• Kimberly Showalter, Aileen Hoffmann, Gerald Rouleau, David Aaby, Jungwha Lee, Carrie Richardson, Jane Dematte, Rishi Agrawal, Rowland W Chang, and Monique Hinchcliff.
• From the Department of Medicine, Division of Rheumatology, Division of Pulmonary and Critical Care Medicine, Department of Preventive Medicine, Institute for Public Health and Medicine, Department of Radiology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
• J Rheumatol. 2018 Nov 1; 45 (11): 1572-1576.

ObjectiveForced 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.MethodsPatients 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.ResultsThe 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).ConclusionRadiographic 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.

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