Pulmonary artery involvement in Wegener's granulomatosis

T. Clark, G.S. Hoffman

Cleveland Clinic Foundation, Center for Vasculitis Care and Research, Cleveland, Ohio, USA.

ABSTRACT
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.

Key words
Wegener's granulomatosis, vasculitis, pulmonary artery.


Please address correspondence to: Gary S. Hoffman, MD, MS, Director, Center for Vasculitis Care and Research, Harold C. Schott Chair of Rheumatic and Immunologic Diseases, Professor of Medicine, Lerner College of Medicine of Case Western Reserve University, Cleveland Clinic Foundation A50, Cleveland, OH 44195, USA.

Clin Exp Rheumatol 2003; 21: (Suppl. 32): S124-S126.
© Copyright Clinical and Experimental Rheumatology 2003.