Granulomatosis with polyangiitis can cause periaortitis and pericarditis.

Granulomatosis with polyangiitis (GPA) is characterized by necrotizing granulomatous inflammation usually involving the upper and lower respiratory tracts and necrotizing vasculitis predominantly affecting small-sized vessels. Although categorized as a small vessel vasculitis, GPA can cause periaortitis and pericarditis. Physicians should recognize all categories of vasculitis can affect any size arteries.

Clinical Case

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is characterized by necrotizing granulomatous inflammation usually involving the upper and lower respiratory tracts and necrotizing vasculitis predominantly affecting small‐ to medium‐sized vessels. The Chapel Hill Consensus Conference (CHCC) criteria formally classified GPA as a small vessel vasculitis associated with proteinase 3‐antineutrophil cytoplasmic antibody (PR3‐ANCA) 1. Aortic and pericardial involvement in GPA is extremely rare.

A 60‐year‐old man presented to our hospital with the chief complaint of fever and cough. He had been diagnosed with GPA based on the presence of inflammatory nasopharyngeal lesions and glomerulonephritis 26 years ago and was treated with prednisolone. Thereafter, GPA had remained in remission with no treatment. A physical examination revealed conjunctival hyperemia. Laboratory test results were as follows: leukocyte count, 6.85 × 109/L; hemoglobin, 11.5 mmol/L; C‐reactive protein (CRP) level, 13.2 mg/dL; and stable serum creatinine level, 0.58 mg/dL. The serum levels of PR3‐ANCA and antimyeloperoxidase‐ANCA were 1500 U/mL and <1.0 U/mL, respectively. Urine test results were positive for hematuria and uric protein. A chest X‐ray showed bilateral pulmonary lesions. A thoracic computed tomography scan revealed the presence of a concentric soft tissue mass around the aortic arch with double ring enhancement and enhanced pericardial thickening, in addition to multiple pulmonary nodules (Fig. 1A–C). Aortic lumen stenosis, occlusion, and aneurysms were not observed. According to the American College of Rheumatology criteria for GPA, our patient was diagnosed with a recurrence of GPA. Treatment was started with oral methylprednisolone at 1 mg/kg daily and cyclophosphamide at 2 mg/kg daily. A radiological examination conducted at 2 months after starting treatment showed a marked reduction in the size of all multicentric nodular pulmonary lesions, the concentric soft‐tissue cuff around the aortic arch, and pericardial thickening (Fig. 2A–C). There was a simultaneous improvement in symptoms. PR3‐ANCA and CRP levels decreased to within normal ranges.

Figure 1

A thoracic computed tomography scan before treatment showed a concentric soft tissue mass around the aortic arch with double ring enhancement (A) and enhanced pericardial thickening (B), in addition to pulmonary nodules (C).

Figure 2

A computed tomography examination conducted at 2 months after starting treatment showed a marked reduction in the size of the concentric soft‐tissue cuff around the aortic arch (A), pericardial thickening (B), and nodular pulmonary lesion (C).

Although categorized as a small vessel vasculitis, GPA can also cause periaortitis and pericarditis 2, 3. Physicians and radiologists should recognize that all categories of vasculitis can affect arteries of any size; hence, the current CHCC criteria recommend heightened caution 1.

Conflict of Interest

None declared.

Authorship

MM, SO, KH, HY, DU, HH, YS, SO, KF, and YY: contributed to the writing and approval of the final manuscript.