RESUMO
BACKGROUND: The Sarcoidosis Diagnostic Score (SDS) system has been established for sarcoidosis patients based on the WASOG organ involvement criteria. We evaluated modifications of the SDS system to determine if they improved its the diagnostic accuracy. METHODS: Biopsy-confirmed patients with sarcoidosis seen during a 7-month period at 9 sarcoidosis centers across the world. Patients with non-sarcoidosis seen at the same sites were served as control patients. Comparing the SDS-biopsy and SDS-clinical values of five groups: duration of symptoms prior to evaluation (≤1 years vs.>1 years, ≤2 years vs.>2 years), organ involvement (lung, eye, or cardiac), race, and sex. RESULTS: A total of 990 patients with sarcoidosis and 1011 controls were included in this study. The SDS-clinical was significantly more discriminating for those undergoing assessment with symptoms for more than one year (z-statistic=2.570, p = 0.0102) or two years (z-statistic=2.546, p = 0.0109). However, the addition of two points for both >1 years and >2 years since onset of symptoms did not increase sensitivity and specificity of diagnosis with the SDS system. The SDS-clinical cut-off for patients with ocular or cardiac disease was two points higher than that for lung disease. There was no difference in SDS-clinical or biopsy AUC values based on gender or race. CONCLUSIONS: The longer the duration of symptoms prior to diagnosis, the more likely the diagnosis of sarcoidosis was correct. For patients presenting with ocular or cardiac symptoms, evidence of multi-organ involved can improve the diagnostic accuracy of the SDS-clinical.
RESUMO
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.