Vasculitis of the Gastrointestinal Tract in Chronic Periaortitis

Abstract

The term "chronic periaortitis" (CP), proposed by Mitchinson in 1984, comprises 3 main entities: idiopathic retroperitoneal fibrosis (IRF), inflammatory abdominal aortic aneurysms (IAAAs), and perianeurysmal retroperitoneal fibrosis (PRF).The presence of constitutional symptoms, high acute-phase reactants, positive autoantibodies, and associated autoimmune diseases suggests a systemic inflammatory process. Histopathologic findings show vasculitis with fibrinoid necrosis involving the aortic vasa vasorum as well as the small and medium retroperitoneal vessels.We reviewed the medical records of 608 patients with a diagnosis of vasculitis involving the gastrointestinal (GI) tract at the Mayo Clinic between January 1996 and December 2007. Only patients with biopsy-proven or typical angiographic findings of vasculitis localized to the GI tract were included.Five patients were identified with evidence of CP (1 patient with PRF, 1 with IRF, and 3 with IAAAs). Three patients were men, and the median age at diagnosis was 49 years. The diagnosis of GI vasculitis and CP was made simultaneously in 4 patients. At the time of onset, all patients had abdominal pain and constitutional manifestations; the median erythrocyte sedimentation rate was 62.5 mm/1 h (range, 20-86 mm/1 h). All patients had evidence of mesenteric vasculitis at angiography. Three patients also had associated renal artery stenoses. Abdominal computed tomography showed spleen infarcts in 2 patients, bowel wall thickening in 1, and liver infarction in 1. Two patients underwent surgical intervention for acute abdomen; there was histologic evidence of small bowel infarcts and infarction of the spleen and liver in 1. Oral prednisone was administered to all 5 patients (median starting dose, 60 mg/d; range, 25-80 mg/d). Three patients also received immunosuppressive agents, 1 tamoxifen, and 1 anti-tumor necrosis factor therapy. All patients had at least 1 relapse or recurrence of vasculitis, but at last visit, GI vasculitis and CP were in remission in all 5 patients.This study provides evidence that GI manifestations due to mesenteric vasculitis may be associated with CP. Vasculitic involvement of the renal arteries is also frequently present in these patients. Aggressive immunosuppressive treatment should be promptly initiated to forestall abdominal complications. These findings reinforce the hypothesis that a vasculitic process plays an important role in the pathogenesis of CP.

Idiopathic Retroperitoneal Fibrosis

“…The periaortic tissue can extend to the mesenteric and celiac arteries, causing stenosis and ischemic complications resembling mesenteric vasculitis. 24 Up to one-third of patients with abdominal RPF also have thoracic aorta involvement, most of them presenting with thoracic aorta aneurysm, the progression of which must also be carefully monitored. 7 Laboratory Findings Acute-phase reactants such as erythrocyte sedimentation rate and C-reactive protein levels are increased in the majority of patients at presentation and are routinely used to monitor disease activity.…”

Retroperitoneal fibrosis

“…[17][18][19] In a study of five patients with concomitant gastrointestinal vasculitis and chronic periaortitis, Salvarani et al found that histological data and imaging indicate the possibility of chronic periaortitis and its separate disease entities as a type of large-vessel vasculitis. 20 More recently, researchers have found an association between RPF and IgG4-related systemic disease. In a review of 14 published cases of IgG4-related RPF, Stone found that all of the cases identified as IgG4-related, based on a serology, were male, which confirmed prior evidence of male predominance in IgG4-related systemic diseases.…”

Single-organ gallbladder vasculitis: Characterization and distinction from systemic vasculitis involving the gallbladder. An analysis of 57 patients

“…8,24,39,55,62,63 Gastrointestinal involvement is usually associated with a worse prognosis in these forms of SV. 46,57,62 Abdominal territories may rarely be the site of a focal singleorgan vasculitis (SOV).…”