Figure 2.

Frequency of PR3- and MPO-ANCA specificity by a variety of clinical phenotypes (organ groupings are not mutually exclusive)

No Lung and No ENT: Vasculitis in any organ except the lungs and the ENT system.

Lung, no ENT: Vasculitis localized in the lungs but not in the ENT system.

Lung*: Vasculitis localized in the lungs without indicative markers (nodules, or cavities) or histological proof (granulomas) of granulomatous inflammation.

Plus Gastrointestinal (GI): Vasculitis localized at any organ plus involvement of the gastrointestinal tract.

Plus Skin: Vasculitis localized at any organ plus dermal involvement.

Plus Nerves: Vasculitis localized at any organ plus involvement of the nerves.

Any Lung: Any type of pulmonary vasculitis such as pulmonary hemorrhage, infiltrates, nodules, cavities, granulomas, or respiratory arrest.

ENT, no Lung: Vasculitis localized at the ENT system but not in the lungs.

Any ENT: Any type of vasculitic manifestation of the ENT system.

Lung with nodules: Vasculitis localized at the lungs with radiographic proof of nodules.

Lung plus ENT: Any type of pulmonary vasculitis plus any type of vasculitic manifestation of the ENT system.