HR of death and subdistribution HR (SHR) of relapse in patients with AAV stratified by clinical diagnosis and across subgroups identified by cluster analyses
| Variable | No. | Risk of death | Risk of relapse | ||
|---|---|---|---|---|---|
| HR (95% CI) | p Value | SHR (95% CI) | p Value | ||
| Diagnosis | |||||
| GPA | 396 | 1 (reference) | 1 (reference) | ||
| MPA | 277 | 2.44 (1.73 to 3.43) | <0.0001 | 0.35 (0.26 to 0.48) | <0.0001 |
| Cluster model 1 | |||||
| Non-renal AAV | 86 | 1 (reference) | 1 (reference) | ||
| Renal AAV with WEERD | 153 | 2.40 (0.99 to 5.83) | 0.05 | 0.61 (0.42 to 0.90) | 0.01 |
| Renal AAV with LEERD | 327 | 3.31 (1.44 to 7.62) | 0.005 | 0.37 (0.26 to 0.53) | <0.0001 |
| CV AAV | 58 | 5.42 (2.10 to 13.98) | <0.0005 | 0.81 (0.48 to 1.35) | 0.42 |
| GI AAV | 49 | 5.71 (2.21 to 14.72) | <0.0005 | 0.24 (0.11 to 0.50) | 0.0001 |
| Cluster model 2 | |||||
| Non-renal AAV | 84 | 1 (reference) | 1 (reference) | ||
| Renal AAV with PR3-ANCA | 270 | 2.07 (0.81 to 5.27) | 0.13 | 0.58 (0.41 to 0.82) | 0.002 |
| Renal AAV without PR3-ANCA | 212 | 5.87 (2.36 to 14.57) | <0.0005 | 0.26 (0.17 to 0.39) | <0.0001 |
| CV AAV | 58 | 6.41 (2.33 to 17.67) | <0.0005 | 0.78 (0.47 to 1.31) | 0.35 |
| GI AAV | 49 | 6.74 (2.45 to 18.55) | <0.0005 | 0.23 (0.11 to 0.48) | 0.0001 |
Cluster model 1 included nine clinical variables, and cluster model 2 included these variables in addition to ANCA specificity.
AAV, ANCA-associated vasculitis; ANCA, anti-neutrophil cytoplasmic antibody; GPA, Granulomatosis with polyangiitis (Wegener's); LEERD, low-extent extra-renal disease; MPA, microscopic polyangiitis; PR3, proteinase 3; WEERD, wide-extent extra-renal disease.