Internal and Emergency Medicine

, Volume 12, Issue 3, pp 287–299 | Cite as

Idiopathic retroperitoneal fibrosis and its overlap with IgG4-related disease

  • Giovanni Maria Rossi
  • Rossana Rocco
  • Eugenia Accorsi Buttini
  • Chiara Marvisi
  • Augusto VaglioEmail author


Retroperitoneal fibrosis (RPF) is a rare disease characterised by fibrous tissue proliferation in the retroperitoneum, with encasement of the ureters and large vessels of the abdomen as the most destructive of potentially severe complications. It can either be idiopathic, or secondary to infections, malignancies, or the use of certain drugs. The idiopathic form accounts for approximately 75% of the cases, and is usually responsive to immunosuppressive therapy. In recent years, the emergence of a new clinical entity, IgG4-related disease (IgG4-RD), shed light on many fibro-inflammatory disorders once thought to be separate clinical entities, although frequently associated in the so-called multifocal fibrosclerosis. Among these, together with sclerosing pancreatitis and cholangitis, pseudotumour of the orbit, idiopathic mediastinal fibrosis and other conditions, is idiopathic retroperitoneal fibrosis (IRF). Both IRF and IgG4-RD can be associated with a wide variety of disorders, usually governed by immune-mediated (and particularly auto-immune) mechanisms. In our review, we discuss the clinical and therapeutic challenges IRF presents to the internist, as well as the meaning of its recent inclusion in the IgG4-RD spectrum from a clinical practice standpoint.


Retroperitoneal fibrosis IgG4 ANCA Hashimoto Periaortitis Hydronephrosis Vasculitis 



Retroperitoneal fibrosis


Idiopathic retroperitoneal fibrosis


Anti-neutrophil cytoplasmic antibody


IgG4-related disease


Glomerular filtration rate




Computed tomography


Magnetic resonance imaging


Hepatitis B virus


Hepatitis C virus


Human immunodeficiency virus


Anti-nuclear antibodies


18F-fluorodeoxyglucose positron emission tomography


Acute kidney injury


Chronic kidney disease


Compliance with ethical standards

Conflict of interest

The authors have no conflict of interest with the publication of this article.

Statement of human and animal rights

This article was written in respect of human rights.

Informed consent

Informed consent was obtained from patients whose computed tomography scans are shown in the paper.


There was no specific funding for this work.


  1. 1.
    Vaglio A, Maritati F (2016) Idiopathic retroperitoneal fibrosis. J Am Soc Nephrol 27(7):1880–1889. doi: 10.1681/ASN.2015101110 CrossRefPubMedGoogle Scholar
  2. 2.
    Stone JH, Zen Y, Deshpande V (2012) IgG4-related disease. N Engl J Med 366(6):539–551. doi: 10.1056/NEJMra1104650 CrossRefPubMedGoogle Scholar
  3. 3.
    Palmisano A, Vaglio A (2009) Chronic periaortitis: a fibro-inflammatory disorder. Best Pract Res Clin Rheumatol 23(3):339–353. doi: 10.1016/j.berh.2008.12.002 CrossRefPubMedGoogle Scholar
  4. 4.
    Perugino CA, Wallace ZS, Meyersohn N, Oliveira G, Stone JR, Stone JH (2016) Large vessel involvement by IgG4-related disease. Medicine (Baltimore) 95(28):e3344. doi: 10.1097/md.0000000000003344 CrossRefGoogle Scholar
  5. 5.
    Vaglio A, Pipitone N, Salvarani C (2011) Chronic periaortitis: a large-vessel vasculitis? Curr Opin Rheumatol 23(1):1–6. doi: 10.1097/BOR.0b013e328341137d CrossRefPubMedGoogle Scholar
  6. 6.
    Palmisano A, Urban ML, Corradi D, Cobelli R, Alberici F, Maritati F, Versari A, Pipitone N, Salvarani C, Buzio C, Vaglio A (2015) Chronic periaortitis with thoracic aorta and epiaortic artery involvement: a systemic large vessel vasculitis? Rheumatology (Oxford) 54(11):2004–2009. doi: 10.1093/rheumatology/kev225 CrossRefGoogle Scholar
  7. 7.
    Pipitone N, Vaglio A, Salvarani C (2012) Retroperitoneal fibrosis. Best Pract Res Clin Rheumatol 26(4):439–448. doi: 10.1016/j.berh.2012.07.004 CrossRefPubMedGoogle Scholar
  8. 8.
    Vaglio A, Salvarani C, Buzio C (2006) Retroperitoneal fibrosis. Lancet (London, England) 367 (9506):241–251. doi: 10.1016/S0140-6736(06)68035-5
  9. 9.
    Urban ML, Palmisano A, Nicastro M, Corradi D, Buzio C, Vaglio A (2015) Idiopathic and secondary forms of retroperitoneal fibrosis: a diagnostic approach. Rev Med Interne 36(1):15–21. doi: 10.1016/j.revmed.2014.10.008 CrossRefPubMedGoogle Scholar
  10. 10.
    Greco P, Vaglio A, Corradi D, Cobelli R, Zompatori M, Buzio C (2005) Tuberculosis as a trigger of retroperitoneal fibrosis. Clin Infect Dis 41(7):e72–e75. doi: 10.1086/433189 CrossRefPubMedGoogle Scholar
  11. 11.
    Alberti C (2015) Drug-induced retroperitoneal fibrosis: short aetiopathogenetic note, from the past times of ergot-derivatives large use to currently applied bio-pharmacology. G Chir 36(4):187–191PubMedPubMedCentralGoogle Scholar
  12. 12.
    Gianfreda D, Musetti C, Nicastro M, Maritati F, Cobelli R, Corradi D, Vaglio A (2016) Erdheim–Chester disease as a mimic of IgG4-related disease: a case report and a review of a single-center cohort. Medicine (Baltimore) 95(21):e3625. doi: 10.1097/MD.0000000000003625 CrossRefGoogle Scholar
  13. 13.
    van Bommel EF, Jansen I, Hendriksz TR, Aarnoudse AL (2009) Idiopathic retroperitoneal fibrosis: prospective evaluation of incidence and clinicoradiologic presentation. Medicine (Baltimore) 88(4):193–201. doi: 10.1097/MD.0b013e3181afc420 CrossRefGoogle Scholar
  14. 14.
    Kermani TA, Crowson CS, Achenbach SJ, Luthra HS (2011) Idiopathic retroperitoneal fibrosis: a retrospective review of clinical presentation, treatment, and outcomes. Mayo Clin Proc 86(4):297–303. doi: 10.4065/mcp.2010.0663 CrossRefPubMedPubMedCentralGoogle Scholar
  15. 15.
    Magrey MN, Husni ME, Kushner I, Calabrese LH (2009) Do acute-phase reactants predict response to glucocorticoid therapy in retroperitoneal fibrosis? Arthritis Rheum 61(5):674–679. doi: 10.1002/art.24461 CrossRefPubMedGoogle Scholar
  16. 16.
    Pelkmans LG, Aarnoudse AJ, Hendriksz TR, van Bommel EF (2012) Value of acute-phase reactants in monitoring disease activity and treatment response in idiopathic retroperitoneal fibrosis. Nephrol Dial Transplant 27(7):2819–2825. doi: 10.1093/ndt/gfr779 CrossRefPubMedGoogle Scholar
  17. 17.
    Wilson MW, Milks MW (2015) Acute renal vein thrombosis and nephrotic syndrome in the setting of retroperitoneal fibrosis. Oxf Med Case Rep 6:309–310. doi: 10.1093/omcr/omv043 CrossRefGoogle Scholar
  18. 18.
    Palmisano A, Cobelli R, Buzio C, Vaglio A (2009) Peri-renal collateral circles. Urology 74(2):292–293. doi: 10.1016/j.urology.2009.02.052 CrossRefPubMedGoogle Scholar
  19. 19.
    Scheel PJ Jr, Feeley N (2009) Retroperitoneal fibrosis: the clinical, laboratory, and radiographic presentation. Medicine (Baltimore) 88(4):202–207. doi: 10.1097/MD.0b013e3181afc439 CrossRefGoogle Scholar
  20. 20.
    Erbel R, Aboyans V, Boileau C, Bossone E, Bartolomeo RD, Eggebrecht H, Evangelista A, Falk V, Frank H, Gaemperli O, Grabenwoger M, Haverich A, Iung B, Manolis AJ, Meijboom F, Nienaber CA, Roffi M, Rousseau H, Sechtem U, Sirnes PA, Allmen RS, Vrints CJ; Guidelines ESCCfP (2014) 2014 ESC guidelines on the diagnosis and treatment of aortic diseases: document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC). Eur Heart J 35 (41):2873–2926. doi: 10.1093/eurheartj/ehu281
  21. 21.
    Erbel R, Aboyans V, Boileau C, Bossone E, Di Bartolomeo R, Eggebrecht H, Evangelista A, Falk V, Frank H, Gaemperli O, Grabenwoger M, Haverich A, Iung B, John Manolis A, Meijboom F, Nienaber CA, Roffi M, Rousseau H, Sechtem U, Sirnes PA, von Allmen RS, Authors/Task Force m (2015) Corrigendum to: 2014 ESC guidelines on the diagnosis and treatment of aortic diseases. Eur Heart J 36(41):2779. doi: 10.1093/eurheartj/ehv178 CrossRefPubMedGoogle Scholar
  22. 22.
    Caiafa RO, Vinuesa AS, Izquierdo RS, Brufau BP, Ayuso Colella JR, Molina CN (2013) Retroperitoneal fibrosis: role of imaging in diagnosis and follow-up. Radiographics 33 (2):535–552. doi: 10.1148/rg.332125085
  23. 23.
    Williams RG, Nelson JA (1978) Retractile mesenteritis: initial presentation as colonic obstruction. Radiology 126(1):35–37. doi: 10.1148/126.1.35 CrossRefPubMedGoogle Scholar
  24. 24.
    Cronin CG, Lohan DG, Blake MA, Roche C, McCarthy P, Murphy JM (2008) Retroperitoneal fibrosis: a review of clinical features and imaging findings. AJR Am J Roentgenol 191(2):423–431. doi: 10.2214/AJR.07.3629 CrossRefPubMedGoogle Scholar
  25. 25.
    Rubenstein WA, Gray G, Auh YH, Honig CL, Thorbjarnarson B, Williams JJ, Haimes AB, Zirinsky K, Kazam E (1986) CT of fibrous tissues and tumors with sonographic correlation. AJR Am J Roentgenol 147(5):1067–1074. doi: 10.2214/ajr.147.5.1067 CrossRefPubMedGoogle Scholar
  26. 26.
    Degesys GE, Dunnick NR, Silverman PM, Cohan RH, Illescas FF, Castagno A (1986) Retroperitoneal fibrosis: use of CT in distinguishing among possible causes. AJR Am J Roentgenol 146(1):57–60. doi: 10.2214/ajr.146.1.57 CrossRefPubMedGoogle Scholar
  27. 27.
    Bakir B, Yilmaz F, Turkay R, Ozel S, Bilgic B, Velioglu A, Saka B, Salmaslioglu A (2014) Role of diffusion-weighted MR imaging in the differentiation of benign retroperitoneal fibrosis from malignant neoplasm: preliminary study. Radiology 272(2):438–445. doi: 10.1148/radiol.14131565 CrossRefPubMedGoogle Scholar
  28. 28.
    Vaglio A, Versari A, Fraternali A, Ferrozzi F, Salvarani C, Buzio C (2005) (18)F-fluorodeoxyglucose positron emission tomography in the diagnosis and followup of idiopathic retroperitoneal fibrosis. Arthritis Rheum 53(1):122–125. doi: 10.1002/art.20914 CrossRefPubMedGoogle Scholar
  29. 29.
    Jansen I, Hendriksz TR, Han SH, Huiskes AW, van Bommel EF (2010) (18)F-fluorodeoxyglucose position emission tomography (FDG-PET) for monitoring disease activity and treatment response in idiopathic retroperitoneal fibrosis. Eur J Intern Med 21(3):216–221. doi: 10.1016/j.ejim.2010.02.008 CrossRefPubMedGoogle Scholar
  30. 30.
    Pipitone N, Ghinoi A, Versari A, Vaglio A, Palmisano A, Salvarani C (2008) Images in cardiovascular medicine. Chronic periaortitis. Circulation 118(11):1214–1216. doi: 10.1161/CIRCULATIONAHA.108.766493 CrossRefPubMedGoogle Scholar
  31. 31.
    Rossi GM, Emmi G, Corradi D, Urban ML, Maritati F, Landini F, Galli P, Palmisano A, Vaglio A (2016) Idiopathic mediastinal fibrosis: a systemic immune-mediated disorder. Clin Rev Allergy Immunol, A Case Series and a Review of the Literature. doi: 10.1007/s12016-016-8584-1 Google Scholar
  32. 32.
    Anderton SM, Fillatreau S (2008) Activated B cells in autoimmune diseases: the case for a regulatory role. Nat Clin Pract Rheumatol 4(12):657–666. doi: 10.1038/ncprheum0950 CrossRefPubMedGoogle Scholar
  33. 33.
    Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, Matsui S, Yoshino T, Nakamura S, Kawa S, Hamano H, Kamisawa T, Shimosegawa T, Shimatsu A, Nakamura S, Ito T, Notohara K, Sumida T, Tanaka Y, Mimori T, Chiba T, Mishima M, Hibi T, Tsubouchi H, Inui K, Ohara H (2011) Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD). Mod Rheumatol 22(1):21–30. doi: 10.1007/s10165-011-0571-z CrossRefGoogle Scholar
  34. 34.
    Deshpande V, Zen Y, Chan JK, Yi EE, Sato Y, Yoshino T, Kloppel G, Heathcote JG, Khosroshahi A, Ferry JA, Aalberse RC, Bloch DB, Brugge WR, Bateman AC, Carruthers MN, Chari ST, Cheuk W, Cornell LD, Fernandez-Del Castillo C, Forcione DG, Hamilos DL, Kamisawa T, Kasashima S, Kawa S, Kawano M, Lauwers GY, Masaki Y, Nakanuma Y, Notohara K, Okazaki K, Ryu JK, Saeki T, Sahani DV, Smyrk TC, Stone JR, Takahira M, Webster GJ, Yamamoto M, Zamboni G, Umehara H, Stone JH (2012) Consensus statement on the pathology of IgG4-related disease. Mod Pathol 25(9):1181–1192. doi: 10.1038/modpathol.2012.72
  35. 35.
    Swartz RD (2009) Idiopathic retroperitoneal fibrosis: a review of the pathogenesis and approaches to treatment. Am J Kidney Dis 54(3):546–553. doi: 10.1053/j.ajkd.2009.04.019 CrossRefPubMedGoogle Scholar
  36. 36.
    Castelein T, Coudyzer W, Blockmans D (2015) IgG4-related periaortitis vs idiopathic periaortitis: is there a role for atherosclerotic plaque in the pathogenesis of IgG4-related periaortitis? Rheumatology (Oxford) 54(7):1250–1256. doi: 10.1093/rheumatology/keu462 CrossRefGoogle Scholar
  37. 37.
    Goldoni M, Bonini S, Urban ML, Palmisano A, De Palma G, Galletti E, Coggiola M, Buzio C, Mutti A, Vaglio A (2014) Asbestos and smoking as risk factors for idiopathic retroperitoneal fibrosis: a case-control study. Ann Intern Med 161(3):181–188. doi: 10.7326/M13-2648 CrossRefPubMedGoogle Scholar
  38. 38.
    Swartz RD (2015) Retroperitoneal fibrosis and asbestosis—a plausible association? Am J Kidney Dis 65(3):378–380. doi: 10.1053/j.ajkd.2014.09.009 CrossRefPubMedGoogle Scholar
  39. 39.
    Vaglio A, Palmisano A, Alberici F, Maggiore U, Ferretti S, Cobelli R, Ferrozzi F, Corradi D, Salvarani C, Buzio C (2011) Prednisone versus tamoxifen in patients with idiopathic retroperitoneal fibrosis: an open-label randomised controlled trial. Lancet 378(9788):338–346. doi: 10.1016/s0140-6736(11)60934-3 CrossRefPubMedGoogle Scholar
  40. 40.
    Ceresini G, Urban ML, Corradi D, Lauretani F, Marina M, Usberti E, Palmisano A, Buzio C, Vaglio A (2015) Association between idiopathic retroperitoneal fibrosis and autoimmune thyroiditis: a case-control study. Autoimmun Rev 14(1):16–22. doi: 10.1016/j.autrev.2014.08.006 CrossRefPubMedGoogle Scholar
  41. 41.
    Fernandez-Lopez R, Lojo JA, Acevedo-Banez I, Gonzalez-Leon R, Borrego-Dorado I (2016) (18)F-FDG PET/CT in patients with idiopathic retroperitoneal and mediastinal fibrosis. Eur J Nucl Med Mol Imaging 43(9):1739–1740. doi: 10.1007/s00259-016-3342-4 CrossRefPubMedGoogle Scholar
  42. 42.
    Fofi C, Prosperi D, Pettorini L, Festuccia F, Pirisino R, Lanni V, Scopinaro F, Punzo G, Mene P (2016) Diagnosis and follow-up of idiopathic retroperitoneal fibrosis: role of (18)F-FDG-PET/CT and biochemical parameters in patients with renal involvement. Intern Emerg Med 11(6):809–816. doi: 10.1007/s11739-016-1426-6 CrossRefPubMedGoogle Scholar
  43. 43.
    Ruhlmann V, Poeppel TD, Brandt AS, Gruneisen J, Ruhlmann M, Theysohn JM, Forsting M, Bockisch A, Umutlu L (2016) (18)F-FDG PET/MRI evaluation of retroperitoneal fibrosis: a simultaneous multiparametric approach for diagnosing active disease. Eur J Nucl Med Mol Imaging 43(9):1646–1652. doi: 10.1007/s00259-016-3351-3 CrossRefPubMedGoogle Scholar
  44. 44.
    Grotz W, von Zedtwitz I, Andre M, Schollmeyer P (1998) Treatment of retroperitoneal fibrosis by mycophenolate mofetil and corticosteroids. Lancet (London, England) 352 (9135):1195. doi: 10.1016/s0140-6736(05)60533-8
  45. 45.
    Jois RN, Kerrigan N, Scott DG (2007) Mycophenolate mofetil for maintenance of remission in idiopathic retroperitoneal fibrosis. Rheumatology (Oxford) 46(4):717–718. doi: 10.1093/rheumatology/kel417 CrossRefGoogle Scholar
  46. 46.
    Scheel PJ Jr, Feeley N, Sozio SM (2011) Combined prednisone and mycophenolate mofetil treatment for retroperitoneal fibrosis: a case series. Ann Intern Med 154(1):31–36. doi: 10.7326/0003-4819-154-1-201101040-00005 CrossRefPubMedGoogle Scholar
  47. 47.
    Scheel PJ Jr, Piccini J, Rahman MH, Lawler L, Jarrett T (2007) Combined prednisone and mycophenolate mofetil treatment for retroperitoneal fibrosis. J Urol 178(1):140–143. doi: 10.1016/j.juro.2007.03.057 (Discussion 143–144)
  48. 48.
    Swartz RD, Lake AM, Roberts WW, Faerber GJ, Wolf JS Jr (2008) Idiopathic retroperitoneal fibrosis: a role for mycophenolate mofetil. Clin Nephrol 69(4):260–268CrossRefPubMedGoogle Scholar
  49. 49.
    Alberici F, Palmisano A, Urban ML, Maritati F, Oliva E, Manenti L, Ferretti S, Cobelli R, Buzio C, Vaglio A (2013) Methotrexate plus prednisone in patients with relapsing idiopathic retroperitoneal fibrosis. Ann Rheum Dis 72(9):1584–1586. doi: 10.1136/annrheumdis-2013-203267 CrossRefPubMedGoogle Scholar
  50. 50.
    Scavalli AS, Spadaro A, Riccieri V, Ricciuti GP, Taccari E, Marini M, Zoppini A (1995) Long-term follow-up of low-dose methotrexate therapy in one case of idiopathic retroperitoneal fibrosis. Clin Rheumatol 14(4):481–484CrossRefPubMedGoogle Scholar
  51. 51.
    Prucha M, Kolombo I, Stadler P (2016) Combination of steroids and azathioprine in the treatment of Ormond’s disease—a single centre retrospective analysis. Prague Med Rep 117(1):34–41. doi: 10.14712/23362936.2016.3 CrossRefPubMedGoogle Scholar
  52. 52.
    Binder M, Uhl M, Wiech T, Kollert F, Thiel J, Sass JO, Walker UA, Peter HH, Warnatz K (2012) 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 71(2):311–312. doi: 10.1136/annrheumdis-2011-200148 CrossRefPubMedGoogle Scholar
  53. 53.
    Loricera J, Blanco R, Castaneda S, Humbria A, Ortego-Centeno N, Narvaez J, Mata C, Melchor S, Aurrecoechea E, Calvo-Alen J, Lluch P, Moll C, Minguez M, Herrero-Beaumont G, Bravo B, Rubio E, Freire M, Peiro E, Gonzalez-Vela C, Rueda-Gotor J, Pina T, Palmou-Fontana N, Calvo-Rio V, Ortiz-Sanjuan F, Gonzalez-Gay MA (2014) Tocilizumab in refractory aortitis: study on 16 patients and literature review. Clin Exp Rheumatol 32(3 Suppl 82):S79–S89PubMedGoogle Scholar
  54. 54.
    Maritati F, Corradi D, Versari A, Casali M, Urban ML, Buzio C, Vaglio A (2012) Rituximab therapy for chronic periaortitis. Ann Rheum Dis 71(7):1262–1264. doi: 10.1136/annrheumdis-2011-201166 CrossRefPubMedGoogle Scholar
  55. 55.
    Wagenknecht LV (1987) Ureteral endosplints: a quiet revolution of urological treatment. Scand J Urol Nephrol Suppl 104:133–139PubMedGoogle Scholar
  56. 56.
    Pocock RD, Stower MJ, Ferro MA, Smith PJ, Gingell JC (1986) Double J stents. A review of 100 patients. Br J Urol 58(6):629–633CrossRefPubMedGoogle Scholar
  57. 57.
    Schlick RW, Seidl EM, Kalem T, Volkmer B, Planz K (1998) New endoureteral double-J stent resists extrinsic ureteral compression. J Endourol 12(1):37–40. doi: 10.1089/end.1998.12.37 CrossRefPubMedGoogle Scholar
  58. 58.
    Hausegger KA, Portugaller HR (2006) Percutaneous nephrostomy and antegrade ureteral stenting: technique-indications-complications. Eur Radiol 16(9):2016–2030. doi: 10.1007/s00330-005-0136-7 CrossRefPubMedGoogle Scholar
  59. 59.
    Mertens S, Zeegers AG, Wertheimer PA, Hendriksz TR, van Bommel EF (2014) Efficacy and complications of urinary drainage procedures in idiopathic retroperitoneal fibrosis complicated by extrinsic ureteral obstruction. Int J Urol 21(3):283–288. doi: 10.1111/iju.12234 CrossRefPubMedGoogle Scholar
  60. 60.
    Cristian S, Cristian M, Cristian P, Constantin G, Savu C, Huri E, Sinescu I (2015) Management of idiopathic retroperitoneal fibrosis from the urologist’s perspective. Therap Adv Urol 7(2):85–99. doi: 10.1177/1756287214565637 CrossRefGoogle Scholar
  61. 61.
    Hamano H, Kawa S, Horiuchi A, Unno H, Furuya N, Akamatsu T, Fukushima M, Nikaido T, Nakayama K, Usuda N, Kiyosawa K (2001) High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med 344(10):732–738. doi: 10.1056/nejm200103083441005 CrossRefPubMedGoogle Scholar
  62. 62.
    Kamisawa T, Egawa N, Nakajima H (2003) Autoimmune pancreatitis is a systemic autoimmune disease. Am J Gastroenterol 98(12):2811–2812. doi: 10.1111/j.1572-0241.2003.08758.x CrossRefPubMedGoogle Scholar
  63. 63.
    Kamisawa T, Funata N, Hayashi Y, Eishi Y, Koike M, Tsuruta K, Okamoto A, Egawa N, Nakajima H (2003) A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol 38(10):982–984. doi: 10.1007/s00535-003-1175-y CrossRefPubMedGoogle Scholar
  64. 64.
    Kamisawa T, Zen Y, Pillai S, Stone JH (2015) IgG4-related disease. Lancet (London, England) 385(9976):1460–1471. doi: 10.1016/s0140-6736(14)60720-0
  65. 65.
    Kamisawa T, Shimosegawa T, Okazaki K, Nishino T, Watanabe H, Kanno A, Okumura F, Nishikawa T, Kobayashi K, Ichiya T, Takatori H, Yamakita K, Kubota K, Hamano H, Okamura K, Hirano K, Ito T, Ko SB, Omata M (2009) Standard steroid treatment for autoimmune pancreatitis. Gut 58(11):1504–1507. doi: 10.1136/gut.2008.172908 CrossRefPubMedGoogle Scholar
  66. 66.
    Tabata T, Kamisawa T, Takuma K, Anjiki H, Egawa N, Kurata M, Honda G, Tsuruta K, Setoguchi K, Obayashi T, Sasaki T (2009) Serum IgG4 concentrations and IgG4-related sclerosing disease. Clin Chim Acta 408(1–2):25–28. doi: 10.1016/j.cca.2009.06.036 CrossRefPubMedGoogle Scholar
  67. 67.
    Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, Matsui S, Sumida T, Mimori T, Tanaka Y, Tsubota K, Yoshino T, Kawa S, Suzuki R, Takegami T, Tomosugi N, Kurose N, Ishigaki Y, Azumi A, Kojima M, Nakamura S, Inoue D; Research Program for Intractable Disease by Ministry of Health L, Welfare Japan Gt (2012) A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details. Mod Rheumatol 22(1):1–14. doi: 10.1007/s10165-011-0508-6
  68. 68.
    Yamamoto M, Takahashi H (2016) IgG4-related disease in organs other than the hepatobiliary-pancreatic system. Semin Liver Dis 36(3):274–282. doi: 10.1055/s-0036-1584317 CrossRefPubMedGoogle Scholar
  69. 69.
    Brito-Zeron P, Ramos-Casals M, Bosch X, Stone JH (2014) The clinical spectrum of IgG4-related disease. Autoimmun Rev 13(12):1203–1210. doi: 10.1016/j.autrev.2014.08.013 CrossRefPubMedGoogle Scholar
  70. 70.
    Zen Y, Onodera M, Inoue D, Kitao A, Matsui O, Nohara T, Namiki M, Kasashima S, Kawashima A, Matsumoto Y, Katayanagi K, Murata T, Ishizawa S, Hosaka N, Kuriki K, Nakanuma Y (2009) Retroperitoneal fibrosis: a clinicopathologic study with respect to immunoglobulin G4. Am J Surg Pathol 33(12):1833–1839CrossRefPubMedGoogle Scholar
  71. 71.
    Yamashita K, Haga H, Mikami Y, Kanematsu A, Nakashima Y, Kotani H, Ogawa O, Manabe T (2008) Degree of IgG4+ plasma cell infiltration in retroperitoneal fibrosis with or without multifocal fibrosclerosis. Histopathology 52(3):404–409. doi: 10.1111/j.1365-2559.2007.02959.x CrossRefPubMedGoogle Scholar
  72. 72.
    Koo BS, Koh YW, Hong S, Kim YJ, Kim YG, Lee CK, Yoo B (2015) Clinicopathologic characteristics of IgG4-related retroperitoneal fibrosis among patients initially diagnosed as having idiopathic retroperitoneal fibrosis. Mod Rheumatol 25(2):194–198. doi: 10.3109/14397595.2014.931908 CrossRefPubMedGoogle Scholar
  73. 73.
    Khosroshahi A, Carruthers MN, Stone JH, Shinagare S, Sainani N, Hasserjian RP, Deshpande V (2013) Rethinking Ormond’s disease: “idiopathic” retroperitoneal fibrosis in the era of IgG4-related disease. Medicine (Baltimore) 92(2):82–91. doi: 10.1097/MD.0b013e318289610f CrossRefGoogle Scholar
  74. 74.
    Kasashima S, Zen Y, Kawashima A, Endo M, Matsumoto Y, Kasashima F (2009) A new clinicopathological entity of IgG4-related inflammatory abdominal aortic aneurysm. J Vasc Surg 49 (5):1264–1271. doi: 10.1016/j.jvs.2008.11.072 (Discussion 1271)
  75. 75.
    Dedinska I, Svetlik D, Adamicova K, Machalekova K, Makovicky P, Jezikova A, Laca L, Miklusica J, Galajda P, Mokan M (2016) Treatment of Ormond disease and idiopathic membranous glomerulonephritis by using rituximab. Iran J Kidney Dis 10(5):332–335PubMedGoogle Scholar
  76. 76.
    Carruthers MN, Topazian MD, Khosroshahi A, Witzig TE, Wallace ZS, Hart PA, Deshpande V, Smyrk TC, Chari S, Stone JH (2015) Rituximab for IgG4-related disease: a prospective, open-label trial. Ann Rheum Dis 74(6):1171–1177. doi: 10.1136/annrheumdis-2014-206605 CrossRefPubMedGoogle Scholar
  77. 77.
    Willscher MK, Mozden PJ, Olsson CA (1978) Retroperitoneal fibrosis with ureteral obstruction secondary to Actinomyces israeli. Urology 12(5):569–571CrossRefPubMedGoogle Scholar
  78. 78.
    Niemann N, Hochman FL, Huang RS (2014) Histoplasmosis as a possible cause of retroperitoneal fibrosis and median arcuate ligament syndrome: a case report. Int J Surg Case Rep 5(8):473–475. doi: 10.1016/j.ijscr.2014.04.012 CrossRefPubMedPubMedCentralGoogle Scholar
  79. 79.
    Lipton S, Schwab P (2011) Neoplasm mimics of rheumatologic presentations: sialadenitis, ocular masquerade syndromes, retroperitoneal fibrosis, and regional pain syndromes. Rheum Dis Clin N Am 37(4):623–637. doi: 10.1016/j.rdc.2011.09.004 CrossRefGoogle Scholar
  80. 80.
    Mirault T, Lambert M, Puech P, Argatu D, Renaud A, Duhamel A, Glovacki F, Villers A, Hachulla E, Biserte J, Hatron PY, Lemaitre L (2012) Malignant retroperitoneal fibrosis: MRI characteristics in 50 patients. Medicine (Baltimore) 91(5):242–250. doi: 10.1097/MD.0b013e31826b1c1d CrossRefGoogle Scholar

Copyright information

© SIMI 2017

Authors and Affiliations

  • Giovanni Maria Rossi
    • 1
  • Rossana Rocco
    • 1
  • Eugenia Accorsi Buttini
    • 1
  • Chiara Marvisi
    • 1
  • Augusto Vaglio
    • 1
    Email author
  1. 1.Nephrology UnitParma University HospitalParmaItaly

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