Table 2.
Subgroup demographics with respect to interstitial lung disease patterns, as determined in the original CT Thorax.
| UIP | NSIP | DIP | CPFE | Unspecific | ||||||
|---|---|---|---|---|---|---|---|---|---|---|
| Patient, n (%) | 13 | (43) | 9 | (29) | 2 | (6) | 2 | (6) | 5 | (16) |
| Female, n (%) | 9 | (69) | 5 | (56) | 2 | (100) | 1 | (50) | 3 | (60) |
| Age Onset, years | 58.9 | [50.6–68.8] | 57.3 | [50.0–64.3] | 36.8 | [36.2–37.4] | 60.6 | [43.5–77.8] | 67.8 | [63.9–68.3] |
| Age SS, years | 59.0 | [50.7–68.8] | 57.7 | [50.2–68.4] | 45.7 | [37.5–53.9] | 60.7 | [43.6–77.9] | 67.9 | [64.0–68.4] |
| Age ILD, years | 61.9 | [54.9–69.3] | 57.4 | [51.1–64.4] | 36.9 | [36.2–37.5] | 62.7 | [47.6–77.9] | 68.4 | [65.9–70.8] |
| Never smoker, n (%) | 10 | (77) | 6 | (67) | 1 | (50) | 1 | (50) | 5 | (100) |
| – Pack years | 2 | 9 | 3 | 18 | - | |||||
| ESSDAI score | 18 | [14–25] | 17 | [15–22] | 19 | [13–25] | 12 | [10–14] | 22 | [21–25] |
| Lung function at ILD diagnosis | ||||||||||
| FVC, % pred | 60 | [46–65] | 70 | [54–76] | 50 | [45–55] | 98 | [93–102] | 79 | [78–98] |
| DLCO, % pred | 53 | [36–71] | 47 | [41–68] | 35 | [29–41] | 70 | [55–84] | 79 | [63–84] |
| Treatment | ||||||||||
| 1st line treatment | ||||||||||
| CYC, n (%) | 6 | (45) | 5 | (56) | 2 | (100) | 0 | - | 0 | - |
| AZA, n (%) | 4 | (31) | 1 | (11) | 0 | - | 1 | (50) | 2 | (40) |
| MTX, n (%) | 1 | (8) | 1 | (11) | 0 | - | 1 | (50) | 2 | (40) |
| HCQ, n (%) | 0 | - | 1 | (11) | 0 | - | 0 | - | 1 | (20) |
| RTX, n (%) | 1 | (8) | 0 | - | 0 | - | 0 | - | 0 | - |
| MMF, n (%) | 1 | (8) | 0 | - | 0 | - | 0 | - | 0 | - |
| Number treatments | 3 | [2–4] | 3 | [2–3] | 2 | [1–3] | 3 | [1–4] | 2 | [2–3] |
| Treatment outcomes | ||||||||||
| FVC | ||||||||||
| Improved, n (%) | 2 | (15) | 1 | (12) | 2 | (100) | 0 | - | 0 | - |
| Stabilized, n (%) | 8 | (62) | 4 | (44) | 0 | - | 2 | (100) | 3 | (100) |
| Declined, N (%) | 3 | (23) | 4 | (44) | 0 | - | 0 | - | 0 | - |
| DLCO | ||||||||||
| Improved, n (%) | 3 | (23) | 1 | (11) | 2 | (100) | 0 | - | 1 | (20) |
| Stabilized, n (%) | 9 | (69) | 5 | (56) | 0 | - | 2 | (100) | 4 | (80) |
| Declined, N (%) | 1 | (8) | 3 | (33) | 0 | - | 0 | - | 0 | - |
| Follow up, months | 37 | [12–96] | 17 | [13–50] | 113 | [9–218] | 65 | [10–120] | 170 | [81–182] |
| Deaths, n (%) | 1 | (8) | 0 | 0 | 0 | 0 | ||||
Lung function outcomes based upon ±1% per month change over baseline values at original diagnosis.
UIP, usual interstitial pneumonia; NSIP, non-specific interstitial pneumonia; DIP, desquamative interstitial pneumonia; CPFE, combined pulmonary fibrosis and emphysema; SS, Sjögren's Syndrome; ILD, interstitial lung disease; ESSDAI, EULAR Sjögren's Syndrome Disease Activity Index; FVC, forced vital capacity; DLCO, Carbon monoxide lung diffusion capacity; CYC, cyclophosphamide; AZA, azathioprine; MTX, methotrexate; HCQ, hydroxychloroquine; RTX, rituximab; MMF, mycophenolate mofetil.
Values represent median [inter-quartile range] unless otherwise stated.