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. (%) | Â | Â | Â | Â |
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. (%) | Â | Â | Â | Â |
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 | Â | Â | Â | Â |
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. (%) | Â | Â | Â | Â |
 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 | Â | Â | Â | Â |
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 | Â | Â | Â | Â |
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. (%) | Â | Â | Â | Â |
 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) |