Aortic Aneurysm Occurred as Complication of Granulomatosis with Polyangiitis, Case Report Says

Patients with granulomatosis with polyangiitis can rarely develop abdominal aortic aneurysm, a serious condition that can lead to blood vessel rupture and, in extreme circumstances, death.

The case report with that finding, “Aortic Aneurysm as a Complication of Granulomatosis with Polyangiitis Successfully Treated with Prednisolone and Cyclophosphamide: A Case Report and Review of the Literature,” was published in the journal Case Reports in Rheumatology.

Granulomatosis with polyangiitis (GPA) is characterized by systemic necrotizing vasculitis, which refers to inflammation of the walls of both medium-sized and small blood vessels.

GPA patients have high serum levels of the antibody PR3-ANCA, making it a highly sensitive and specific test for diagnosis of active GPA in more than 90 percent of patients.

Chronic inflammation of the vessel sometimes leads to the formation of an arterial aneurysm, which occurs when blood vessel walls weaken and bulge.

Arterial aneurysms can be quite serious as there is a high risk of rupture, dissection, and death. So, it is very important to be aware of these complications in patients with vasculitis.

However, arterial aneurysms almost exclusively occur in small-sized vessels, and are a very unusual feature of GPA. In fact, large and medium vessel aneurysms rarely have been described in patients with GPA.

In this case report, physicians described the case of a patient with GPA who was found to have nodules (masses) in both lungs that were complicated by an aneurysm in the abdominal aorta — the major blood vessel that carries blood from the heart.

The patient, a 57-year-old Japanese man, arrived at the hospital complaining of back pain and fever that was ongoing for one month. Imaging of the aorta using a computed tomography (CT) scan showed the presence of an aneurysm in the abdominal aorta with a diameter of 34 mm and nodular lesions in both lungs.

The aneurysm was thought to be infected, so doctors gave him antibiotics. Unfortunately, the patient’s condition did not improve.

Laboratory testing indicated he had high levels of white blood cells and C-reactive protein, which is a sign of inflammation. The patient also was found to have high PR3-ANCA, an autoantibody that is present in patients with GPA. This led physicians to suspect a diagnosis of GPA.

Next, physicians conducted a lung biopsy, which revealed the presence of granulation tissue with necrosis (cell death) and multi-nucleated giant cells; both are classic microscopic features of GPA.

The patient was diagnosed with GPA and treated with an intravenous dose of the corticosteroid methylprednisolone, followed by oral prednisolone (a corticosteroid), and intravenous administration of cyclophosphamide, a chemotherapy.

After treatment, the patient’s symptoms and CT results rapidly improved, alongside a decrease in the PR3-ANCA levels. Additionally, the diameter of the aneurysm reduced from 34 mm to 21 mm.

The patient is currently in remission, and the inflammation and the high PR3-ANCA have been completely resolved. The patient did not experience any disease recurrence for four years after therapy began.

The investigators emphasize that when treating GPA patients with an aneurysm with immunosuppressants, the aneurysm should be checked regularly by imaging because it cannot be predicted when the aneurysm might rupture.

“GPA should be included in the differential diagnosis of large vessel vasculitis, which can give rise to life-threatening periaortic [around the aorta] inflammation,” researchers concluded.