Clinical and laboratory findings for group A.
| Case | Global Sclerosis, % | Vasculitis | Organ Involvement (extrarenal) | Initial Therapy | Maintenance Therapy | Disease Duration, mos | Relapse | End of Followup |
|---|---|---|---|---|---|---|---|---|
| 1 | 0 | Interlobular | ILD | PSL CYC | PSL | 35 | 1 | Cr 1.0 |
| 2 | 0 | Interlobular | (–) | PSL CYC | 2 | 0 | Death (renal failure) | |
| 3 | 11 | Arteriole Interlobular | (–) | mPSLpulse PSL, CYC | PSL | 39 | 2 | Cr 1.7 |
| 4 | 20 | Arteriole Interlobular | ILD | mPSLpulse PSL | PSL | 25 | 0 | Cr 2.9 |
| 5 | 8 | Interlobular Arcuate | ILD | mPSLpulse PSL | PSL | 20 | 0 | Cr 1.1 |
| 6 | 4 | Interlobular | Pulmonary hemorrhage | mPSLpulse PSL | PSL | 19 | 0 | Cr 2.1 |
| Intestinal hemorrhage | PE | |||||||
| 7 | 40 | Arteriole Interlobular | ILD | PSL | PSL | 163 | 0 | Death (intestinal perforation) |
| 8 | 10 | Interlobular | Pulmonary tumor Neuritis | mPSLpulse PSL, CYC | PSL | 50 | 3 | Hemodialysis |
| 9 | 12 | Interlobular, Arteriole | ILD Neuritis | mPSLpulse PSL, CYC | PSL MZB | 60 | 1 | Cr 1.8 |
| 10 | 45 | Interlobular Arcuate | Pulmonary tumor Celebral infarction Neuritis Intestinal hemorrhage | mPSLpulse PSL, CYC | PSL | 6 | 0 | Cr 2.6 |
GPA was diagnosed by the following findings: Case 7 : histological granulomatous inflammation of the renal interstitium; Case 8: pulmonary nodules; Case 9: histological granulomatous inflammation in the lungs; Case 10: chronic sinusitis and pulmonary nodules. Hematuria: grade 1, < 1/high power field (HPF); grade 2, 1 to 5/HPF; grade 3, 6 to 10/HPF; grade 4, 11 to 30/HPF; grade 5, > 30/HPF. Crescents: the percentage of glomeruli with crescents. ILD: interstitial lung disease; PSL: prednisonone; mPLSpulse: intravenous methylprednisolone pulse therapy; CYC: cyclophosphamide; PE: plasma exchange; MZB: mizorbine; ANCA: antineutrophil cytoplasmic antibody; GPA: granulomatosis with polyangiitis; CRP: C-reactive protein; Cr: creatinine; MPO: myeloperoxidase; MPA: microscopic polyangiitis; PR3: proteinase 3.