the term chronic periaortitis refers to a spectrum of idiopathic diseases characterized by a fibroinflammatory reaction that extends from the adventitia of the abdominal aorta and the common iliac arteries into the retroperitoneum .
this often leads to the encasement of the adjacent structure , e.g. ureters or inferior vena cava .
idiopathic retroperitoneal fibrosis , inflammatory abdominal aortic aneurysms and perianeurysmal retroperitoneal fibrosis are its various clinical presentations.1)2 ) it is most often diagnosed by a periaortic enhancement based on imaging studies , owing to the difficulty of using an aortic wall biopsy .
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 .
a follow - up chest ct showed an aggravated descending aortic hematoma and bilateral hydronephrosis . on 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
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
1 ) . 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 . because 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.3 - 5 ) 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 ) ( fig .
2 ) . 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 ( fig .
3 ) . in immunochemistry staining , positive findings were revealed at cd20 , cd3 , lambda and kappa stain . with 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 .
however , subsequent studies have suggested that chronic periaortitis is a systemic immune - mediated or inflammatory process.6 ) this is a rare disease with protean manifestations but , if correctly diagnosed , can be successfully managed .
early diagnosis may be important in reducing the mortality.3)4 ) clinical features of chronic periaortitis is usually related to the mechanical effects of the retroperitoneal fibrosis on the adjacent structures , such as back or abdominal pain , ureteral colic - like pain , deep vein thrombosis , claudication , and testicular pain .
however it also presents with constitutional symptoms , such as fatigue , weight loss and anorexia , probably reflecting the systemic inflammatory nature of the disease .
laboratory investigations are useful but not diagnostic.5)7)8 ) the pathologic changes in chronic periaortitis involve both the aortic wall and the surrounding soft tissues . the inflammatory infiltrate
mainly consists of mononuclear cells , such as lymphocytes , plasma cells and macrophages and scattered eosinophils , which predominate at the media - adventitia junction and extend throughout the adventitia.6)8 ) immunoglobulin g4-related lymphoplasmacytic aortitis is characterized by the enhanced tissue migration of igg4-positive lymphocytes accompanied by an increase in serum igg4 levels although this case did not fit that description.9)10 ) diagnosis should start by excluding possible primary causes of retroperitoneal fibrosis and is made by means of imaging studies .
ct and mri are the choice modalities for not only the diagnosis but also follow - up .
iino et al.11 ) have reported a sensitivity of 83.3% , a specificity of 99.7% and an accuracy of 93.7% for the diagnosis of in flammatory abdominal aortic aneurysms using ct.3 ) pet with 18f - fluorodeoxyglucose ( fdg ) is emerging as a useful functional imaging modality for assessing disease activity . in the early stages of the disease , there is a strikingly higher periaortic uptake of fdg , which disappears after successful medical therapy.12 ) this is a rare case which was confirmed as tissue diagnosis , not only an imaging diagnosis with ct finding .
recent retrospective analysis has found that the combination of steroids and azathioprine as effective as and safer than steroids plus cyclophosphamide .
steroid , alone or in combination with surgery , is usually effective , but prospective randomized trials are required to establish the best treatment approach.3)4)13 ) suspicion of chronic periaortitis is mostly important to treatment of these patients and careful follow - up is essential because of its chronic - relapsing clinical course .
 a 73-year - old man with a history of hypertension and ascending aortic dissection was hospitalized for aggravated abdominal pain and general ache for 3 months . 
 follow - up ct showed aggravated abdominal aortic hematoma with aneurysm , atherosclerotic periaortitis and bilateral hydronephrosis . 
 an initial laboratory finding showed elevated levels of inflammatory markers and renal dysfunction . 
 positron emission tomography - ct showed an increased standardized uptake values level in the aortic arch , descending thoracic aorta , major branch , abdominal aorta , and common iliac artery . for bilateral hydronephrosis 
 , a double j catheter insertion was performed . 
 tissue specimens obtained from previous surgery on the aorta indicated the infiltration of lympho - plasma cells without granuloma formation in the aortic wall . after a combined therapy of high dose steroid therapy with azathioprine , the patient 's initial complaints of abdominal pain , weakness and azotemia improved . 
 this case was diagnosed as chronic periaortitis based on aortic inflammation at biopsy , which was complicated with retroperitoneal fibrosis and ureteric obstruction . 
Introduction
Case
Discussion