|
| (1) Clinical manifestations |
| (i) rapidly progressive glomerulonephritis |
| (ii) pulmonary hemorrhage or interstitial pneumonia |
| (iii) organ involvement besides kidney and lung: |
| purpura, subcutaneous bleeding, gastrointestinal bleeding, mononeuritis multiplex, and so forth |
|
| (2) Histological findings |
| necrotizing vasculitis in capillaries, venules, or arterioles, with perivascular inflammatory infiltrate |
|
| (3) Laboratory findings |
| (i) positive MPO-ANCA |
| (ii) elevated level of CRP |
| (iii) proteinuria and/or hematuria, or elevated levels of BUN and/or creatinine |
| (iv) chest X-ray findings: infiltration (pulmonary hemorrhage), and/or interstitial pneumonitis |
|
| (4) Diagnosis |
| (i) Definite |
| (a) At least two clinical manifestations with the histological findings |
| (b) At least two clinical manifestations including items (i) or (ii), and positive MPO-ANCA |
| (ii) Probable |
| (a) At least three clinical manifestations |
| (b) One clinical manifestation and positive MPO-ANCA |
|
| (5) Exclusion diseases |
| (i) polyarteritis nodosa |
| (ii) Wegener’s granulomatosis |
| (iii) allergic granulomatous angiits (Churg-Strauss syndrome) |
| (iv) Goodpasture syndrome |
|
