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Aortic aneurysm rupture as a rare complication of granulomatosis with polyangiitis: a case report
© Ohta et al.; licensee BioMed Central Ltd. 2013
Received: 11 December 2012
Accepted: 5 March 2013
Published: 26 July 2013
Granulomatosis with polyangiitis is characterized by systemic inflammation of medium and small blood vessels. Aortic involvement in granulomatosis with polyangiitis is extremely rare. As far as we know this is the first reported case of successful treatment in a patient with granulomatosis with polyangiitis complicated with aortic aneurysm rupture.
We describe a case of granulomatosis with polyangiitis in a 38-year-old Japanese man who developed an aortic aneurysm rupture 22 years after disease onset. The patient was operated on and a J-graft was inserted. He recovered uneventfully.
Recommendations in regard to, and consideration of, aortic involvement should be kept in mind in the long-term careful follow up of granulomatosis with polyangiitis.
Cases of aortic involvement in granulomatosis with polyangiitis
Duration of complaint
Although GPA is characterized by a systemic necrotizing inflammation of the medium and small arteries, aortic involvement is very uncommon. In general, aortic involvement is more frequently seen in large-vessel vasculitides such as those seen in giant cell arteritis and Takayasu’s arteritis [1–4]. GPA can affect the respiratory and renal tracts. It can also affect the eyes, skin, and peripheral nerves. Non-specific systemic symptoms are common. GPA causes upper respiratory tract disease in >90% of cases and causes sinusitis, nasal crusting, bleeding, and obstruction, and collapse of the nasal bridge [5–8]. It can also cause otitis media and tracheal stenosis. When the lungs are affected, it may present as cough, hemoptysis, and dyspnea. Renal involvement may manifest as hematuria and proteinuria and can lead to renal failure. Ophthalmological manifestations include subconjunctival hemorrhage, scleritis, uveitis, keratitis, proptosis, or ocular muscle paralysis due to retro-orbital inflammation. Serology is positive for anti-proteinase 3 (anti-neutrophil cytoplasmic antibody), which is highly specific for GPA. The differential diagnosis of GPA includes polyarteritis nodosa, Churg–Strauss syndrome, Henoch–Schönlein purpura, temporal arteritis, and Takayasu’s arteritis. The main treatment is immunosuppression and steroid therapy. A combination of cyclophosphamide and prednisone is effective. Our patient had been receiving prednisolone (15mg/day) for 11 years (since his diagnosis) and had remained negative for anti-neutrophil cytoplasmic antibody until he presented with the aneurysm.
Aortic aneurysm is a very rare complication of GPA. To the best of our knowledge, nine other cases of aneurysm of large vessels as a result of GPA have been described [5–15] and this is the first reported case of successful treatment in a patient with GPA complicated with aortic aneurysm rupture. In five of the cases, the patients were operated on and surgical tissue biopsies showed signs of vasculitis indicative of GPA. In one case, the patient was treated with steroids and cyclophosphamide, but medical treatment could not prevent aortic dissection and the patient died from rupture of the dissection. In three cases, the patients were treated medically with drugs that included methylprednisolone and trimethoprim-sulfamethoxazole. CT is an extremely accurate tool for both diagnosis and sizing of aortic aneurysms [5–9]. A longitudinal cohort study of GPA patients evaluated at the U.S. National Institutes of Health between 1968 and 1992 demonstrated that 91% experienced clinical improvement and 75% entered complete remission when treated with cyclophosphamide and methylprednisolone . A meta-analysis revealed that pulse cyclophosphamide is less toxic than continuous cyclophosphamide but may be associated with a higher relapse rate .
In summary, chest pain occurring during a GPA flare may result from aortic involvement and may cause a life-threatening aneurysm rupture for which surgical treatment is indicated. Therefore, aortic involvement should be kept in mind as a potential fatal complication of GPA.
We presented an extremely rare case of thoracic aortic aneurysm rupture as a complication of GPA. GPA should be included in the workup of large-vessel vasculitis, which can give rise to potentially life-threatening periaortic inflammation.
Written informed consent was obtained from the patient for publication of this manuscript and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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