Characteristics of AAV patients at diagnosis and during follow-up (n=101)
| AAV patients | Values |
|---|---|
| At the time of diagnosis | |
| Demographic data | |
| Age (yr) | 62.0 (21.0) |
| Male sex | 34 (33.7) |
| AAV subtypes | |
| MPA | 56 (55.4) |
| GPA | 28 (27.7) |
| EGPA | 17 (16.8) |
| ANCA positivity | |
| MPO-ANCA (or P-ANCA) positivity | 73 (72.3) |
| PR3-ANCA (or C-ANCA) positivity | 18 (17.8) |
| Both ANCA positivity | 5 (5.0) |
| ANCA negativity | 15 (14.9) |
| AAV-specific indices | |
| BVAS | 12.0 (11.0) |
| FFS | 1.0 (1.0) |
| Clinical manifestations at diagnosis | |
| General | 40 (39.6) |
| Cutaneous | 15 (14.9) |
| Muco-membranous /Ocular | 4 (4.0) |
| Ear nose throat | 48 (47.5) |
| Pulmonary | 67 (66.3) |
| Cardiovascular | 11 (10.9) |
| Gastrointestinal | 4 (4.0) |
| Renal | 61 (60.4) |
| Nervous | 25 (24.8) |
| Routine laboratory results | |
| Urine protein creatinine ratio | 1.42 (2.69) |
| Proteinuria | 42 (41.5) |
| Hematuria | 49 (48.5) |
| White blood cell count (/mm3) | 8,910.0 (5,857.5) |
| Haemoglobin (g/dL) | 11.3 (3.5) |
| Platelet count (×1,000/mm3) | 307.5 (178.0) |
| Fasting glucose (mg/dL) | 103.0 (28.5) |
| Blood urea nitrogen (mg/dL) | 17.9 (22.8) |
| Serum creatinine (mg/dL) | 0.9 (1.8) |
| Total protein (g/dL) | 6.8 (1.2) |
| Serum albumin (g/dL) | 3.7 (1.0) |
| Alkaline phosphatase (IU/L) | 74.0 (35.8) |
| Aspartate aminotransferase (IU/L) | 18.0 (7.0) |
| Alanine aminotransferase (IU/L) | 15.0 (13.3) |
| Total bilirubin (mg/dL) | 0.5 (0.4) |
| ESR (mm/hr) | 63.5 (68.3) |
| CRP (mg/L) | 8.0 (69.1) |
| Complement | |
| CH50 (U/mL) | 60.4 (17.9) |
| C3 (mg/dL) | 113.0 (37.6) |
| C4 (mg/dL) | 25.6 (12.4) |
| During the follow-up period | |
| Follow-up duration (mo) | 26.4 (30.4) |
| Poor outcomes during follow-up | |
| All-cause mortality | 9 (8.9) |
| Duration until all-cause mortality (mo) | 26.4 (30.4) |
| Relapse | 31 (30.7) |
| Duration until relapse (mo) | 20.9 (25.5) |
| ESRD | 20 (19.8) |
| Duration until ESRD (mo) | 20.8 (28.3) |
| Medications administered during the follow-up period | |
| Glucocorticoid | 96 (95) |
| Cyclophosphamide | 52 (51.5) |
| Rituximab | 19 (18.8) |
| Azathioprine | 61 (60.4) |
| Mycophenolate mofetil | 13 (12.9) |
| Tacrolimus | 6 (5.9) |
| Methotrexate | 9 (8.9) |
Values are expressed as the median (interquartile range) or number (%). AAV: ANCA-associated vasculitis, ANCA: antineutrophil cytoplasmic antibody, MPA: microscopic polyangiitis, GPA: granulomatosis with polyangiitis, EGPA: eosinophilic GPA, MPO: myeloperoxidase, P: perinuclear, PR3: proteinase 3, C: cytoplasmic, BVAS: Birmingham Vasculitis Activity Score, FFS: Five-Factor score, ESR: erythrocyte sedimentation rate, CRP: C-reactive protein, CH50: total haemolytic complement activity, C3: complement 3, C4: complement 4, ESRD: end-stage renal disease. *Median (interquartile range).