Table 1.
Comparison of demographics and disease states among AAV diseases
|
EGPA |
GPA |
MPA/RLV |
Unclassifiable |
|
|---|---|---|---|---|
| (n = 14) | (n = 33) | (n = 78) | (n = 31) | |
| Male/female |
5/9 |
12/21 |
35/43 |
9/22 |
| Mean (median) age (years)a,c |
58.0 ± 16.9 (62) |
63.6 ± 12.6 (61) |
71.1 ± 10.0 (73) |
70.6 ± 11.8 (73) |
| MPO-ANCAa,c |
7 (50.0) |
18 (54.6) |
76 (97.4) |
29 (93.5) |
| PR3-ANCAb,c |
0 (0) |
15 (45.5) |
2 (2.6) |
1 (3.2) |
| ANCA-negativea,b |
7 (50.0) |
3 (9.1) |
1 (1.3) |
2 (6.5) |
| Serum creatinine (mg/dl)a |
0.71 ± 0.39 |
1.51 ± 1.32 |
2.46 ± 2.18 |
0.69 ± 0.23 |
| Disease severityc | ||||
| Localised |
0 (0) |
4 (12.1) |
0 (0) |
0 (0) |
| Early systemic |
1 (7.1) |
5 (15.1) |
15 (19.2) |
15 (48.4) |
| Generalised |
13 (92.9) |
18 (54.6) |
47 (60.3) |
13 (41.9) |
| Severe |
0 (0) |
6 (18.2) |
16 (20.5) |
3 (9.7) |
| General performanced | ||||
| 0/1/2/3/4 |
1/7/2/4/0 |
8/11/2/11/1 |
10/29/17/16/6 |
3/11/7/9/1 |
| Nongranulomatous pulmonary involvement | ||||
| Interstitial lung diseasea |
2 (14.3) |
3 (9.0) |
37 (47.4) |
19 (61.3) |
| Alveolar haemorrhage | 0 (0) | 2 (6.1) | 9 (11.5) | 2 (6.5) |
Values expressed as mean ± standard deviation or number (percentage) unless otherwise noted. AAV, antineutrophil cytoplasmic antibody-associated vasculitis; ANCA, antineutrophil cytoplasmic antibody; EGPA, eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome); GPA, granulomatosis with polyangiitis (Wegener’s granulomatosis); MPA, microscopic polyangiitis; MPO, myeloperoxidase; PR3, proteinase-3; RLV, renal-limited vasculitis. Comparisons between the EGPA, GPA, and MPV/RLV groups were made by Student t test or Mann–Whitney U test. Statistical significance was determined by P < 0.05/3 using Bonferroni correction: aEGPA versus MPA/RLV, bEGPA versus GPA, cGPA versus MPA/RLV. Unclassifiable AAV was not compared with other forms of AAV. dGeneral performance was categorised according to the World Health Organization performance status except category 5 (death).