Study | Country | Study design | Subjects (n) (M/F) | Diagnosis of systemic autoimmune disease (n) | Age (years) (at the onset of AE) | Smoking (n (%)) | UIP pattern (n (%))a | Follow-up lengths (months) | Frequency of AE (n (%))/Incidence | All-cause mortality (n (%)) |
---|---|---|---|---|---|---|---|---|---|---|
Isobe [41] | Japan | Case–control | 2 (2/0) | RA2 | 74.0 ± 7.1 | – | 2 (100.0) | – | 2/8 (25.0) among RA-ILD | 2/2 (100.0) (overall) |
Lim [42] | Korea | Retrospective-cohort | 25 | Not specified | – | – | – | – | 25/76 (32.9) | – |
Park [43] | Korea | Retrospective-cohort | 4 (3/1) | RA3, SSc1 | median 58 (range 47–68) | 2 (50.0) | 3 (75.0) | – | 4/93 (4.3) among systemic autoimmune disease-ILDg | 4/4 (100.0) (overall) |
Song [44] | Korea | Retrospective-cohort | 14 (8/6) | RA14 | 68.1 ± 7.8d | 7 (50.0) | 14 (100.0) | – | 14/84 (16.7) among RA-ILD | 13/14 (92.9) (overall) |
Suzuki [45] | Japan | Retrospective-cohort | 27 | RA7, SSc11, PM/DM4, SS2, SLE2, MCTD1 | – | – | – | – | 27/205 (13.2) among systemic autoimmune disease-ILDc/3.19 per 100 patient-years, | – |
Tachikawa [46] | Japan | Retrospective-cohort | 15 (7/8) | RA6, SSc3, DM3, CADM3 | 63.3 ± 6.8 | 8 (57.1) (n = 14) | 4 (26.7) | – | – | (33.0) (90 days) |
Tomiyama [47] | Japan | Retrospective-cohort | 13 (3/10) | SSc13 | 49.3 ± 12.7 (at the onset of SSc) | – | – | – | 13/55 (23.6) among SSc-ILD | 6/13 (46.2) (overall) |
Akiyama [48] | Japan | Retrospective-cohort | 6 (2/4) | RA6 | 66.8 ± 6.2 (at the start of treatment) | 2 (40.0) (n = 5) | 3 (50.0) | – | 6/78 (7.7) among RA-ILD | – |
Cao [49] | China | Retrospective-cohort | 70 (35/35) | RA16, PM/DM8, SS25, MCTD6, ANCA-vasculitis5, IPAF10 | 65.8 ± 9.3d | 18 (25.7) | 70 (100.0) | – | 70/1168 (6.0) among systemic autoimmune disease-ILDg | – |
Enomoto [50] | Japan | Retrospective-cohort | 15 (11/4) | RA9, SSc2, MPA3, MPA + SS1 | median 71 (range 57–85) | 13 (86.7) | – | Median 56 (range 0–228)e | – | 5/15 (33.3) (1 month) |
 |  |  |  |  |  |  |  |  |  | 7/15 (46.7) (3 months) |
 |  |  |  |  |  |  |  |  |  | 11/15 (73.3) (overall) |
Hozumi [51] | Japan | Retrospective-cohort | 11 (6/5) | RA11 | median 72 (range 60–86) | 9 (81.8) | 6 (54.5) | Median 8.5 (range 1–17) (years)f | 11/51 (21.6) among RA-ILD | 7/11 (63.6) (overall) |
Ichiyasu [52] | Japan | Retrospective-cohort | 38 | RA6, SSc1, PM5, DM17, SS1, SLE1, MCTD1, MPA6 | – | – | – | – | – | (58.3) (90 days) |
Ishikawa [53] | Japan | Case–control | 9 (3/6) | Not specified | 56.4 ± 15.6 | – | – | – | – | 4/9 (44.4) (3 months) |
Liang [54] | China | Case–control | 64 (25/39) | PM15, DM36, CADM13 | 57.7 ± 11.9d | 14 (21.9) | 15 (23.4) | – | 64/483 (13.3) among IIM-ILD | 25/64 (39.1) (in-hospital or within 2 weeks after discharge) |
Manfredi [55] | Italy | Prospective-cohort | 9 (3/6) | RA1, SSc1, SS1, MCTD1, SSc + DM1, RA + SS1, ANCA-vasculitis1, IPAF2 | 67.8 ± 8.5 (at the diagnosis of ILD) | 1 (11.1) | 6 (66.7) | 23.9 ± 10.9f | 9/78 (11.5) among systemic autoimmune disease-ILDg/5.77 per 100 patient-years | 5/9 (55.6) (overall) |
Okamoto [56] | Japan | Retrospective-cohort | 4 | SSc4 | – |  | 4 (100.0) | – | 4/33 (12.1) among SSc-ILD | 4/4 (100.0) (overall) |
Ota [57] | Japan | Retrospective-cohort | 12 (4/8) | RA12 | median 74.5 (range 50–80) | 4 (33.3) | 10 (83.3) | Median 19.5 (range 9–88)e | – | 2/12 (16.7) (overall) |
Parambil [58] | USA | Case–control | 6 (2/4) | RA4, PM2 | medina 68 (range 43–76) | 3 (50.0) | 2 (33.3) | – | – | 5/6 (83.3) (in-hospital) |
Silva [59] | Canada | Case–control | 8 (2/6) | RA3, PM/DM4, SS1 | median 62 (range 53–72) | – | 5 (62.5) | – | – | 6/8 (75.0) (overall) |
Singh [60] | India | Prospective-cohort | 15 (5/10) | RA1, SSc5, DM3, SS3, MCTD3 | 45.8 ± 13.9 | 4 (26.7) | 2 (13.3) | 24 ± 18.1f | 15/105 (14.3) among systemic autoimmune disease-ILDb | 5/15 (33.3) (in-hospital) |
Su [61] | China | Retrospective-cohort | 26 | Not specified | – | – | – | – | 26/161 (16.1) among systemic autoimmune disease-ILDg | 16/26 (61.5) (1 year) |
Suda [62] | Japan | Retrospective-cohort | 6 (4/2) | RA5, SS1 | 65.7 ± 5.3 | 5 (83.3) | 3 (50.0) | Mean 6.0 (years)f | 6/83 (7.2) among systemic autoimmune disease-ILDg | 5/6 (83.3) (overall) |
Toyoda [63] | Japan | Retrospective-cohort | 10 (7/3) | RA6, PM/DM2, SLE1, SS1 | median 73 (range 61–83) | 6 (60.0) | 8 (80.0) | – | 10/155 (6.5) among systemic autoimmune disease-ILDg | 1/10 (10.0) (30 days) |
 |  |  |  |  |  |  |  |  |  | 3/10 (30.0) (90 days) |
 |  |  |  |  |  |  |  |  |  | 7/10 (70.0) (overall) |
Yamakawa[64] | Japan | Retrospective-cohort | 11 | RA11 | – | – | 3 (27.3) | – | 11/96 (11.5) among RA-ILD | – |