关键词: Clinical presentation Diagnosis Organ involvement Organ screening Sarcoidosis Treatment indication

Mesh : Humans Male Prospective Studies Netherlands / epidemiology Middle Aged Female Sarcoidosis / epidemiology diagnosis drug therapy complications Adult Uveitis / diagnosis epidemiology drug therapy Prevalence Sarcoidosis, Pulmonary / epidemiology diagnosis drug therapy Immunosuppressive Agents / therapeutic use Central Nervous System Diseases / epidemiology Cardiomyopathies / epidemiology diagnosis Pulmonary Fibrosis / epidemiology Kidney Diseases / epidemiology diagnosis

来  源:   DOI:10.1016/j.rmed.2024.107608

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.
OBJECTIVE: 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:结节病中器官受累的临床表现和患病率差异很大,取决于种族,遗传和地理因素。这些数据没有在荷兰人口中进行广泛研究。
目的:确定荷兰新诊断结节病患者器官受累的患病率和全身免疫抑制治疗的适应症。
方法:两家荷兰大型教学医院参与了这项前瞻性队列研究。前瞻性纳入所有新诊断的结节病成年患者,并进行标准化检查。使用WASOG仪器定义器官受累。
结果:在2015年至2020年之间,共包括330名患者,55%是男性,平均年龄为46(SD14)岁。其中大多数是白人(76%)。316例患者(96%)出现肺部受累,包括胸淋巴结肿大。156例患者(47%)存在肺实质疾病。10例患者(3%)有肺纤维化的放射学征象。74例患者(23%)存在皮肤结节病。常规眼科筛查发现29例患者葡萄膜炎(12%,n=256))。分别在5例(2%)和6例(2%)患者中诊断出心脏和神经结节病。在11例(3%)患者中观察到肾脏受累。在29(10%)和48(26%,n=182)患者,分别。在6例患者中发现肝脏受累(2%)。在30%的患者中,诊断时开始全身免疫抑制治疗.
结论:结节病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适应症。
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