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. Author manuscript; available in PMC: 2021 Oct 1.
Published in final edited form as: Arthritis Care Res (Hoboken). 2020 Oct;72(10):1392–1403. doi: 10.1002/acr.24043

Table 5.

Performance of algorithms with exclusion of other causes of interstitial lung disease (Stage IV)

Algorithm Description Sensitivity Specificity PPV NPV %Agreement Kappa
Stringent ILD definition
4A ≥1 pulmonologist ILD diagnosis and exclusion other ILDa 60.4 (49.2, 70.7) 98.7 (97.9, 99.2) 81.1 (70.8, 88.4) 96.4 (94.4, 97.7) 95.4 0.67 (0.57, 0.75)
4C CT 7-180 days prior to ILD diagnosis and exclusion other ILDa 69.7 (56.5, 80.4) 97.9 (97.0, 98.6) 76.0 (65.9, 83.8) 97.2 (95.1, 98.4) 95.5 0.70 (0.60, 0.79)
4D PFT 7-180 days prior to ILD diagnosis and exclusion other ILDa 71.7 (59.6, 81.4) 98.2 (97.7, 98.6) 78.8 (72.7, 83.8) 97.4 (95.6, 98.5) 96.0 0.73 (0.65, 0.80)
4G ≥1 pulmonologist diagnosis and CT or lung biopsy or PFTs 7-180 days prior to ILD diagnosis and exclusion other ILDa 59.8 (48.7, 70.0) 98.8 (98.1, 99.2) 81.9 (72.2, 88.7) 96.4 (94.4, 97.7) 95.5 0.67 (0.57, 0.75)
4H CT and PFTs or lung biopsy 7-180 days prior to ILD diagnosis and exclusion other ILDa 66.9 (54.9, 77.1) 98.7 (98.2, 99.0) 82.9 (76.9, 87.6) 96.9 (94.8, 98.1) 95.9 0.72 (0.63, 0.79)
4I Pulmonologist diagnosis or CT and PFTs or lung biopsy 7-180 days prior to ILD diagnosis and exclusion other ILDa 73.2 (60.3, 83.0) 98.2 (97.4, 98.7) 78.5 (70.1, 85.1) 97.6 (95.7, 98.6) 96.1 0.74 (0.65, 0.81)
Relaxed ILD definition
4A ≥1 pulmonologist ILD diagnosis and exclusion other ILDa 52.8 (42.9, 62.4) 98.8 (98.0, 99.3) 82.9 (72.6, 90.0) 95.0 (92.7, 96.6) 94.2 0.62 (0.52, 0.70)
4C CT 7-180 days prior to ILD diagnosis and exclusion other ILDa 63.0 (51.1, 73.6) 98.3 (97.3, 98.9) 80.3 (70.1, 87.6) 96.0 (93.5, 97.5) 94.7 0.68 (0.58, 0.77)
4D PFT 7-180 days prior to ILD diagnosis and exclusion other ILDa 65.4 (53.9, 75.4) 98.7 (98.2, 99.0) 84.2 (78.6, 88.6) 96.3 (94.1, 97.7) 95.4 0.71 (0.62, 0.79)
4G ≥1 pulmonologist diagnosis and CT or lung biopsy or PFTs 7-180 days prior to ILD diagnosis and exclusion other ILDa 52.2 (42.5, 61.7) 98.9 (98.2, 99.3) 83.7 (74.1, 90.3) 95.0 (92.7, 96.6) 94.3 0.61 (0.52, 0.70)
4H CT and PFTs or lung biopsy 7-180 days prior to ILD diagnosis and exclusion other ILDa 60.0 (49.2, 70.0) 98.9 (98.5, 99.3) 86.6 (81.0, 90.8) 95.6 (93.2, 97.2) 95.0 0.68 (0.59, 0.76)
4I Pulmonologist diagnosis or CT and PFTs or lung biopsy 7-180 days prior to ILD diagnosis and exclusion other ILDa 65.4 (53.6, 75.5) 98.5 (97.7, 99.0) 82.4 (74.0, 88.5) 96.3 (94.1, 97.7) 95.2 0.70 (0.61, 0.78)
a

exclusion of other ILD using diagnostic codes for pneumoconioses, radiation, hypersensitivity pneumonitis, other connective tissue diseases on or after the last ILD diagnosis code date (see Table S3)

Abbreviations: PPV, positive predictive value; NPV, negative predictive value; ILD, interstitial lung disease; CT, computed tomography; PFT, pulmonary function test