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Table 1 Clinical characteristics and laboratory findings of patients with different radiological patterns

From: Pulmonary involvement of ANCA-associated vasculitis in adult Chinese patients

Characteristic

Alveolar hemorrhage (n = 47)

Interstitial lung disease (n = 204)

Pulmonary granuloma (n = 54)

Airway involvement (n = 61)

Age, median (IQR) (years)

56 (47–64)

68 (62–75)d,e,f

59 (53–69)

61 (58–68)

Sex (male), no. (%)

21 (44.7)

110 (53.9)

28 (51.9)

24 (39.3)

Ever smoker, no. (%)

13 (27.8)

85 (41.7)

19 (35.2)

17 (27.9)

MPA, no. (%)

36 (76.6)

187 (91.7)

25 (46.3)d,g,h

51 (83.6)

GPA, no. (%)

11 (23.4)

14 (6.9)e

26 (48.1)d,g,h

9 (14.8)

EGPA, no. (%)

0

3 (1.5)

3 (5.6)

1 (1.6)

Time of onset to diagnosis, median (IQR) (months)

1 (0–2)

2 (0–8)

1 (0–4)

1 (0–8)

Follow-up, median (IQR) (months)

39 (17–64)

40 (17–75)

52 (26–72)

42 (19–84)

Comorbidities, no. (%)

    

Cardiovascular eventsa

18 (38.3)

112 (54.9)d

18 (33.3)

24 (39.3)

Malignancy

1 (2.1)

4 (2.0)

0

3 (4.9)

Organ involvement at diagnosis, no. (%)

    

Fever

23 (48.9)

74 (36.3)

22 (40.7)

28 (45.9)

Weight loss (≥ 2 kg)

18 (38.3)

95 (46.6)

24 (44.4)

32 (52.5)

Cutaneous

5 (10.6)

9 (4.4)

5 (9.3)

2 (3.3)

Ear, nose and throat

14 (29.8)

57 (27.9)d

26 (48.1)

24 (39.3)

Eye

6 (12.8)

10 (4.9)d

10 (18.5)

7 (11.5)

Cardiovascular

2 (4.3)

4 (1.2)

1 (1.9)

2 (3.3)

Gastrointestinal

2 (4.3)

3 (1.5)

3 (5.6)

2 (3.3)

Central nervous system

3 (6.4)

5 (2.5)

4 (7.4)

3 (4.9)

Peripheral nervous system

3 (6.4)

34 (16.7)

10 (18.5)

13 (21.3)

Renalb

46 (97.9)

165 (80.9)

44 (81.5)

52 (85.2)

Renal insufficiencyc

20 (42.6)

50 (24.5)

18 (33.3)

21 (34.4)

Laboratory findings at diagnosis

    

MPO, no. (%)

33 (70.2)

179 (87.7)d,e

34 (63.0)

53 (86.9)h

PR3, no. (%)

11 (23.4)

12 (5.9)d,e

17 (31.5)

4 (6.6)h

Double positive, no. (%)

0

9 (4.4)

0

2 (3.3)

ANCA negative, no. (%)

2 (4.3)

3 (1.5)

2 (3.7)

1 (1.6)

Serum creatinine, mean ± SD, μmol/L

424.1 ± 312.9

323.6 ± 265.3

315.1 ± 249.4

398.0 ± 355.7

C-reactive protein, mean ± SD, mg/L

54.4 ± 53.9

45.9 ± 49.7

55.3 ± 52.0

45.1 ± 50.0

BVAS at onset, median (IQR)

20 (17–24)

17 (14–21)d,e

20 (15–25)

20 (14–24)

Five-Factor Score, no. (%)

    

 0

5 (10.6)

60 (29.4)

15 (27.8)

19 (31.1)

 1

9 (19.1)

53 (26.0)

9 (16.7)

17 (27.9)

 ≥ 2

33 (70.2)e,i

91 (44.6)

28 (51.9)

25 (41.0)

Bronchoalveolar lavage results at diagnosis

    

Lymphocytes count, mean ± SD

8.7 ± 8.0

9.4 ± 11.1

10.8 ± 14.8

5.8 ± 7.7

Lymphocytes > 20%, no. (%)

2/18 (11.1)

7/59 (11.9)

2/20 (10.0)

1/15 (6.7)

Neutrophils count, mean ± SD

43.9 ± 32.6

44.3 ± 30.7

32.8 ± 33.1

62.1 ± 31.0 h

Neutrophils > 5%, no. (%)

16/18 (88.9)

55/59 (93.2)

17/20 (85.0)

14/15 (93.3)

Hemosiderin-laden macrophages count, mean ± SD

60.2 ± 26.8

12.7 ± 20.4d,e

37.8 ± 31.7 g,h

5.1 ± 17.7i

Hemosiderin-laden macrophages ≥ 20%, no. (%)

16/18 (88.9)

15/59 (25.4)d,e

12/20 (60)

1/15 (6.7)h,i

Pulmonary function test results at diagnosis

    

Obstruction, no. (%)

1/9 (11.1)

7/66 (10.6)d

9/19 (47.4)

4/12 (33.3)

Restriction, no. (%)

4/9 (44.4)

43/66 (65.2)

7/19 (36.8)

8/12 (66.7)

FEV1/FVC, mean ± SD

74.9 ± 9.3

80.0 ± 8.6d,f

70.1 ± 12.3

66.1 ± 14.0

FEV1% pred, mean ± SD

88.1 ± 27.7

83.4 ± 20.4

79.4 ± 21.8

70.1 ± 24.2

FVC% pred, mean ± SD

98.5 ± 27.3

79.6 ± 23.8e

86.9 ± 23.1

80.8 ± 14.7

TLC% pred, mean ± SD

94.7 ± 25.9

73.7 ± 17.6e

85.4 ± 19.5

79.5 ± 15.5

FEF 25–75% pred, mean ± SD

88.1 ± 4.0

62.8 ± 25.5

38.4 ± 30.3

23.78 ± 11.1f,i

FEF50% pred, mean ± SD

66.3 ± 33.6

75.3 ± 34.5d

50.0 ± 35.8

38.5 ± 21.8f

FEF25% pred, mean ± SD

54.5 ± 22.8

69.8 ± 44.5

45.4 ± 30.9

42.1 ± 18.7

DLCO % pred, mean ± SD

65.1 ± 28.8

51.6 ± 17.1

66.4 ± 21.9

57.3 ± 16.7

Treatment, no. (%)

    

 Induction

    

  Glucocorticoids pulses

32 (68.1)

78 (38.2)e

23 (42.6)

33 (54.1)

  Immunosuppressant

41 (87.2)

147 (72.1)

37 (68.5)

47 (77.0)

   Cyclophosphamide

38 (80.9)

142 (69.6)

35 (64.8)

45 (73.8)

   Rituximab

0

1 (0.5)

1 (1.9)

0

  Plasma exchanges

22 (46.8)

48 (23.5)e

17 (31.5)

18 (29.5)

  Dialysis

21 (44.7)

50 (24.5)e

17 (31.5)

21 (34.4)

 Maintenance

    

  Immunosuppressant

21/21 (100.0)

99/105 (94.3)

36/37 (97.3)

28/32 (87.5)

   Cyclophosphamide

21/21 (100.0)

95/105 (90.5)

34/37 (91.9)

27/32 (84.3)

   Rituximab

4/21 (19.0)

3/105 (2.9)e

5/37 (13.5)

2/32 (6.3)

   Azathioprine

3/21 (14.3)

28/105 (26.7)

9/37 (24.3)

5/32 (15.6)

   Mycophenolate mofetil

0

8/105 (7.6)

2/37 (5.4)

2/32 (6.3)

  1. AAV ANCA-associated vasculitis, AI airway involvement, ANCA antineutrophil cytoplasm antibodies, BVAS Birmingham vasculitis activity scores, DLCO diffusing capacity for carbon monoxide, EGPA eosinophilic granulomatosis with polyangiitis, FEF25-75 forced expiratory flow at 25–75% of FVC, FEF50 forced expiratory flow at 50% of FVC, FEF25 forced expiratory flow at 50% of FVC, FEV1 forced expiratory volume, FVC forced vital capacity, GPA granulomatosis with polyangiitis, IQR interquartile range, MPA microscopic polyangiitis, no number, MPO myeloperoxidase, PR3 proteinase-3, SD standard deviation, TLC total lung capacity, % pred % predicted
  2. aCardiovascular events were defined as the presence of one or more of the following conditions at the time of AAV diagnosis: ischemic heart disease, congestive heart failure, hypertension, stroke, thromboembolism
  3. bRenal involvement was defined as the presence of hematuria (≥ 10 red blood cells per high power field), proteinuria (≥ 500 mg/24 h), rise in serum creatinine > 30%, biopsy-proven glomerulonephritis, or need to initiate renal replacement therapy all attributable to active vasculitis
  4. cRenal insufficiency was defined as serum creatinine level ≥ 500 μmol/L or undergoing dialysis
  5. p < 0.05: dILD versus PG, eILD versus AH, fILD versus AI, gPG versus AH, hPG versus AI, iAH versus AI