| Abstract|| |
Retroperitoneal fibrosis is an uncommon disease that often presents in a subtle manner. Only a few cases of the combined association of generalized atherosclerosis and retroperitoneal fibrosis are reported in the recent literature, supporting the view that the condition is probably an autoimmune periaortitis. We describe a typical case of retroperitoneal fibrosis associated with generalized atherosclerosis with clinical presentation of progressive renal insufficiency, and claudication from arterial compromise.
Keywords: Retroperitoneal fibrosis, autoimmune periaortitis, Atherosclerosis, Inermittent claudication.
|How to cite this article:|
Barbullushi M, Pasko N, Bezhani E, Duraku A, Rusi R, Hoti K, Bakalli V, Idrizi A. Secondary Retroperitoneal Fibrosis Associated with Generalized Atherosclerosis. Saudi J Kidney Dis Transpl 1999;10:503-5
|How to cite this URL:|
Barbullushi M, Pasko N, Bezhani E, Duraku A, Rusi R, Hoti K, Bakalli V, Idrizi A. Secondary Retroperitoneal Fibrosis Associated with Generalized Atherosclerosis. Saudi J Kidney Dis Transpl [serial online] 1999 [cited 2020 Aug 10];10:503-5. Available from: http://www.sjkdt.org/text.asp?1999/10/4/503/37209
| Introduction|| |
Retroperitoneal fibrosis (PRF) is an uncommon disease that often presents in an insidious manner. The etiology is unclear. This process is a chronic inflammatory response to a number of possible inciting factors, including infection, tumor,  atherosclerosis,  and systemic processes such as vasculitis,  lupus,  and other autoallergic reactions. , Evidence has now accumulated to suggest that the condition is an autoimmune periaoritis.  There are only a few reported cases of combined generalized atherosclerosis and PRF.  We describe a typical presentation of RPF associated with generalized atherosclerosis with clinical presentation of progressive renal insufficiency, and claudication from arterial compromise.
| Case Report|| |
A 57-year-old man who had a history hypertension for years presented with progressive renal insufficiency over a period of eight months. Renal work up during this period included a normal intravenous pyelogram and renal ultrasound confirming the presence of obstructive uropathy. Although all the characteristics of RPF were present in this case, including male gender, the correct diagnosis was not determind until obstructive acute renal failure occurred, and the patient was referred to our clinic for further diagnostic evaluation.
Physical examination at admission revealed a blood pressure of 160/95 mm/Hg on the left arm and 190/110 mm/Hg on the right arm. Peripheral pulses were absent on both legs, which were swollen and tender. There were no other apparent skeletal muscle injuries. Fundus oculi was II-III grade hypertensive reinopathy as (Salus Gunn). Examination of ears, nose and throat was negative, and the patients had a normal neurological examination. The rest of the systemic examination was also normal. The urine output was 200 ml/day. An X-ray film of the chest was normal. Abdominal ultrasonography showed bilateral hydronephrosis. Radiography with iodinated contrast media showed that lower one-third of both ureters was pulled towards midline and an almost complete obstruction was present [Figure - 1]. Echocardiology showed left ventricular hypertrophy. Laboratory investigations on admission revealed raised serum creatinine (900 µmol/L), and blood urea nitrogen (BUN) (33 mmol/L). specific urinary gravity was 1.010, proteinuria 2+ and imcrohematuria 2+ were present. Hemoglobin was 92 g/L, white cell count 15,200 cells/µL, platelets 210,000/µL erythrocyte sedimentation rate 40 mm/h, reticulocyte count 3.8%, hematocrit 28% and serum fibrinogen 300 mg/dl. Coagulation parameters, serum immunoglobulines, serum completment, and alkaline and acid phosphatase were normal. Lupus (LE) cells, rheumatoid factor, tumor markers, prostatic specific antigen, anti-nuclear antibodies, anti-DNA antibodies, and Coombs test were negative.
Initially, according to clinical examination, laboratory and radiological investigations, and clinical course, we diagnosed him as having idiopathic RPF.
Alternative diagnoses were very carefully excluded in this case by appropriate investigations. These was no history of any chronic drug intake known to be associated with RF. Past medical history of intermittent claudication (confirmed by Doppler examination of peripheral arteries), high blood pressure different in the two arms, and the absence of peripheral pulses on both legs, suggested the presence of generalized atherosclerosis. Fruthermore, this suggested the association RPF with generalized atherosclerosis. The other possible inciting factors of RPF, including infection, tumor, and systemic processes such as vasculitis, lupus, and other autoallegic reactions were excluded by appropriate investigations.
| Discussion|| |
RPF is an inflammatory fibroproliferative process that affects middle-aged and older patients with a 2:1 male/female predominance., The reaction to advanced inflammatory atherosclerosis with retroperitoneal fibrosis has been studied by a number of investigators. ,, A specific inflammatory response to oxidized lipids, in areas of pre-existing atherosclerosis, has been postulated as one possible inciting event in the resultant so-called "chronic periaortitis".  Ceroid is an oxidized lowdensity lipoprotein (LDL), and it has been proposed that there is an autoallergy to this substance. Autoantibodies to ceriod have been found in patients with periaortritis., This predisposing event promotes an influx of chronic inflammatory cells to the area of atherosclerosis. , Hughes and Buskley  described a predominance of macrophages in areas of periaortitis. Macrophages become lipidladen foam cells in atherosclerotic plaque and have also been found to secrete substances that cause cell proliferation.  Other researchers have found an abundance of B and T lymphocytes, plasma cells, and eosinophils in areas of periartitis. , The infiltrating inflammatory cells are then the source of numerous cytokines, growth factors, and profibronogenic substances., Specifically, macrophages secrete fibroblast growth factor, and T lymphocytes secrete fibroblast growth factor and collagen synthesis stimulating factor  thus resulting in fibroblast proliferation and fibrosis.
In this study, the case described is a typical presentation of RPF associated with generalized atherosclerosis with clinical presentation of renal failure and claudication from arterial compromise.
| References|| |
|1.||Parums DV. Inflammaatory mediators in atherosclerosis. Biochem Soc Trans 1990;18:1069-72. [PUBMED] |
|2.||Witten DM. Retroperitoneal fibrosis. In: Clinical urography, edited by Pollack HM, Philadelphia, WB Saudners Co 1990:2469-83. |
|3.||Baker LR, Whitfield HN. The Patient with urinary tract obstruction. In: Oxford Textbook of clinical Nephrol 1992;3:2019-24. |
|4.||Pryor JP, Castle WM, Dukes DC, Smith JC, Watson ME, Williams JL. Do beta-adreno receptor blocking drugs cause retroperitoneal fibrosis? Br Med J 1983;287:639-41. |
|5.||Hughes D, Buckley PJ. Idiopathic retroperitoneal fibrosis is a macro-phagerich process. Implications for its pathogenesis and treatment. Am J Surg Pathol 1993;17:482-90. |
|6.||Littlejohn GO, Keystone EC. The association of retroperitoneal fibrosis with systemic vasculitis and HLA-B27: a case report and view of the literature. J Rheumatol 1981;8:665-9. [PUBMED] |
|7.||Bashour B. systemic lupus erythe-matousus with retroperitoneal fibrosis and thrombosis of the inferior vena cava. South Med J 1993;86:1309-10. [PUBMED] [FULLTEXT]|
|8.||Ranshaw AL, Roskell DE, Parums DV. Cytokine gene expression in aortic adventitial inflammation associated with advanced atherosclerosis (chronic periaortritis). J Clin Pathol 1994;47:721-7. |
|9.||Parums DV, Choudhury RP, Shields SA, Davies AH. Characterization of inflammatory cells associated with "Idiopathic retroperitoneal fibrosis> Br J Urol 1991;67:56-8. |
|10.||Koep L, Zuidema GD. The clinical significance of retroperitoneal fibrosis. Surgery 1977;81:250-7. [PUBMED] |
|11.||Hartman D. Genitourinary case of the day. Retroperitoneal fibrosis AJR 1994;162:1454-6. |
|12.||Demko TM, Diamond JR, Groff J. Obstructive nephropathy as a result of retroperitoneal fibrosis: a review of its pahogenesis and associations. J Am Soc of Nephrol 1997;685-7. |
Department of Nephrology - Dialysis, University Hospital Center “Mother Teresa”, Rruga e dibres, No. 370, Tirana
[Figure - 1]