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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.

Objective: To describe the clinical features and outcomes of patients with localized vasculitis of the gastrointestinal tract (LVGT)., Methods: Medical records of 608 patients diagnosed with vasculitis involving the intra-abdominal vasculature and/or abdominal viscera between January 1996 and December 2007 were reviewed. Only patients with histopathological confirmation or typical angiographic findings of vasculitis localized to the abdomen were included., Results: We identified 18 cases with LVGT over the 12-year study period. The patients were predominantly Caucasian (89%) and female (67%) with a median age at diagnosis of 53.5 (range 17.4-83.3) years. Most of the patients presented with abdominal pain and 12 (66.6%) patients presented with an acute abdomen requiring surgical intervention. At diagnosis, the median ESR was 30.5 (range 4-77) mm/h. Autoantibody screening was generally unrevealing. Abdominal CT scan findings included: bowel wall thickening, bowel infarction and solid organ infarcts. In 14 patients, the diagnosis of vasculitis was established by abdominal angiography. Histological evidence of vasculitis was recorded in 5 (28%) patients, most commonly from gall bladder or small intestine specimens. Corticosteroid therapy was administered to 10 (56%) patients, 5 of whom also received other immunosuppressive agents. Median duration of follow-up was 10.5 (range 2-156) months. No evidence of vasculitis outside the abdomen was observed during follow-up. Seven (39%) patients died during the follow-up period. Survival of the patient cohort (compared with an age-matched US white population) was significantly reduced (P < 0.001)., Conclusion: LVGT is an uncommon form of vasculitis that can be associated with significant morbidity and mortality.

Objective: This study reviewed the outcomes of open and endovascular revascularization for mesenteric vasculitis (MV)., Methods: We reviewed the clinical data of all patients who underwent revascularization for occlusive MV from 1984 to 2008. Patients treated for aneurysms or mucosal bleeding without ischemic symptoms were excluded. End points were early mortality and morbidity, survival, freedom from mesenteric symptoms, and patency. Outcomes of open reconstructions were compared with the results of 163 patients who underwent open operations for atherosclerotic disease., Results: There were 15 patients (13 females, 2 males) with a mean age of 38 years (range, 15-66 years). Etiologies were Takayasu's arteritis in 7, polyarteritis nodosa in 4, indeterminate in 3, and giant cell arteritis in 1. The celiac axis was affected in 13, superior mesenteric artery (SMA) in 13, renal arteries in 8, and the aorta in 4. Seven patients had active disease, and eight were in remission. Nine (60%) presented with symptomatic chronic (n = 8) and acute (n = 1) mesenteric ischemia. Six patients with asymptomatic disease underwent mesenteric revascularization during other aortic-based operations. Fourteen patients (93%) had 10 mesenteric bypasses (8 aortic based; 2 iliac), three had aortoplasties, of which two had mesenteric patch angioplasties, and one underwent arcuate ligament release with patch angioplasty. One patient (7%) underwent percutaneous transluminal angioplasty of SMA stenosis. There were no early deaths. Early complications occurred in three patients (20%) after open reconstruction, including gastrointestinal hemorrhage, ileus with re-exploration, and superior mesenteric vein thrombosis. Median follow-up was 22 months. One graft thrombosis in a patient with active disease was treated with redo bypass 74 months after aorta-celiac-SMA bypass. All patients were alive at 10 years, with similar expected survival compared with the general population (P = .69). Compared with patients with atherosclerotic disease, open reconstructions for MV had similar freedom from mesenteric symptoms (83% vs 75%, P = .80) and similar primary graft patency (83% vs 84%, P = .9)., Conclusion: Mesenteric vasculitis is a rare manifestation of Takayasu arteritis, polyarteritis nodosa, indeterminate, or giant cell arteritis. Open revascularization is durable and effective when needed., (Copyright 2010 Society for Vascular Surgery. Published by Mosby, Inc. All rights reserved.)

This report details an unusual case of Epstein-Barr virus (EBV)-associated post-transplant lymphoproliferative disorder (PTLD) involving the epicardial coronary arteries and myocardial arterioles of an allograft heart, morphologically mimicking vasculitis, a finding associated with acute rejection, cardiac allograft vasculopathy (CAV), infection and infarction. Recognition of this novel PTLD pattern as a potential imitator of acute rejection in particular is important because reduction of immunosuppression (to treat PTLD) is antithetical to the usual therapy for rejection.

Objective To evaluate the efficacy and safety of mycophenolate mofetil (MMF) in adult primary central nervous system vasculitis (PCNSV). Methods We studied a cohort of 163 patients with PCNSV who were seen at the Mayo Clinic from 1983 to 2011. We compared patients treated with MMF and those receiving other therapies. Results We identified 16 patients treated with MMF. MMF in combination with GCs achieved a favorable response in most patients. A significant proportion of patients treated with MMF had a less severe disability at last follow-up compared to those receiving other therapies ( p = 0.023) and cyclophosphamide and prednisone ( p = 0.017). No statistically significant differences were observed regarding relapses and ability to discontinue therapy at last follow-up. A trend to a more favorable treatment response was observed in patients treated with MMF compared to those treated with other therapies ( p = 0.075). Only 1 patient suspended MMF for severe leukopenia. Conclusion MMF seems to be an effective and safe therapy for adult PCNSV. [ABSTRACT FROM AUTHOR]

Objective To describe the treatment and outcomes of patients with primary central nervous system (CNS) vasculitis. Methods We retrospectively studied a cohort of 163 consecutive patients with primary CNS vasculitis who were seen at the Mayo Clinic over a 29-year period. We analyzed treatments, treatment responses, and factors predictive of outcomes. Results A favorable response was observed in 85% of patients treated with prednisone alone and in 80% of patients treated with prednisone and cyclophosphamide. Relapses were observed in 27% of patients, and 25% of patients had discontinued therapy by the time of the last followup visit. Treatment with prednisone alone was associated with more frequent relapses (odds ratio [OR] 2.90), while large vessel involvement (OR 6.14) and cerebral infarcts at the time of diagnosis (OR 3.32) were associated with a poor response to treatment. Prominent gadolinium-enhanced cerebral lesions or meninges were linked with continued treatment at the last followup encounter (OR 2.28). Higher disability scores at the last followup visit were associated with increasing age at the time of diagnosis (OR 1.44) and cerebral infarctions (OR 3.74), while lower disability scores were associated with gadolinium-enhanced cerebral lesions or meninges (OR 0.35) and cerebral amyloid angiopathy (OR 0.24). Increased mortality was associated with increasing age at diagnosis (hazard ratio [HR] 1.39), diagnosis by angiography (HR 3.28), cerebral infarction (HR 4.44), and large vessel involvement (HR 4.98), while reduced mortality was associated with gadolinium-enhanced cerebral lesions or meninges (HR 0.20). Conclusion The majority of patients with primary CNS vasculitis responded to treatment. Recognition of findings at diagnosis that predict the course or outcome may aid in decision-making regarding therapy. [ABSTRACT FROM AUTHOR]

Objective. To describe a subset of cases in a large cohort of patients with primary CNS vasculitis (PCNSV) who appear to have a rapidly progressive clinical course.Method. In the present study, we use our updated cohort of 131 consecutive patients with PCNSV seen over the 25-year period of 1983–2007 at Mayo Clinic, Rochester, MN, USA. The diagnosis of PCNSV was based on brain/spinal cord biopsy or cerebral angiography. The modified Rankin scale was used to identify rapidly progressive disease and included patients with Rankin scores indicating severe disability or death at diagnosis or within 6 months after the diagnosis. We compared patients with rapidly progressive disease to those without.Results. Compared with the 120 patients without rapidly progressive vasculitis, the 11 patients with rapidly progressive vasculitis more frequently had paraparesis/quadriparesis at presentation, angiographic presence of bilateral, large-vessel vasculitis and MRI evidence of cerebral infarctions; those infarctions were more frequently multiple and bilateral, and more frequently involved both the cortex and subcortical regions on initial MRI. Granulomatous and/or necrotizing histopathological patterns of vasculitis were observed in patients with positive biopsies.Conclusion. Rapidly progressive PCNSV appears to form a subset of PCNSV at the worst end of the clinical spectrum of this vasculitis, characterized by bilateral, multiple, large cerebral vessel lesions and multiple CNS infarctions. [ABSTRACT FROM AUTHOR]

The article discusses a study on patients with primary central nervous system vasculitis (PCNSV) who underwent magnetic resonance imaging (MRI). The study analyzed various data of 101 patients with the disease including demographics, neurologic outcome and biopsy of brain or spinal cord. It was found that prominent gadolinium meningeal enhancement were seen after MRI.