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Table 1 Characteristics of included studies

From: A systematic review of the incidence, risk factors and prognosis of acute exacerbation of systemic autoimmune disease-associated interstitial lung disease

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

–

  1. Values indicate mean ± SD or number (percentage) unless otherwise specified
  2. AE, acute exacerbation; ANCA, anti-neutrophil cytoplasmic antibody; CADM, clinically amyopathic dermatomyositis; DM, dermatomyositis; IIM, idiopathic inflammatory myositis; ILD, interstitial lung disease; IPAF, interstitial pneumonia with autoimmune features; IQR, interquartile range; MCTD, mixed connective tissue disease; MPA, microscopic polyangiitis; PM/DM, polymyositis/dermatomyositis; RA, rheumatoid arthritis; SLE, systemic lupus erythematosus; SS, Sjögren’s syndrome; SSc, systemic sclerosis; UIP, usual interstitial pneumonia
  3. aIndicating radiological and/or pathological UIP pattern
  4. bSSc 5/30 (16.7%), MCTD 3/28 (10.7%), RA 1/16 (6.3%), DM 3/9 (33.3%), SS 3/6 (50.0%), others 0/16 (0.0%)
  5. cSSc 11/42 (26.2%), RA 7/71 (9.9%), PM/DM 4/42 (9.5%), SLE 2/5 (40.0%), SS 2/24 (8.3%), MCTD 1/6 (16.7%), others 0/15 (0.0%)
  6. dUnknown point in time
  7. eAfter the development of acute exacerbation
  8. fBefore the development of acute exacerbation
  9. gUnclear underlying systemic autoimmune disease