, mean age was 46 (SD 14) years. Most of them were white (76%). Pulmonary involvement including thoracic lymph node enlargement was present in 316 patients (96%). Pulmonary parenchymal disease was present in 156 patients (47%). Ten patients (3%) had radiological signs of pulmonary fibrosis. Cutaneous sarcoidosis was present in 74 patients (23%). Routine ophthalmological screening revealed uveitis in 29 patients (12%, n = 256)). Cardiac and neurosarcoidosis were diagnosed in respectively five (2%) and six patients (2%). Renal involvement was observed in 11 (3%) patients. Hypercalcaemia and hypercalciuria were observed in 29 (10%) and 48 (26%, n = 182) patients, respectively. Hepatic involvement was found in 6 patients (2%). In 30% of the patients, systemic immunosuppressive treatment was started at diagnosis.

CONCLUSIONS

High-risk organ involvement in sarcoidosis is uncommon at diagnosis. Indication for systemic immunosuppressive therapy was present in a minority of patients.

摘要

背景

结节病的临床表现和器官受累的患病率因种族、遗传和地理因素而有很大差异。这些数据在荷兰人群中尚未广泛研究。

目的

确定荷兰新诊断结节病患者的器官受累患病率和全身免疫抑制治疗的指征。

方法

两家大型荷兰教学医院参与了这项前瞻性队列研究。所有新诊断的结节病成年患者均前瞻性纳入,并进行了标准化检查。使用 WASOG 仪器定义器官受累。

结果

在 2015 年至 2020 年间,共纳入 330 例患者,其中 55%为男性,平均年龄为 46(SD 14)岁。他们大多为白人(76%)。316 例(96%)存在肺部受累,包括胸内淋巴结肿大。156 例(47%)存在肺部实质疾病。10 例(3%)患者存在肺纤维化的影像学征象。74 例(23%)存在皮肤结节病。常规眼科筛查发现 29 例(12%,n=256)患者存在葡萄膜炎。诊断出 5 例(2%)和 6 例(2%)心脏和神经结节病。分别有 11 例(3%)和 48 例(26%,n=182)患者出现肾脏受累和高钙尿症。29 例(10%)患者出现高钙血症和高钙尿症,48 例(26%,n=182)患者出现高钙血症和高钙尿症。6 例(2%)患者出现肝脏受累。30%的患者在诊断时开始接受全身免疫抑制治疗。

结论

结节病的高危器官受累在诊断时并不常见。少数患者存在全身免疫抑制治疗的指征。

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