Activity stages of ANCA-associated vasculitides (AAV) – EUVAS definition.
| Activity stage | Definition |
| Localized stage | Upper and/or lower respiratory tract without systemic manifestation, without B symptoms, not organ-threatening1 |
| Early systemic stage | All organ involvement possible, not life-threatening or organ-threatening2 |
| Generalization stage | Renal involvement (kidney involvement) or other organ-threatening manifestation (serum creatinine < 500 µmol/l (5.6 mg/dl))3 |
| Severe, vital-threatening generalization stage | Renal failure or other organ failure (creatinine > 500 µmol/l (5.6 mg/dl))3 |
| Refractory stage | Progressive disease, refractory to standard therapy (glucocorticoids, cyclophosphamide) |
Legend1ANCA often negative2ANCA negative or positive3ANCA almost always positive.
B symptomatology
- Unexplained, persistent, or recurrent fever (>38°C).
- Night sweats (wet hair, soaked sleepwear).
- Unwanted weight loss (> 10% percent of body weight within 6 months).
Granulomatosis with polyangiitis (GPA), formerly Wegener’s granulomatosis, can be classified according to the ACR criteria* :
- Inflammation in the nose or mouth with ulcers (ulcerations) and purulent (purulent) nasal secretions
- On chest x-ray: nodules, infiltrations, or evidence of caverns (evidence of “cavities”)
- Pathological (pathological) urine sediment with microhematuria ((presence of blood in the urine (hematuria), which can be detected microscopically or by means of test strips (Sangur test )) or erythrocyte cylinder.
- Bioptically (affecting the tissue sample) detected granulomatous inflammation in arterial vessel walls or perivascular
A diagnosis of granulomatosis with polyangiitis can be made if 2 of the 4 criteria are present.
* American College of Rheumatology (ACR)
