Case report of a 28-year-old man with aortic dissection and pulmonary shadow due to granulomatosis with polyangiitis

Abstract

Background: Granulomatosis with polyangiitis (GPA) is characterised by the main violation of the upper and lower respiratory tract and kidney. GPA is considered a systemic vasculitis of medium-sized and small blood vessels where aortic involvement is extremely rare.

Case presentation: A 28-year-old male was admitted to the hospital due to 4 h of chest pain. Computed tomography scan of the aorta showed a thickened aortic wall, pulmonary lesions, bilateral pleural effusion and pericardial effusion. The aortic dissection should be considered. An emergency operation was performed on the patient. Surgical biopsies obtained from the aortic wall showed destructive changes, visible necrosis, granulation tissue hyperplasia and a large number of acute and chronic inflammatory cells. Nearly a year later, the patient was re-examined for significant pulmonary lesions. His laboratory studies were significantly positive for anti-neutrophilic antibody directed against proteinase 3. Finally, the diagnosis of GPA was obviously established.

Conclusions: Although GPA rarely involves the aorta, we did not ignore the fact that GPA may involve large blood vessels. In addition, GPA should be included in the systemic vasculitis that can give rise to aortitis and even aortic dissection.

Keywords: Aortic dissection; Aortitis; Granulomatosis with polyangiitis; Vasculitis.

Fig. 1

Computed tomography scan of the aorta shows a thickened aortic wall, pulmonary lesions, bilateral pleural effusion, and pericardial effusion: a Ascending aortic dissection and thickened aortic wall (white arrow); b Bilateral pleural effusion (red arrow) and pericardial effusion (white arrow); c pulmonary lesions (red arrow); d pulmonary lesions (red arrow)

Fig. 2

Cardiac colour ultrasound suggesting aortic hematoma or dissection (red arrow)

Fig. 3

Pathological characteristics of the patient: a inflammatory cell infiltration, cellulose exudation and necrosis in clamped lung tissues; b epithelioid cells and multinucleated giant cells forming a granulomatous reaction in the aorta; c small vasculitis in the aorta; d cellulose exudation and inflammatory granulation tissue hyperplasia in the pericardium(haematoxylin–eosin stain, magnification × 200)