Abstract：
Rationale: The Sarcoidosis Diagnostic Score (SDS) has been established to quantitate the clinical features consistent with sarcoidosis in a monocentric study. Objectives: We aimed to confirm the diagnostic value of SDS in a large, multicontinental study and to assess the utility of SDS in differentiating sarcoidosis from alternative diagnoses, including infectious and noninfectious granulomatous diseases. Methods: We included patients with biopsy-confirmed sarcoidosis at nine centers across the world. Patients without sarcoidosis seen at the same sites served as control patients. Using a modified World Association of Sarcoidosis and Other Granulomatous Disorders organ assessment instrument, we scored all patients for the presence of granuloma on biopsy, highly probable symptoms, and least probable symptoms for each area. Two sarcoidosis scores were generated: SDS Biopsy (with biopsy) and SDS Clinical (without biopsy). SDS Clinical and Biopsy were calculated for all patients. We calculated and compared the area under the curve (AUC) for SDS Clinical and Biopsy according to different diagnosis scenarios. Results: A total of 1,041 patients with sarcoidosis and 1,035 without sarcoidosis were included. The results for SDS Clinical (AUC, 0.888; 95% confidence interval [CI], 0.874-0.902) and SDS Biopsy (AUC, 0.979; 95% CI, 0.973-0.985) according to AUC were good to excellent for differentiating sarcoidosis from alternative diagnosis. SDS Clinical was less discriminatory in males (P = 0.01) and in high tuberculosis prevalence centers (P < 0.001). However, SDS Clinical (AUC, 0.684; 95% CI, 0.602-0.766) and SDS Biopsy (AUC, 0.754; 95% CI, 0.673-0.835) were not sufficiently discriminative for noninfectious granulomatous diseases, but both SDSs could differentiate sarcoidosis from infectious granulomatous diseases. Algorithms were proposed for the SDS Clinical and SDS Biopsy to assist the clinician in the diagnostic process, and cutoff values were proposed for the SDS Clinical and SDS Biopsy, allowing the diagnosis of sarcoidosis to be safely confirmed or rejected in most cases except for noninfectious granulomatous disease. Conclusions: This multicontinental study confirms that both SDS Clinical and SDS Biopsy have good to excellent performance in discriminating sarcoidosis from alternative diagnoses. Differences in the AUC were seen for high tuberculosis prevalence versus low tuberculosis prevalence centers and for males versus females. Both SDSs had good discriminatory function for infectious granulomatous disease but failed in cases of noninfectious granulomatous disease such as berylliosis.
摘要：
原理：结节病诊断评分（SDS）已建立，以定量单中心研究中与结节病一致的临床特征。目的：我们的目的是证实SDS在一个大的诊断价值，多大陆研究，并评估SDS在区分结节病与替代诊断中的实用性，包括感染性和非感染性肉芽肿病。方法：我们纳入了全世界9个中心活检证实结节病的患者。在相同部位没有结节病的患者作为对照患者。使用改良的世界结节病和其他肉芽肿性疾病协会器官评估工具，我们对所有患者的肉芽肿进行活检，极可能的症状,和每个地区最不可能的症状。产生两个结节病评分:SDS活检(带活检)和SDS临床(不带活检)。对所有患者进行SDS临床和活检。我们根据不同的诊断方案计算并比较了SDS临床和活检的曲线下面积（AUC）。结果：共纳入1,041例结节病患者和1,035例非结节病患者。SDS临床结果(AUC，0.888；95%置信区间[CI]，0.874-0.902）和SDS活检（AUC，0.979;95％CI，0.973-0.985）根据AUC对于区分结节病与替代诊断是良好的。男性（P=0.01）和高结核病患病率中心（P<0.001）的SDS临床歧视较少。然而,SDS临床（AUC，0.684；95%CI，0.602-0.766）和SDS活检（AUC，0.754；95%CI，0.673-0.835）对非感染性肉芽肿疾病的区分度不足，但两种SDS均可将结节病与感染性肉芽肿病区分开来.提出了SDS临床和SDS活检的算法，以协助临床医生进行诊断。并提出了SDS临床和SDS活检的临界值，允许结节病的诊断在大多数情况下被安全确认或拒绝，除了非感染性肉芽肿疾病。结论：这项多大陆研究证实，SDS临床和SDS活检在区分结节病与其他诊断方面均具有良好至出色的表现。对于高结核病患病率与低结核病患病率中心以及男性与女性，观察到AUC的差异。两种SDS对感染性肉芽肿性疾病均具有良好的辨别功能，但在诸如铍病之类的非感染性肉芽肿性疾病中失败。
