Table 5.

Assessment of extension and severity of AAV (including GPA).

WGET Research Group 2002	EUVAS 2009	FVSG (FFS) 2009	ACR 2021		EULAR 2022
			Severe disease	Non-severe disease	Disease with organ/life-threatening manifestations	Disease without organ/life-threatening manifestations
1.Limited -disease which do not pose an immediate threat to either a critical organ or to the patient’s life, specifically: - There are not RBC casts in the urine - The serum Cr must be ≤1.4 mg/dL and the rise in serum Cr cannot be greater than 25% above the patient’s baseline - Lug involvement must be circumscribed (the room air Pa02 > 70 mmHg or air 02 saturation by pulse oximetry >90%) - No disease may exist within any other critical organ (e.g., GI, eyes, CNS) 2. Severe – disease which is not classifiable as limited	1. Localized – limited to the upper and/or lower respiratory tract 2. Early systemic-any, without organ-threatening disease 3. Generalised-renal or other organ-threatening disease, Cr < 500 μmol/L (5.6 mg/dL) 4. Severe – renal or other vital organ failure, Cr > 500 μmol/L (5.6 mg/dL) 5. Refractory – progressive disease unresponsive to treatment	1.Age > 65 years 2.Cardiac insufficiency 3.Renal insufficiency (stabilized peak Cr ≥ 150 μmol/L) 4.GI 5.Absence of ENT FFS = 0 non-severe disease FFS ≥ 1 severe disease Mortality rates: FFS = 0 9% FFS = 1 21% FFS ≥ 2 40%	Vasculitis with life- or organ-threatening manifestations, e.g., DAH, glomerulonephritis, CNS involvement, mononeuritis multiplex, cardiac involvement, mesenteric ischemia, limb/digit ischemia	Vasculitis without life- or organ-threatening manifestations, e.g., rhinosinusitis, mild systemic symptoms, uncomplicated cutaneous disease, mild inflammatory arthritis	DAH Glomerulonephritis Meningeal involvement CNS involvement Retro-orbital disease Cardiac involvement Mesenteric involvement Mononeuritis multiplex	Nasal and paranasal disease (without bony erosion, cartilage collapse or olfactory dysfunction or deafness) Skin involvement (without ulceration) Myositis (skeletal muscle only) Non-cavitating lung nodules Episcleritis
					There are only examples (many other symptoms of AAV exist); assessment of severity may differ in the individual patients

FFS, five-factor score; GPA, granulomatosis with polyangiitis; GI, gastrointestinal involvement; Cr, creatinine; RBC, red blood cells; CNS, central nervous system; ENT, ear, nose, throat; DAH, diffuse alveolar haemorrhage; ACR, American College of Rheumatology; FVSG, French vasculitis study group; EULAR, European league against rheumatism; EUVAS, European vasculitis study group; WGET, Wegener’s granulomatosis etanercept trial.