Sarcoidosis Diagnostic Score: A Systematic Evaluation to Enhance the Diagnosis of Sarcoidosis

Abstract

The diagnosis of sarcoidosis is made by the combination of clinical features and biopsy results. The clinical features of sarcoidosis can be quite variable. We developed a Sarcoidosis Diagnostic Score (SDS) to summarize the clinical features of patients with possible sarcoidosis. Biopsy-confirmed patients with sarcoidosis seen during a 7-month period at the University of Cincinnati sarcoidosis clinic were prospectively identified. Patients with nonsarcoidosis seen at the same clinic were used as control patients. Using a modified World Association of Sarcoidosis and Other Granulomatous Disorders organ assessment instrument, we scored all patients for presence of biopsy,≥1 highly probable symptom, and≥1 at least probable symptom for each area. Two sarcoidosis scores were generated: SDS biopsy (with biopsy) and SDS clinical (without biopsy). The 980 evaluable patients were divided into two cohorts: an initial 600 patients (450 with biopsy-confirmed sarcoidosis, 150 control patients) to establish cutoff values for SDS biopsy and an SDS clinical and a validation cohort of 380 patients (103 biopsy-confirmed patients with sarcoidosis and 277 control patients). The best cutoff value for SDS biopsy was≥ 6 (sensitivity, 99.3%; specificity, 100%). For the total the 980 patients, an SDS clinical≥ 3 had a sensitivity of 90.6%, specificity of 88.5%, and a likelihood ratio of 7.9. An SDS clinical score≥ 4 had a lower sensitivity of (76.9%) but higher specificity (98.6%). For sarcoidosis, the presence of specific clinical features, especially multiorgan involvement, can enhance the diagnostic certainty. The SDS scoring system quantitated the clinical features consistent with sarcoidosis.