|  | Specicifc antibodies | ||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|
Total | SScAb positive IPAF | Anti-CCP positive IPAF | ARS Ab positive IPAF | Anti-SSB positive IPAF | Other IPAF | |||||||
Number of patients, n | 98 | 28 | 13 | 9 | 4 | 44 | ||||||
HRCT pattern | Â | Â | Â | Â | Â | Â | Â | Â | Â | Â | Â | Â |
 OP, n (%) | 20 | (20.4%) | 3 | (10.7%) | 3 | (23.1%) | 2 | (22.2%) | 1 | (25%) | 11 | (23.4%) |
 NSIP + OP, n (%) | 15 | (15.3%) | 1 | (3.6%) | 1 | (7.7%) | 4 | (44.4%) | 2 | (50%) | 7 | (14.9%) |
 NSIP, n (%) | 63 | (64.3%) | 24 | (85.7%) | 9 | (69.2%) | 3 | (33.3%) | 1 | (25%) | 26 | (55.3%) |
5-year survival,% | 71.1% | 42.1% | 66.7% | 75.8% | 100.0% | 75.8% | ||||||
Treatment,n (%) | 79 | (80.6%) | 23 | (82.1%) | 10 | (76.9%) | 7 | (77.8%) | 4 | (100%) | 34 | (77.2%) |
 corticosteroid, n (%) | 27 | (27.6%) | 5 | (17.9%) | 2 | (15.4%) | 2 | (22.2%) | 3 | (75%) | 15 | (34.1%) |
 corticosteroid + ISa, n (%) | 44 | (44.9%) | 16 | (57.1%) | 6 | (46.2%) | 5 | (55.6%) | 1 | (25%) | 16 | (36.4%) |
 corticosteroid + IS + pirfenidone, n (%) | 1 | (1.0%) | 0 | (0%) | 0 | (0%) | 0 | (0%) | 0 | (0%) | 1 | (2.3%) |
 IS only, n (%) | 5 | (5.1%) | 2 | (7.1%) | 2 | (15.4%) | 0 | (0%) | 0 | (0%) | 0 | (0%) |
 pirfenidone, n (%) | 2 | (2.0%) | 0 | (0%) | 0 | (0%) | 0 | (0%) | 0 | (0%) | 2 | (4.5%) |
Response to Treatment, n (%) | 70 | (71.4%) | 20 | (71.4%) | 9 | (69.2%) | 7 | (77.8%) | 4 | (100%) | 30 | (68.1%) |
 Improve/stable/deteriorate, n | 44/20/6 | 7/11/2 | 5/3/1 | 5/1/1 | 4/0/0 | 23/5/2 | ||||||
Overall death, n (%) | 27 | (27.6%) | 13 | (46.4%) | 3 | (23.1%) | 1 | (11.1%) | 0 | (0%) | 10 | (22.7%) |
 Death due to respiratory failure,n (%) | 15 | (15.3%) | 8 | (28.6%) | 1 | (7.7%) | 0 | (0%) | 0 | (0%) | 6 | (13.6%) |
 Acute exacerbation, n (%) | 5 | (5.1%) | 2 | (7.1%) | 0 | (0%) | 0 | (0%) | 0 | (0%) | 3 | (6.8%) |