Pulmonary artery involvement in Wegener's granulomatosis

Classification and nomenclature schemes are guidelines and are not intended to recognize and distinguish the entire spectrum of any single disease. It may in fact be misleading to suggest that a classic presentation of a given disease plus atypical features should be considered an "overlap" of two separate, often rare, conditions. We report a case of typical Wegener's granulomatosis (WG), with the coexistence of pulmonary artery stenosis, a lesion more commonly observed in TA. This is not the first or only example of large vessel vasculitis occurring in patients with WG. This observation cautions clinicians to avoid rigid application of classification and nomenclature systems and raises questions about determinants of vasculitis subsets and organ targeting.