Background
Methods
Study subjects and diagnostic criteria
Clinical characteristics
Pulmonary function tests
Analysis of subsets of lymphocytes from bronchoalveolar lavage fluid (BALF)
High-resolution CT scanning
Treatment and follow-up
Statistical analysis
Results and discussion
Comparison of clinical manifestations among the CTD-NSIP, i-NSIP-Ab + and i-NSIP-Ab- groups
SAD-NSIP group | i-NSIP-Ab + group | i-NSIP-Ab-group | P | |
---|---|---|---|---|
N (%) | N (%) | N (%) | ||
Patient’s number | ||||
Initial presentation | 23(23.7) | 30(30.9) | 44(45.4) | 0.050 |
After follow up | 28(28.9) | 31(32.0) | 38(39.1) | 0.306 |
Age (years), Mean ± SD | ||||
Initial presentation | 49.4 ± 10.6 | 45.7 ± 10.0 | 49.5 ± 12.5 | 0.307 |
After follow up | 50.4 ± 9.9 | 46.3 ± 10.6 | 48.5 ± 12.8 | 0.386 |
Male | ||||
Initial presentation | 6/23(26.1) | 6/30(20.0) | 15/44(34.1) | 0.405 |
After follow up | 8/28(28.6) | 7/31(22.6) | 13/38(34.2) | 0.569 |
Duration (months), Mean ± SD | ||||
Initial presentation | 16.7 ± 27.6 | 10.2 ± 13.8 | 10.9 ± 15.4 | 0.378 |
After follow up | 14.9 ± 25.3 | 9.8 ± 13.8 | 11.8 ± 16.3 | 0.575 |
Follow-up time (months), Mean ± SD | ||||
Initial presentation | 48.4 ± 28.2 | 58.4 ± 32.7 | 54.9 ± 38.5 | 0.504 |
After follow up | 51.9 ± 29.8 | 58.3 ± 32.5 | 55.2 ± 38.9 | 0.745 |
Smoking, | ||||
Initial presentation | 2/23(8.7) | 2/30(6.7) | 4/44(9.1) | 0.944 |
After follow up | 2/28(7.1) | 2/31(6.5) | 4/38(10.5) | 0.803 |
SAD-NSIP | i-NSIP-Ab+ | i-NSIP-Ab- | P | |
---|---|---|---|---|
N (%) | N (%) | N (%) | ||
Cough, initial presentation | 14/23(60.9) | 21/30(70.0) | 22/44(50.0) | 0.150 |
After follow up | 17/28(60.7) | 21/31(67.7) | 19/38(50.0) | 0.215 |
Dyspnea, initial presentation | 13/23(56.5) | 21/30(70.0) | 34/44(77.3) | 0.334 |
After follow up | 17/28(60.7) | 22/31(71.0) | 29/38(76.3) | 0.389 |
Fever, initial presentation | 5/23(21.7) | 10/30(33.3) | 10/44(22.7) | 0.475 |
After follow up | 9/28(36.0) | 9/31(29.0) | 7/38(18.4) | 0.399 |
Weight loss, initial presentation | 1/23(4.3) | 7/30(23.3) | 8/44(18.2) | 0.168 |
After follow up | 3/28(10.7) | 8/31(25.8) | 5/38(13.2) | 0.230 |
Arthralgia, initial presentation | 5/23(21.7) | 5/30(16.7) | 6/44(13.6) | 0.697 |
After follow up | 7/28(25.0) | 6/31(19.4) | 3/38(7.9) | 0.158 |
Raynaud’s phenomenon, initial presentation | 3/23(13.0) | 3/30(10) | 0 | 0.063 |
After follow up | 3/28(10.7) | 3/31(9.7) | 0 | 0.126 |
Dry eyes or dry mouth, initial presentation | 3/23(13.0) | 1/30(3.3) | 3(6.8) | 0.396 |
After follow up | 3/28(10.7) | 1/31(3.2) | 3(6.5) | 0.529 |
Oral ulcer, initial presentation | 4/23(17.4) | 2/30(6.7) | 1/44(2.3) | 0.075 |
After follow up | 4/28(14.3) | 2/31(6.5) | 1/38(2.6) | 0.191 |
Skin rash, initial presentation | 8/23(34.8) | 12/30(40.0) | 1/44(2.3) | 0.000* |
After follow up | 8/28(28.6) | 13/31(41.9) | 0 | 0.000* |
Proximal muscle weakness, initial presentation | 3/23(13.0) | 0 | 2/44(4.5) | 0.101 |
After follow up | 3/28(10.7) | 0 | 2/38(5.3) | 0.178 |
Morning stiffness, initial presentation | 2/23(10.7) | 0 | 0 | 0.037** |
After follow up | 2/28(7.1) | 0 | 0 | 0.081 |
Gastroesophageal reflux, initial presentation | 2/23(8.7) | 0 | 0 | 0.037** |
After follow up | 2/28(7.1) | 0 | 0 | 0.081 |
Photosensitivity, initial presentation | 0 | 0 | 1/44(2.3) | 0.544 |
After follow up | 0 | 0 | 1/38(2.6) | 0.456 |
Crackles, initial presentation | 17/23(73.9) | 16/30(53.3) | 28/44(63.6) | 0.173 |
After follow up | 18/28(64.3) | 16/31(51.6) | 27/38(71.0) | 0.343 |
Clubbing, initial presentation | 2/23(8.7) | 3/30(10.0) | 1/44(2.3) | 0.339 |
After follow up | 2/28(7.1) | 3/31(9.7) | 1/38(2.6) | 0.467 |
SAD-NSIP group | i-NSIP-Ab + group | i-NSIP-Ab-group | p | |
---|---|---|---|---|
ABG | ||||
PaO2 (mmHg), initial presentation | 74.1 ± 8.9 | 74.2 ± 10.8 | 74.2 ± 10.8 | 0.648 |
After follow up | 73.0 ± 10.7 | 73.2 ± 11.8 | 73.9 ± 10.6 | 0.934 |
PaCO2(mmHg), initial presentation | 36.8 ± 4.9 | 35.3 ± 4.2 | 35.7 ± 4.5 | 0.478 |
After follow up | 37.1 ± 4.9 | 35.4 ± 4.2 | 35.2 ± 4.1 | 0.206 |
PFT | ||||
FVC (%), initial presentation | 70.1 ± 16.3 | 72.3 ± 13.5 | 74.5 ± 12.3 | 0.789 |
After follow up | 68.9 ± 14.9 | 74.1 ± 14.7 | 75.7 ± 14.4 | 0.664 |
TLC (%), initial presentation | 75.1 ± 12.4 | 74.1 ± 13.2 | 73.4 ± 12.1 | 0.912 |
After follow up | 73.4 ± 11.7 | 74.4 ± 14.9 | 76.0 ± 17.6 | 0.847 |
DLCO (%), initial presentation | 55.1 ± 10.9 | 53.4 ± 16.2 | 52.1 ± 15.2 | 0.198 |
After follow up | 51.4 ± 11.0 | 54.2 ± 18.0 | 56.8 ± 16.6 | 0.173 |
BALF | ||||
M (%), initial presentation | 42.9 ± 21.7 | 43.8 ± 22.6 | 45.6 ± 23.7 | 0.397 |
After follow up | 46.9 ± 24.7 | 44.0 ± 21.7 | 43.6 ± 24.3 | 0.425 |
L (%), initial presentation | 39.2 ± 20.9 | 38.9 ± 22.4 | 41.3 ± 23.5 | 0.876 |
After follow up | 37.1 ± 22.8 | 38.4 ± 20.7 | 44.8 ± 25.0 | 0.776 |
N (%), initial presentation | 10.1 ± 18.2 | 13.6 ± 14.2 | 12.1 ± 11.9 | 0.671 |
After follow up | 11.9 ± 19.6 | 13.2 ± 15.7 | 10.5 ± 13.1 | 0.807 |
E (%), initial presentation | 5.0 ± 8.8 | 5.2 ± 6.0 | 4.1 ± 5.0 | 0.801 |
After follow up | 5.3 ± 8.2 | 4.8 ± 5.7 | 3.9 ± 4.6 | 0.752 |
CD4/CD8, initial presentation | 1.5 ± 1.9 | 1.3 ± 1.3 | 1.2 ± 0.9 | 0.813 |
After follow up | 1.2 ± 1.8 | 1.2 ± 1.1 | 1.4 ± 1.5 | 0.947 |
Radiologic and pathologic findings in CTD-NSIP, i-NSIP-Ab + and i-NSIP-Ab- groups
SAD-NSIP | i-NSIP-Ab+ | i-NSIP-Ab- | P | |
---|---|---|---|---|
n (%) | n (%) | n (%) | ||
Baseline Chest CT Findings
| ||||
Ground glass opacity, initial presentation | 17/23(73.9) | 20/30(66.7) | 25/44(56.8) | 0.358 |
After follow up | 19/28(67.9) | 21/31(67.7) | 23/38(60.5) | 0.765 |
Patchy opacity, initial presentation | 9/23(39.1) | 10/30(33.3) | 12/44(27.2) | 0.602 |
After follow up | 10/28(35.7) | 11/31(35.5) | 10/38(26.3) | 0.633 |
Reticular opacity, initial presentation | 18/23(78.2) | 19/30(63.3) | 27/44(61.4) | 0.358 |
After follow up | 22/28(78.6) | 20/31(64.5) | 22/38(57.9) | 0.211 |
Traction bronchiectasis, initial presentation | 9/23(39.1) | 6/30(20.0) | 8/44(18.2) | 0.167 |
After follow up | 10/28(35.7) | 6/31(19.4) | 7/38(18.4) | 0.208 |
Pathological pattern
| ||||
Cellular pattern, initial presentation | 12/23(52.2) | 15/30(50.0) | 22/44(50.0) | 0.984 |
After follow up | 15/28(53.6) | 14/31(45.2) | 20/38(52.6) | 0.768 |
Mixed pattern, initial presentation | 8/23(34.8) | 13/30(43.3) | 17/44(38.6) | 0.815 |
After follow up | 10/28(35.7) | 14/31(45.2) | 14/38(36.8) | 0.707 |
Fibrotic pattern, initial presentation | 3/23(13.0) | 2/30(6.7) | 5/44(11.4) | 0.716 |
After follow up | 3/28(10.7) | 4/31(12.9) | 3/38(7.9) | 0.791 |
Treatment and survival
SAD-NSIP group | i-NSIP-Ab + group | i-NSIP-Ab-group | |
---|---|---|---|
Initial therapy | |||
Prednisone, N (%) | 9/23(39.1) | 30/30(100) | 44/44(100) |
Prednisone + immunosuppressive agent, N (%) | 14/23(60.9) | 0 | 0 |
Relapse, N (%) | 6/23(26.1) | 13/30(4.3) | 21/44(47.7) |
Emerging autoantibody, N (%) | 0 | 0 | 3/44(6.8) |
classifiable SAD, N (%) | 0 | 2/30(6.7) | 3/44(6.8) |
PAH, N (%) | 1/23(4.3) | 1/30(3.3) | 1/44(2.3) |
Lung infection, N (%) | 1/23(4.3) | 3/30(10.0) | 4/44(9.1) |
Death, N (%) | 8/23(34.8) | 8/30(26.7) | 9/44(20.5) |
Underlying disease, N (%) | 4/23(17.4) | 6/30(20.0) | 6/44(13.6) |
Lung infection, N (%) | 3/23(13.0) | 2/30(6.7) | 2/44(4.5) |
Other, N (%) | 1/23(4.3) | 0 | 1/44(2.3) |