Atypical Manifestations of Granulomatosis with Polyangiitis: The Diagnostic Challenge for Pulmonologists

Abstract

This is a review considering atypical manifestations of granulomatosis with polyangiitis (GPA). Virtually any organ can be affected, and in some patients, GPA can manifest unusually. Since thoracic involvement of GPA often predominates, the first who might be expected to establish a diagnosis are pulmonary specialists. We would like to familiarize pulmonary specialists with several extra-ELK (E: ear-nose-throat; L: lung; K: kidney) involvements of the disease. We describe sites rarely affected by GPA like the breast, skeletal system, orbit and eye, heart and vessels, central nervous system, urogenital system as well as endocrine and gastrointestinal tract involvement.

A rare case report of granulomatosis with polyangiitis presenting with thrombus of the ascending aorta

“…2 Cardiac manifestations of GPA are rare and can include pericarditis, cardiomyopathy, coronary arteritis, valvular lesions and conduction abnormalities. 3 Large vessels may also be affected, despite it being classified as a small vessel vasculitis. Specifically for the aorta, which was affected in our case, this can present with aneurysmal formation, dissection, rupture, regurgitation or death.…”

Gastrointestinal Hemorrhage With Gastritis and Pancolitis as the Sole Presentation for Granulomatosis With Polyangiitis Flare

“… 11 GI manifestations occur late in the disease course of GPA. 14 Although some studies have explored endoscopic findings of GI disease in other vasculitides, because of the rarity of GPA in the GI tract, most of the work in this area has focused on the widely varying GI manifestations of GPA. 15 Currently, neither the American College of Rheumatology nor the American College of Gastroenterology has guidelines on the endoscopic diagnosis or medical management of GI manifestations of GPA.…”

Ocular Involvement of Granulomatosis with Polyangiitis

“…Many regions of the body may be affected by this disease, but the typical course of GPA is associated with the following disturbances: upper respiratory tract inflammation with sinusitis and nasal lesions, pulmonary infiltrations, and glomerulonephritis [ 4 , 5 ]. The prevalence of ocular manifestations of the disease is 50%, with 8–16% of cases developing them as the first manifestation [ 6 , 7 , 8 , 9 ].…”