P.E

A 70-year-old gentleman with NIDDM, HTN and hypothyroidism who have been on glucocorticoid therapy since 2015 for IgG4-RelatedDisease (IgG4-RD) with a good initial response to treatment. He had CT-guided left para-aortic core biopsy that was complicated by massive bleeding managed by coil embolization. Histopathology showed characteristic features of IgG4-RD. In 2017, the patient was referred to our vascular clinic with incidental findings of soft tissue thickening around the mid-distal.

infrarenal aorta extending to both common iliac arteries on his last routine CT follow up scans (Fig.1 A, B, C, yellow arrow; video 1: axial movie CT aortogram). He had chronic abdominal and back pain with recurrent UTI for four years, however, there was no evidence of urinary tract obstruction and physical examination was unremarkable. We planned to continue medical treatment with follow-up.

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Eight months later he had a relapse and developed hydronephrosis. Right nephrostomy and antegrade JJ-stent was inserted following a complicated failed retrograde approach. In addition, rituximab was started to control his relapse. He continues to deteriorate with multiple relapses and worsening abdominal and back pain unresponsive to opiates. Therefore, azathioprine was initiated, however, recurrent pain was significantly affecting his quality of life.

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After frequent relapses despite optimum medical therapy, a decision was made to proceed with endovascular treatment. PreoperativeCT angiogram showed persistent soft tissue thickening (Fig. 2A, B, C, yellow arrow; video 2: axial movie CT aortogram), right JJ-stent in place (Fig. 2A, red arrow) and previous embolization coil (Fig. 2B, blue arrow).A percutaneous endovascular aorto-bi-iliac stenting was performed using unibody AFX stent-graft (Endologix Inc, Irvine, CA, USA) extended proximally with uncovered Sinus XL stent (OptiMed GmbH, Ettlingen, Germany) and distally with covered BeGraft stents (Bentley, InnoMed GmbH Hechingen, Germany)in both common iliac arteries (video 3 intraoperative completion angiogram; video 4 postoperative 3D reconstruction CT aortogram). The surgery went uneventful.At six-week follow up, the patient symptoms showed marked improvement and at one-year follow-up he has no pain, not receiving any pain medications with significant improvement in quality of life. Furthermore, His JJ-stent was removed and his abdominal CT angiogram revealed complete resolution of periaortitis and patent stent without any evidence of endoleak (Fig. 3 A, B, C, the blue arrow shows previous embolization coil; video 5 axial movie CT aortogram).
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To our knowledge, this is the 2nd successful case in literature to treat IgG4-RD periaortitis not associated with inflammatory aneurysm with endovascular stent graft after refractory medical therapy. Further studies are needed to investigate the long-term outcome of the endovascular approach in such a rare disease entity.Early diagnosis and treatment of the underrecognized IgG4-RD are important before reaching the poorly responsive fibrotic stage with morbidity related to organ damage. Although the majority of patients respond to medical therapy, relapses are still common.In cases of IgG4-RD periaortitis which is refractoryto medical therapy, endovascular intervention may provide a promising outcome.