Published: 2021-09-22

An extraordinary pulmonary artery involvement in granulomatosis with polyangiitis

Ahmad A. Alkhdairi, Ayman A. Alharbi


Granulomatosis with polyangiitis (GPA) is an autoimmune mediated systemic disease and characterized by pauci-immune vasculitis mainly of small and medium vessels with typical necrotizing granulomatous lesions in the affected tissues. A 44 year old lady who was known to have GPA presented with unusual presentation of its category with extraordinary large vessels involvement including pulmonary arteries and her condition improved with immunosuppressive therapy. We reported this case, to improve the awareness about other overlapping categories of vessels vasculitis that may involve large vessels and main pulmonary arteries and to avoid misdiagnosing these patients with category of classical large vessels vasculitis.


GPA, Pulmonary artery, Large vessels, Wegener's granulomatosis

Full Text:



Kahl L. The Washington manual of rheumatology subspecialty consult. 2nd ed. Philadelphia: Wolters Kluwer Health/Lippincott Williams and Wilkins; 2012: 194-237.

Klippel JH, Stone JH, Crofford LJ, White PH. Primer on the Rheumatic diseases. 13th ed. New York, NY: Springer; 2008: 416-26.

Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. Overview of the 2012 revised International Chapel Hill. Consensus Conference nomenclature of vasculitides. Arthritis Rheum. 2013;65(1):1-11.

Greco A, Marinelli C, Fusconi M, Macri GF, Gallo A, DeVirgilio A, et al. Clinic manifestations in granulomatosis with polyangiitis. Int J Immunopathol Pharmacol. 2016;29(2):151-9.

Comarmond C, Cacoub P. Granulomatosis with polyangiitis (Wegener): clinical aspects and treatment. Autoimmun Rev. 2014;12(11):1121-5.

Ozaki T, Maeshima K, Kiyonaga Y, Torigoe M, Imada C, Hamasaki H, et al. Large-vessel involvement in granulomatosis with polyangiitis successfully treated with rituximab: a case report and literature review. Mod Rheumatol. 2017;27(4):699-704.

Clark T, Hoffman GS. Pulmonary artery involvement in Wegener's granulomatosis. Clin Exp Rheumatol. 2003;21:124-6.

Yates M, Watts RA, Bajema IM, Cid MC, Crestani B, Hauser T, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2016;75(9):1583-94.