Objective: Understand the clinical features of chronic periaortitis.
Methods: The medical records of 28 cases with definite diagnosis of chronic periaortitis were reviewed retrospectively.
Results: Among these 28 cases, 20 (71.4%) fulfilled the diagnostic criteria of idiopathic retroperitoneal fibrosis (IPF), 5 (17.8%) were inflammatory abdominal aortic aneurysm (IAAA) and 3 (10.7%) were perianeurysmal retroperitoneal fibrosis (PARF). The common symptoms were abdominal blunt pain (35.7%), lumbago (25%), loss of body weight (25%), abdominal distension (17.8%) and lower extremities pitting edema (17.8%). Hydronephrosis was found by B ultrasonography in 17 (85%) patients with IPF. Dilated abdominal aorta were found in 7 patients. One case was complicated with ankylosing spondylitis and another one was diagnosed to have undifferentiated connective diseases. All 5 IAAA patients were treated by aneurysm segregation or stent implantation, but none had medical therapy. The 3 perianeurysmal retroperitoneal fibrosis patients were mis-diagnosed as with either IPF or AAA. Seventeen patients in this group were treated with corticosteroid combined with immunosuppressive agents or tamoxifen. Four cases were followed up and their conditions were improved which were demonstrated by repeated CT or MRI.
Conclusion: Chronic periaortitis is a rare autoimmune rheumatic disease. IPF is the most common one compared to IAAA and PARF. It can be complicated with autoimmune disorders or serum auto-antibodies. No case with other organ fibrosis disorder wis observed in this study. Radiological examination and pathological examination are necessary for confirming the diagnosis. Corticosteroid combined with immunosuppressive agents or tamoxifen is the effective treatment.