Retroperitoneal fibrosis – diagnosis and treatment
Authors:
Z. Adam 1; A. Čermák 2; Z. Adamová 3; Z. Řehák 4; R. Koukalová 4; L. Pour 1
Authors place of work:
Interní hematologická a onkologická klinika, Lékařská fakulta Masarykovy univerzity a Fakultní nemocnice Brno
1; Urologická klinika Masarykovy univerzity a Fakultní nemocnice Brno
2; Chirurgické oddělení Nemocnice ve Frýdku-Místku, chirurgické oddělení, Vsetínská nemocnice
3; Oddělení nukleární medicíny, Masarykův onkologický ústav Brno
4
Published in the journal:
Rozhl. Chir., 2022, roč. 101, č. 6, s. 265-272.
Category:
Review
doi:
https://doi.org/10.33699/PIS.2022.101.6.265–271
Summary
Idiopathic retroperitoneal fibrosis (IRF) is a rare condition characterized by the development of a peri-aortic and peri-iliac tissue showing chronic inflammatory infiltrates and pronounced fibrosis. Ureteral entrapment with consequent obstructive uropathy is one of the most common complications which can lead to acute renal failure and, in the long term, to varying degrees of chronic kidney disease. Common symptoms at onset include lower back, abdominal or flank pain. Pain is frequently referred to the hip, to the groin and to the lateral regions of the leg, often with nocturnal exacerbations and not responding to position changes. The disease is commonly associated with signs of systemic inflammatory response (malaise, fever, and anorexia and weight loss). Glucocorticoids are considered the cornerstone of the therapy. The use of other immunosuppressive agents, including cyclophosphamide, azathioprine, methotrexate, mycophenolate mofetil and biological agents such as rituximab, tocilizumab and infliximab have been reported as a valuable option mostly in case reports, cases series and small studies. These agents allowed to reduce cumulative dose of glucocorticoids and their adverse effects. Combined therapy is preferable for all patients who suffer from significant glucocorticoid- related toxicity or in cases where glucocorticoids alone are insufficient to treat the condition.
Keywords:
Cyclophosphamide – rituximab – retroperitoneal fibrosis – IgG4 related disease
Zdroje
1. Průcha M, Beňo P, Bartůněk M, et al. Idiopatická retroperitoneální fibróza – Ormondova choroba: kazuistika. Vnitřní lékařství 2008;54(3):282–286.
2. Němec P, Rybníčková S, Fabián P, et al. Idiopathic retroperitoneal fibrosis: an unusual cause of low back pain. Clinical rheumatology 2008;27(3):381–384.
3. Němec P, Rybníčková S, Fabian P, et al. Idiopatická retroperitoneální fibróza: Méně častá příčina bolestí dolní části zad. Použití tamoxifenu v terapii onemocnění. Česká revmatologie 2008;16(1):23–29.
4. Fedorko M, Krhovský M. Perirenální retroperitoneální fibróza. Urologie pro praxi 2009;10(5):299–301.
5. Kašík J, Kolombo I, Porš J, et al. Primární retroperitoneální fibróza – morbus Ormond. Urologie pro praxi 2008;9 (3):128– 132.
6. Sobotka R, Hanuš T. Primární retroperitoneální fibróza, Urologie pro praxi 2006;7(4):156–159.
7. Pulcer J, Staněk R, Kokoř T, et al. Fokální Ormondova choroba (idiopatická retroperitoneální fibróza). Urologie pro praxi 2015;16(Suppl. A).
8. Ormond JK. Bilateral ureteral obstruction due to envelopment and compression by an inflammatory retroperitoneal process. J Urol. 1948;59:1072–107.
9. Kharagjitsing H, van Eekeren R, van Puijenbroek EP, et al. Retroperitoneal fibrosis and β-blocking agents: Is there an association? Br J Clin Pharmacol. 2021;87(7):2891–2901. doi:10.1111/bcp. 14702.
10. Brasselet D, Chouchana L, Vial T, et al. Drug-induced retroperitoneal fibrosis: a case/non-case study in the French Pharmaco Vigilance Database. Expert Opin Drug Saf. 2020;19(7):903–914. doi:1 0.1080/14740338.2020.1766022.
11. van Bommel EF, Jansen I, Hendriksz TR, et al. Idiopathic retroperitoneal fibrosis: prospective evaluation of incidence and clinicoradiologic presentation. Medicine (Baltimore) 2009;88:93–201. doi:10.1097/ MD.0b013e3181afc420.
12. Mizushima I, Kawano M. Renal involvement in retroperitoneal fibrosis: Prevalence, impact and management challenges. Int J Nephrol Renovasc Dis. 2021;14:279–289. doi:10.2147/IJNRD. S239160.
13. Švihra J, Študent V. Retroperitoneálna fibróza. Urologie pro praxi 2016;17(1):14– 16.
14. Průcha M, Kolombo I, Štádler P. Ormond‘ s disease – IgG4-related disease. Prague Medical Report 2015;116 (3):181– 192. doi:10.14712/23362936.2015.57.
15. Posová H. Ormondova nemoc: Imunopatologické stavy v kazuistikách. Mladá fronta, 2016.2016:159–163.
16. Raglianti V, Rossi GM, Vaglio A. Idiopathic retroperitoneal fibrosis: an update for nephrologists. Nephrol Dial Transplant. 2021;36(10):1773–1781. doi:10.1093/ndt/ gfaa083.
17. Fenaroli P, Maritati F, Vaglio A. Into clinical practice: Diagnosis and therapy of retroperitoneal fibrosis. Curr Rheumatol Rep. 2021;23(3):18. doi:10.1007/s11926-020- 00966-9.
18. Kolář J, Pavrovský J, Balaš V, et al. Ileus jako první projev retroperitoneální fibrózy. Rozhledy v chirurgii. 1972;51(6):341–344.
19. Kermani TA, Crowson CS, Achenbach SJ. Idiopathic retroperitoneal fibrosis: a retrospective review of clinical presentation, treatment, and outcomes. Mayo Clin Proc. 2011;86:297–303. doi:10.4065/ mcp.2010.0663.
20. Scheel PJ Jr, Feeley N. Retroperitoneal fibrosis: the clinical, laboratory, and radiographic presentation. Medicine (Baltimore) 2009;88:202–207. doi:10.1016/j. rdc.2013.02.004
21. Cronin CG, Lohan DG, Blake MA, et al. Retroperitoneal fibrosis: a review of clinical features and imaging findings. AJR Am J Roentgenol. 2008;191:423–431. doi:10.2214/AJR.07.3629
22. Vaglio A, Maritati F. Idiopathic Retroperitoneal Fibrosis. JASN 2016;27(7):1880– 1889. doi:10.1681/ASN.2015101110.
23. Marvisi C, Accorsi Buttini E, Vaglio A. Aortitis and periaortitis: The puzzling spectrum of inflammatory aortic diseases. Presse Med. 2020;49(1):104018. doi:10.1016/j.lpm.2020.104018.
24. Nikiphorou E, Galloway J, Fragoulis GE. Overview of IgG4-related aortitis and periaortitis. A decade since their first description. Autoimmun Rev. 2020;19(12):102694. doi:10.1016/j.autrev. 2020.102694.
25. Palmisano A, Urban ML, Corradi D. Chronic periaortitis with thoracic aorta and epiaortic artery involvement: a systemic large vessel vasculitis? Rheumatology (Oxford) 2015;54:2004–2009. doi:10.1093/rheumatology/kev225.
26. Kučera M. Lokalizované fibrotické procesy. Referátový výběr z revmatologie 1999;39:(1):36–40.
27. Kučera M, Kratochvíl P. Lokalizované fibrotické procesy (onemocnění) léčebné možnosti. Referátový výběr z revmatologie 2000;40(4):273–277.
28. Martorana D, Marquez A, Carmona FD, et al. A large-scale genetic analysis reveals an autoimmune origin of idiopathic retroperitoneal fibrosis. J Allergy Clin Immunol. 2018;142(5):1662–1665. doi:10.1016/j.jaci.2018.06.045.
29. Clevenger JA, Wang M, MacLennan GT, et al. Evidence for clonal fibroblast proliferation and autoimmune process in idiopathic retroperitoneal fibrosis. Hum Pathol. 2012;43:1875–1880. doi: 10.1016/j.humpath.2012.01.012.
30. Peisen F, Thaiss WM, Ekert K, et al. Retroperitoneal fibrosis and its differential diagnoses: The role of radiological imaging. Rofo 2020;192(10):929–936. doi:10.1055/a-1181-9205.
31. Kawano M, Saeki T, Nakashima H. IgG4-related kidney disease and retroperitoneal fibrosis: An update. Mod Rheumatol. 2019;29(2):231–239. doi:10.1080/143975 95.2018.1554321.
32. Forestier A, Buob D, Mirault T, et al. No specific imaging pattern can help differentiate IgG4-related disease from idiopathic retroperitoneal fibrosis: 18 histologically proven cases. Clin Exp Rheumatol. 2018;36(3):371–375.
33. Rossi GM, Rocco R, Accorsi Buttini E, et al. Idiopathic retroperitoneal fibrosis and its overlap with IgG4-related disease. A. Intern Emerg Med. 2017;12:287–299. doi:10.1007/s11739-016-1599-z.
34. Laco J, Podhola M, Kamarádová K, et al. Idiopathic vs. secondary retroperitoneal fibrosis: a clinicopathological study of 12 cases, with emphasis to possible relationship to IgG4-related disease. Virchows Archiv 2013;463 (5):721–730. doi:10.1007/ s00428-013-1480-7.
35. Hermanová M. Idiopatická retroperitoneální fibróza – editorial. Vnitřní lékařství 2011;57(5):439–440.
36. Michaligová A, Plank L, Ježíková A, et al. Ormondova choroba (idiopatická retroperitoneálna fibróza). Vnitřní lékařství 2011;57(5):511–515.
37. Miniberger M, Kašpar M, Drechslerová J, et al. Ormondův syndrom (syndrom retroperitoneální fibrózy). Diagnostické obtíže u necharakteristické formy onemocnění při vyšetření výpočetní tomografií. Česká radiologie 1998;52(4):214–220.
38. Řehák Z, Němec P, Fabian P, et al. Využití pozitronové emisní tomografie (18F-FDG PET) v diagnostice idiopatické retroperitoneální fibrózy – kazuistika. Slovenská rádiológia 2009;16(1):68–73.
39. Roussel E, Callemeyn J, Van Moerkercke W. Standardized approach to idiopathic retroperitoneal fibrosis: a comprehensive review of the literature. Acta Clin Belg. 2020;75(4):239–244. doi:10.1080/178432 86.2019.1609152.
40. Wang K, Wang Z, Zeng Q, et al. Clinical characteristics of IgG4-related retroperitoneal fibrosis versus idiopathic retroperitoneal fibrosis. PLoS One. 2021;16(2):e0245601. doi:10.1371/journal. pone.0245601. doi:10.1371/journal. pone.0245601.
41. Novák I, Morávek P, Baše J, et al. Dlouhodobé výsledky léčby nemocných s retroperitoneální fibrózou, Rozhledy v chirurgii 1994;73(6):287–290.
42. Radoňák J, Vajo J, Toncr I, et al. Neobvyklá lokalizovaná forma retroperitoneálnej fibrózy. Rozhledy v chirurgii 1999;78(8):378–380.
43. van Bommel EF, Siemes C, Hak LE, et al. Long-term renal and patient outcome in idiopathic retroperitoneal fibrosis treated with prednisone. Am J Kidney Dis.2007;49:615–625. doi:10.1053/j.ajkd. 2007.02.268.
44. Mizushima I, Inoue D, Yamamoto M, et al. Clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis: a retrospective multicenter study. Arthritis Res Ther. 2014;16:R156. doi:10.1186/ar4671.
45. Brandt AS, Kamper L, Kukuk S, et al. Tamoxifen monotherapy in the treatment of retroperitoneal fibrosis. Urol Int. 2014;93:320–325. doi:10.1055/s-0031-1273450.
46. van Bommel EF, Pelkmans LG, van Damme H, et al. Long-term safety and efficacy of a tamoxifen-based treatment strategy for idiopathic retroperitoneal fibrosis. Eur J Intern Med. 2013;24:444– 450. doi:10.1016/j.ejim.2012.11.010.
47. Vaglio A, Palmisano A, Alberici F, et al. Prednisone versus tamoxifen in patients with idiopathic retroperitoneal fibrosis: an open-label randomized controlled trial. Lancet 2011;378:338–346. doi:10.1016/S0140-6736(11)60934-3.
48. Scavalli AS, Spadaro A, Riccieri V, et al. Long-term follow-up of low-dose methotrexate therapy in one case of idiopathic retroperitoneal fibrosis. Clin Rheumatol. 1995;14(4):481–484. doi:10.1007/ BF02207688.
49. Alberici F, Palmisano A, Urban ML, et al. Methotrexate plus prednisone in patients with relapsing idiopathic retroperitoneal fibrosis. Ann Rheum Dis. 2013;72:1584–1586. doi:10.1136/annrheumdis- 2013-203267.
50. Scavalli AS, Di Rocco G, Giannotti D, et al. Two-years follow-up of low-dose methotrexate and 6-methylprednisolone therapy in a patient with idiopathic retroperitoneal fibrosis. Eur Rev Med Pharmacol Sci. 2012;16(15):2171–2174.
51. Binder M, Uhl M, Wiech T, et al. Cyclophosphamide is a highly effective and safe induction therapy in chronic periaortitis: a long-term follow-up of 35 patients with chronic periaortitis. Ann Rheum Dis. 2012;71:311–312. doi:10.1136/annrheumdis- 2011-200148.
52. Kohler HP, Laeng RH, Egger C, et al. Systemische Fibrose (generalisierte Form des Morbus Ormond). Bericht über einen Fall, der mit Cyclophosphamid und Kortikosteroiden eine komplette Remission erlangte Schweiz Med Wochenschr. 1995;125(44):2131–2136.
53. Colomer Rubio E, Blanes Gallego A, et al. Mesenteric panniculitis with retroperitoneal involvement resolved after treatment with intravenous cyclophosphamide pulses. An Med Interna. 2003;20(1):31–33.
54. Moroni G, Gallelli B, Banfi G, et al. Longterm outcome of idiopathic retroperitoneal fibrosis treated with surgical and/or medical approaches. Nephrol. Dial. Transplant. 2006;21:2485–2490. doi:10.1093/ ndt/gfl228.
55. Průcha M, Kolombo I, Štádler P. Combination of steroids and azathioprine in the treatment of Ormond’s disease – a single centre retrospective analysis. PragueMed Rep. 2016;117:34–41. doi:10.14712/23362936.2016.3.
56. Grotz W, von Zedtwitz I, Andre M, et al. Treatment of retroperitoneal fibrosis by mycophenolate mofetil and corticosteroids. Lancet 1998;352(9135):1195. doi:10.1016/S0140-6736(05)60533-8.
57. Witschi M, Gugger M, Nicod LP. Treatment of mediastinal fibrosis with mycophenolate mofetil. Respiration. 2009;78(3):330– 333. doi:10.1159/000209508.
58. Adler S, Lodermeyer S, Gaa J, et al. Successful mycophenolate mofetil therapy in nine patients with idiopathic retroperitoneal fibrosis. Rheumatology (Oxford) 2008;47(10):1535–1538. doi:10.1093/ rheumatology/ken291.
59. Jois RN, Kerrigan N, Scott DG. Mycophenolate mofetil for maintenance of remission in idiopathic retroperitoneal fibrosis. Rheumatology (Oxford) 2007;46(4):717– 718. doi:10.1093/rheumatology/kel417.
60. Khalil F, Mir MA, Venuto RC. Mycophenolate mofetil in the treatment of retroperitoneal fibrosis. Clin Rheumatol. 2008;27(5):679–681. doi:10.1007/s10067- 007-0826-4.
61. Scheel PJ Jr, Feeley N, Sozio SM. Combined prednisone and mycophenolate mofetil treatment for retroperitoneal fibrosis: a case series. Ann Intern Med. 2011;154:31–36.PMCID:PMC3540636.
62. Scheel PJ Jr, Piccini J, Rahman MH, et al. Combined prednisone and mycophenolate mofetil treatment for retroperitoneal fibrosis. J Urol. 2007;178(1):140–143. doi:10.1016/j.juro.2007.03.057.
63. Swartz RD, Lake AM, Roberts WW, et al. Idiopathic retroperitoneal fibrosis: a role for mycophenolate mofetil. Clin Nephrol. 2008;69(4):260–268. doi:10.5414/ cnp69260.
64. Marzano A, Trapani A, Leone N, et al. Treatment of idiopathic retroperitoneal fibrosis using cyclosporin. Ann Rheum Dis. 2001;60(4):427–428. doi:10.1136/ ard.60.4.427.
65. Al-Harthy F, Esdaile J, Berean KW, et al. Multifocal idiopathic fibrosclerosis: treatment of 2 cases with cyclosporine. J Rheumatol. 2006;33(2):358–361. PMID:16465670.
66. Marcolongo R, Tavolini IM, Laveder F, et al. Immunosuppressive therapy for idiopathic retroperitoneal fibrosis: a retrospective analysis of 26 cases. Am J Med. 2004;116:194–197. doi:10.1016/j.amjmed. 2003.08.033.
67. Maritati F, Corradi D, Versari A, et al. Rituximab therapy for chronic periaortitis. Ann Rheum Dis. 2012;71:1262–1264. doi:10.1136/annrheumdis-2011-201166.
68. Almeqdadi M, Al-Dulaimi M, Perepletchikov A, et al. Rituximab for retroperitoneal fibrosis due to IgG4-related disease: A case report and literature review. Clin Nephrol Case Stud. 2018;6:4–10. doi:10.1136/annrheumdis-2011-201166.
69. Carruthers MN, Topazian MD, Khosroshahi A, et al. Rituximab for IgG4-related disease: a prospective, open-label trial. Ann Rheum Dis. 2015;74:1171–1177. doi:10.1136/annrheumdis-2014-206605.
70. Dedinská I, Svetlík D, Adamicova K, et al. Treatment of Ormond disease and idiopathic membranous glomerulonephritis by using rituximab. Iran J Kidney Dis. 2016;10(5):332–335.
71. Hamdan A, Moeen Z, Tariq H, et al. An interesting case of immunoglobulin G4-related retroperitoneal fibrosis treated with rituximab. Cureus 2021;13(9):e17940. doi:10.7759/cureus.17940.
72. Wallwork R, Wallace Z, Perugino C, et al. Rituximab for idiopathic and IgG4-related retroperitoneal fibrosis. Medicine (Baltimore) 2018;97(42):e12631. doi:10.1097/ MD.0000000000012631.
73. Boyeva V, Alabsi H, Seidman MA, et al. Use of rituximab in idiopathic; retroperitoneal fibrosis. BMC Rheumatol. 2020;4:40. doi:10.1186/s41927-020-00140-9.
74. Urban ML, Maritati F, Palmisano A, et al. Rituximab for chronic periaortitis without evidence of IgG4-related disease: a longterm follow-up study of 20 patients. Ann Rheum Dis. 2020;79(3):433–434. doi:10.1136/annrheumdis-2019-216258.
75. Benucci M, Damiani A, Li Gobbi F, et al. Jaccoud‘s arthropathy, an unusual manifestation of idiopathic retroperitoneal fibrosis: rapid improvement of symptoms after tocilizumab treatment. Reumatismo. 2017;69 (2):88–91. doi:10.4081/reumatismo. 2017.968.
76. Perrotta FM, Fici M, Guerra G, et al. Chronic periaortitis with retroperitoneal fibrosis successfully treated with first line tocilizumab monotherapy: a case report. Clin Exp Rheumatol. 2017;35 Suppl 103(1):226–227.
77. Vaglio A, Catanoso MG, Spaggiari L, et al. Interleukin-6 as an inflammatory mediator and target of therapy in chronic periaortitis. Arthritis Rheum. 2013;65(9):2469–2475. doi:10.1002/ art.38032.
78. Loricera J, Blanco R, Castaeda S, et al. Tocilizumab in refractory aortitis: study on 16 patients and literature review. Clin Exp Rheumatol. 2014;32(3 Suppl 82):S79–89.
79. Catanoso MG, Spaggiari L, Magnani L, et al. Efficacy of infliximab in a patient with refractory idiopathic retroperitoneal fibrosis. Clin Exp Rheumatol. 2012;30(5):776–778.
80. Pelkmans LG, Aarnoudse AJ, Hendriksz TR, et al. Value of acute-phase reactants in monitoring disease activity and treatment response in idiopathic retroperitoneal fibrosis. Nephrol Dial Transplant. 2012;27:2819–2825. doi:10.1093/ ndt/gfr779.
81. Vaglio A, Versari A, Fraternali A, et al. (18) F-fluorodeoxyglucose positron emission tomography in the diagnosis and followup of idiopathic retroperitoneal fibrosis. Arthritis Rheum 2005;53:122–125. doi:10.1002/art.20914.
82. Jansen I, Hendriksz TR, Han SH, et al. 18F-fluorodeoxyglucose position emission tomography (FDG-PET) for monitoring disease activity and treatment response in idiopathic retroperitoneal fibrosis. Eur J Intern Med 2010;21:216–221. doi:10.1016/j.ejim.2010.02.008.
83. Němec P, Řehák Z, Fabián P. Využití pozitronové emisní tomografie (18F-FDG PET) v diagnostice chronických periaortitid. Vnitřní lékařství 2008;54(11):1093– 1099. PMID:19069683.
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