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AB0537 Isolated Aortitis or Aortitis Related with Retroperitoneal Fibrosis: A Single Center Experience of 12 Patients
  1. D. Üsküdar Cansu1,
  2. I. Ak Sivrikoz2,
  3. M. Kebapçi3,
  4. C. Korkmaz4
  1. 1Rheumatology, Associated Proff
  2. 2Nuclear medicine
  3. 3Radiology
  4. 4Rheumatology, Eskişehir Osmangazi University, Eskişehir, Turkey

Abstract

Background Aortitis is the inflammation of aortic wall and it can result from infectious or non-infectious conditions. Retroperitoneal fibrosis (RPF) may be accompanied by idiopathic aortitis.

Objectives In this report, we aimed to retrospectively evaluate in terms of clinical course, treatment and outcomes of 12 patients with isolated aortitis or aortitis related with RPF.

Methods We reviewed files of our patients diagnosed with non-infectious aortitis or aortitis with RPF between Jan-1998-Dec-2015. Their clinical presentations, co-morbidities, location of aortic involvement, treatment features and outcomes were recorded. The diagnosis of aortitis and RPF were based on positron emission tomography (FDG-PET-CT) scan and/or computed tomography. The presence of RPF was confirmed by biopsy in 4 patients.

Results The study group consisted of 12 patients (8 M, 4 F). The mean age at the time of diagnosis was 60 yrs (42–70 yrs). Isolated aortitis was found in 5 patients, RPF plus aortitis in 6 patients, pure RPF in 1 patient. 6 patients with RPF plus aortitis presented with hydronephrosis and 1 patient with pure RPF presented with cystocele. In patients with isolated aortitis, 4 patients had subclavian and thoraco-abdominal involvement, 1 patient had bilateral subclavian and abdominal aorta involvement. 5 patients with RPF plus aortitis had only abdominal aorta involvement, 1 patient had thoraco-abdominal involvement. 1patient with abdominal aorta involvement had aortic aneurysm. Constitutional symptoms including fever, weight loss, and fatigue existed in all patients at the first admission. Hydronephrosis and post-obstructive renal failure were treated with double J stent and steroid treatment in 6 patients with RPF. Acute phase reactants increased in all patients. Ig G4 levels were determined in 8 patients and only 1 patient had increased level of Ig G4. All patients were given only prednisolone or prednisolone plus immunosuppressive drugs such as azathioprine or methotrexate. Clinical manifestations and increased acute phase reactants resolved in a reasonable time. 2 patients passed away due to sepsis and intra-cerebral infection, respectively. One of these 2 patients had many co morbidities.

Conclusions While constitutional symptoms were a dominant complaint in isolated aortitis, hydronephrosis and acute renal failure as well as back pain were the chief manifestations of RPF. The presence of co-morbidities may play a role in mortality.

Acknowledgement None

Disclosure of Interest None declared

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