Performance of Forced Vital Capacity and Lung Diffusion Cutpoints for Associated Radiographic Interstitial Lung Disease in Systemic Sclerosis

Objective. Forced vital capacity (FVC) and DLCO are used for screening of systemic sclerosis associated interstitial lung disease (SSc-ILD). The study purpose was to determine the sensitivity, specificity, and negative predictive value (NPV) (proportion of true negative screening tests) of FVC and DLCO thresholds for SSc-ILD on chest high-resolution computed tomography (HRCT) scans. Methods. Patients fulfilling American College of Rheumatology 2013 SSc criteria with a chest HRCT scan and pulmonary function tests (PFT) were studied. A thoracic radiologist quantified radiographic ILD. Optimal FVC and DLCO % predicted thresholds for ILD were identified using receiver-operating characteristic curves. The FVC and DLCO combinations with greatest sensitivity and specificity were also determined. Subanalysis was performed in patients with positive Scl-70 autoantibodies. Results. The study included 265 patients. Of 188 (71%) with radiographic ILD, 59 (31%) had normal FVC (>= 80% predicted), and 65 out of 151 (43%) had normal DLCO (>= 60% predicted). FVC < 80% (sensitivity 0.69, specificity 0.73), and DLCO < 62% (sensitivity 0.60, specificity 0.70) were optimal thresholds for radiographic SSc-ILD. All FVC and DLCO threshold combinations evaluated had NPV < 0.70. The NPV for radiographic ILD for FVC < 80% was lower in patients with positive Scl-70 autoantibody (NPV = 0.05) compared to negative Scl-70 autoantibody (NPV = 0.57). Conclusion. Radiographic ILD is prevalent in SSc despite normal PFT. No % predicted FVC or DLCO threshold combinations yielded high NPV for SSc-ILD screening. Normal FVC and DLCO in patients with SSc, especially those with positive Scl-70 autoantibodies, should not obviate consideration of HRCT for ILD evaluation.