A Case of Chronic Periaortitis with Retroperitoneal Fibrosis
The patient, a 73-year-old man, was admitted with abdominal pain and general ache for 3 months. Eight years ago, a coronary angiogram at another medical center, because of chest pain, showed normal coronary arteries. Three years ago, he started to complain of an intermittent dull pain from the upper abdomen to the chest. A year ago, he visited another medical center with acute chest pain where he was diagnosed as acute aortic dissection, Debakey type I, at that time. He then underwent ascending aorta replacement using a graft. Nine months later, his abdominal pain with general ache worsened. A follow-up chest CT showed an aggravated descending aortic hematoma and bilateral hydronephrosis.\nOn admission day, his vital signs were stable; blood pressure was 110/68 mm Hg, heart rate 65 beats per minute, body temperature 36.5℃. Physical examination revealed no abnormal findings. The initial laboratory findings showed an elevated level of inflammatory markers; erythrocyte sedimentation rate (ESR) 100 mm/hr (normal range 0-10 mm/h), C-reactive protein (CRP) 4.19 mg/dL (normal range <0.5 mg/dL) and Ferritin 837.5 ng/mL (normal range 30-400 ng/mL). The serum creatinine was 1.82 mg/dL but the urinalysis did not showed overt proteinuria, probably caused by post-renal obstruction. The immunoglobulin G (IgG) level was borderline high, 1627 mg/dL (normal range: 700-1600 mg/dL) and the IgG subtype IV accounted for approximately 4.4%, 0.73 g/L (normal range: 0.06-1.21 g/L). Autoantibodies, such as antinuclear antibody, antineutrophil cytoplasmic autoantibody and rheumatoid factor were negative. Complement three (C3) and four (C4) levels were also between normal range, 138.7/38.6 mg/dL (normal range 90-180 mg/dL, 10-40 mg/dL). The 2D-echocardiography showed normal systolic function and normal sized left ventricle without regional abnormality. The follow-up CT scan showed aggravated abdominal aortic intramural hematoma but also bilateral ureteral obstruction related to retroperitoneal fibrosis. For bilateral hydronephrosis due to ureteral obstruction, we performed a double J catheter insertion at both ureters (). The serum creatinine level had not risen since, but it was also not normalized and remained around 1.8 mg/dL. After consultation with the physician who conducted the chest surgery, we continued medical therapy for chronic abdominal aortic intramural hematoma.\nBecause of the chronic recurrent disease course and CT finding, we considered the diagnosis of chronic periaortitis with retroperitoneal fibrosis. Chronic periaortitis may present as dull, poorly localized pain, nonspecific systemic illness, raised inflammatory markers and renal impairment due to ureteric obstruction.- Positron emission tomography (PET)-CT showed an increased standardized uptake values level in not only the aortic arch, descending thoracic aorta and major branch (maximum 4.3), but also the abdominal aorta (maximum 5.2) and common iliac artery (maximum 5.3) (). On review of the biopsy specimen achieved from previous surgery on the ascending aorta, the hematoxylin-eosin stain showed a consistent infiltration of lympho-plasma cells without granuloma formation in the media of the aortic wall (). In immunochemistry staining, positive findings were revealed at CD20, CD3, Lambda and Kappa stain.\nWith the diagnosis of chronic aortitis with retroperitoneal fibrosis complicated to bilateral hydronephrosis, we consulted rheumatology, and high-dose steroid therapy (1 mg/kg prednisolone) and azathioprine (50 mg t.i.d) medication were started. Several days later, his complaints including weakness and abdominal pain improved and laboratory findings demonstrated improving results (ESR 39 mm/hr, CRP 0.09 mg/dL, serum creatinine 1.37 mg/dL). He was discharged and re-visited our institution 2 weeks later for follow up. He complained of weakness and follow up laboratory findings showed extensive elevation of inflammatory markers; ESR 107 mm/hr, CRP 39.28 mg/hr. On that very day, he died a sudden death.