Morbus Ormond (idiopatic retroperitoneal fibrosis)

Česká verzia

Authors: A. Michaligová ¹; L. Plank ²; A. Ježíková ¹; V. Maňka ¹; P. Makovický ¹; M. Mokáň ¹
Authors place of work: I. Interná klinika Jesseniovej lekárskej fakulty UK a Univerzitnej nemocnice Martin, Slovenská republika, prednosta prof. MUDr. Marián Mokáň, DrSc., FRCP Edin ¹; Ústav patologickej anatómie Jesseniovej lekárskej fakulty UK a Univerzitnej nemocnice Martin, Slovenská republika, prednosta prof. MUDr. Lukáš Plank, CSc. ²
Published in the journal: Vnitř Lék 2011; 57(5): 511-515
Category: Case Reports

Summary

Idiopathic retroperitoneal fibrosis (IRF) is a rare condition characterized by the development of fibrotic tissue around the abdominal aorta and iliac arteries and often involves structures as ureters and the inferior vena cava. The age at onset of signs and symptoms is between 40–60 years, males predominane over females. In most cases the clinical manifestation is presented as compressive syndrom of ureters, therefore the first known cases were described by urologists. In this report we present the case of 37-years old male examinated for persistent fever about 38 °C and high inflammatory activity in spite of empiric antibiotic therapy. Positron emission tomography (PET) showed locality of high metabolic activity of fluorodeoxyglucose with maximum paraaortal left. Microscopic examination of extracted mass showed presence of fibrous and inflammatory components. With clinical presentation, imaging and histological findings we made out the diagnosis of idiopathic retroperitoneal fibrosis – morbus Ormond.

Key words:
idiopatic retroperitoneal fibrosis – Ormond’s disease – fever

Zdroje

1. Vaglio A, Salvarani C, Buzio C. Retroperitoneal fibrosis. Lancet 2006; 367: 241–251.

2. Albarran J. Rétention rénale par périurétérite. Libération externe de l’uretère. Assoc Fr Urol 1905; 9: 511–517.

3. Ormond JK. Bilateral ureteral obstruction due to envelopment and compression by an inflammatory retroperitoneal process. J Urol 1948; 59: 1072–1079.

4. Uibu T, Oksa P, Auvinen A et al. Asbestos exposure as a risk factor for retroperitoneal fibrosis. Lancet 2004; 363: 1422–1426.

5. Gilkeson GS, Allen NB. Retroperitoneal fibrosis. A true connective tissue disease. Rheum Dis Clin North Am 1996; 22: 356–364.

6. Armigliato M, Paolini R, Bianchini E et al. Hashimoto’s thyroiditis and Graves’ disease associated with retroperitoneal fibrosis. Thyroid 2002; 12: 829–831.

7. Littlejohn GO, Keystone EC. The association of retroperitoneal fibrosis with systemic vasculitis and HLA-B27: a case report and review of the literature. J Rheumatol 1981; 8: 665–669.

8. Vaglio A, Greco P, Corradi D et al. Autoimmune aspect of chronic periaortitis. Autoimmun Rev 2006; 5: 458–464.

9. Vaglio A, Corradi D, Manenti L et al. Evidence of autoimmunity in chronic periaortitis: a prospective study. Am J Med 2003; 114: 454–462.

10. Martorana D, Vaglio A, Greco D et al. Chronic periaortitis and HLA-DRB1*03: another clue to an autoimmune origin. Arthritis Rheum 2006; 55: 126–130.

11. Allibone GW, Saxton HM. The association of aorto-iliac aneurysms with ureteral obstruction. Urol Radiol 1980; 1: 205–210.

12. Lorentzen JE, Sørensen IN, Brun B et al. Abdominal aortic aneurysm in combination with retroperitoneal fibrosis. Acta Chir Scand Suppl 1980; 502: 94–97.

13. Stewart TW jr. Idiopathic retroperitoneal fibrosis – past and present. NY State J Med 1989; 89: 503–504.

14. Wu J, Catalano E, Coppola D. Retroperitoneal fibrosis (Ormond’s disease): clinical pathologic study of eight cases. Cancer Control 2002; 9: 432–437.

15. Serratrice J, Granel B, De Roux B et al. „Coated aorta“: a new sign of Erdheim-Chester disease. J Rheumatol 2000; 6: 1550–1553.

16. van Bommel EF, Siemes C, Hak LE et al. Long-term renal and patient outcome in idiopathic retroperitoneal fibrosis treated with prednison. Am J Kidney Dis 2007; 49: 615–625.

17. Lepor H, Walsh PC. Idiopathic retroperitoneal fibrosis. J Urol 1979; 122: 1–6.

18. Miller OF, Smith LJ, Ferrara EX et al. Presentation of idiopathic retroperitoneal fibrosis in the pediatric population. J Pediatr Surg 2003; 38: 1685–1688.

19. Průcha M, Beňo P, Bartůňek M et al. Idiopatická retroperitoneálna fibróza – Ormondova choroba: kazuistika. Vnitř Lék 2008; 54: 282–286.

20. Jois RN, Kerrigan N, Scott DG. Mycophenolate mofetil for maintenance of remision in idiopathic retroperitoneal fibrosis. Rheumatology (Oxford) 2007; 46: 717–718.