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Retroperitoneal Fibrosis
Alice Fung, MD; Matthew T. Heller, MD, FSAR
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KEY FACTS

  • Imaging

    • Top Differential Diagnoses

      • Pathology

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Retroperitoneal fibrosis (RPF)
            • Synonyms

              • Ormond disease, periaortitis
            • Definitions

              • Spectrum of diseases resulting in proliferation of fibroinflammatory tissue in infrarenal portion of retroperitoneum
                • IgG4-related disease (IgG4-RD)
                • Systemic and organ-specific autoimmune diseases
                • Chronic periaortitis: Inflammatory abdominal aortic aneurysms
                  • Presumed etiology: Hypersensitivity to antigens in atheromatous plaques
                  • Severe form: Perianeurysmal fibrosis
                  • Not always included in RPF since treatment and prognosis are related to underlying vascular disease

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • Nuclear Medicine Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Kurowecki D et al: Cross-sectional pictorial review of IgG4-related disease. Br J Radiol. 92(1103):20190448, 2019
                                              2. Morin G et al: Persistent FDG/PET CT uptake in idiopathic retroperitoneal fibrosis helps identifying patients at a higher risk for relapse. Eur J Intern Med. 62:67-71, 2019
                                              3. Cohan RH et al: Imaging appearance of fibrosing diseases of the retroperitoneum: can a definitive diagnosis be made? Abdom Radiol (NY). 43(5):1204-14, 2018
                                              4. Vaglio A et al: Idiopathic retroperitoneal fibrosis. J Am Soc Nephrol. 27(7):1880-9, 2016
                                              5. He Y et al: Spectrum of IgG4-related disease on multi-detector CT: a 5-year study of a single medical center data. Abdom Imaging. 40(8):3104-16, 2015
                                              6. Liang W et al: Imaging-based evaluation of retroperitoneal fibrosis: a challenge for radiologists. Radiology. 274(3):937-8, 2015
                                              7. Bakir B et al: Role of diffusion-weighted MR imaging in the differentiation of benign retroperitoneal fibrosis from malignant neoplasm: preliminary study. Radiology. 272(2):438-45, 2014
                                              8. Caiafa RO et al: Retroperitoneal fibrosis: role of imaging in diagnosis and follow-up. Radiographics. 33(2):535-52, 2013
                                              9. George V et al: Chronic fibrosing conditions in abdominal imaging. Radiographics. 33(4):1053-80, 2013
                                              10. Goenka AH et al: Imaging of the retroperitoneum. Radiol Clin North Am. 50(2):333-55, vii, 2012
                                              11. Rosenkrantz AB et al: Utility of MRI features for differentiation of retroperitoneal fibrosis and lymphoma. AJR Am J Roentgenol. 199(1):118-26, 2012
                                              12. Li KP et al: Idiopathic retroperitoneal fibrosis (RPF): clinical features of 61 cases and literature review. Clin Rheumatol. 30(5):601-5, 2011
                                              13. Cronin CG et al: Retroperitoneal fibrosis: a review of clinical features and imaging findings. AJR Am J Roentgenol. 191(2):423-31, 2008
                                              14. Vaglio A et al: Retroperitoneal fibrosis. Lancet. 367(9506):241-51, 2006
                                              15. Hamano H et al: Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis. Lancet. 359(9315):1403-4, 2002
                                              16. Vivas I et al: Retroperitoneal fibrosis: typical and atypical manifestations. Br J Radiol. 73(866):214-22, 2000
                                              Related Anatomy
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                                              Related Differential Diagnoses
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                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Imaging

                                                • Top Differential Diagnoses

                                                  • Pathology

                                                    • Clinical Issues

                                                      • Diagnostic Checklist

                                                        TERMINOLOGY

                                                        • Abbreviations

                                                          • Retroperitoneal fibrosis (RPF)
                                                        • Synonyms

                                                          • Ormond disease, periaortitis
                                                        • Definitions

                                                          • Spectrum of diseases resulting in proliferation of fibroinflammatory tissue in infrarenal portion of retroperitoneum
                                                            • IgG4-related disease (IgG4-RD)
                                                            • Systemic and organ-specific autoimmune diseases
                                                            • Chronic periaortitis: Inflammatory abdominal aortic aneurysms
                                                              • Presumed etiology: Hypersensitivity to antigens in atheromatous plaques
                                                              • Severe form: Perianeurysmal fibrosis
                                                              • Not always included in RPF since treatment and prognosis are related to underlying vascular disease

                                                        IMAGING

                                                        • General Features

                                                          • Radiographic Findings

                                                            • CT Findings

                                                              • MR Findings

                                                                • Ultrasonographic Findings

                                                                  • Nuclear Medicine Findings

                                                                    • Imaging Recommendations

                                                                      DIFFERENTIAL DIAGNOSIS

                                                                        PATHOLOGY

                                                                        • General Features

                                                                          • Gross Pathologic & Surgical Features

                                                                            • Microscopic Features

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      DIAGNOSTIC CHECKLIST

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Kurowecki D et al: Cross-sectional pictorial review of IgG4-related disease. Br J Radiol. 92(1103):20190448, 2019
                                                                                          2. Morin G et al: Persistent FDG/PET CT uptake in idiopathic retroperitoneal fibrosis helps identifying patients at a higher risk for relapse. Eur J Intern Med. 62:67-71, 2019
                                                                                          3. Cohan RH et al: Imaging appearance of fibrosing diseases of the retroperitoneum: can a definitive diagnosis be made? Abdom Radiol (NY). 43(5):1204-14, 2018
                                                                                          4. Vaglio A et al: Idiopathic retroperitoneal fibrosis. J Am Soc Nephrol. 27(7):1880-9, 2016
                                                                                          5. He Y et al: Spectrum of IgG4-related disease on multi-detector CT: a 5-year study of a single medical center data. Abdom Imaging. 40(8):3104-16, 2015
                                                                                          6. Liang W et al: Imaging-based evaluation of retroperitoneal fibrosis: a challenge for radiologists. Radiology. 274(3):937-8, 2015
                                                                                          7. Bakir B et al: Role of diffusion-weighted MR imaging in the differentiation of benign retroperitoneal fibrosis from malignant neoplasm: preliminary study. Radiology. 272(2):438-45, 2014
                                                                                          8. Caiafa RO et al: Retroperitoneal fibrosis: role of imaging in diagnosis and follow-up. Radiographics. 33(2):535-52, 2013
                                                                                          9. George V et al: Chronic fibrosing conditions in abdominal imaging. Radiographics. 33(4):1053-80, 2013
                                                                                          10. Goenka AH et al: Imaging of the retroperitoneum. Radiol Clin North Am. 50(2):333-55, vii, 2012
                                                                                          11. Rosenkrantz AB et al: Utility of MRI features for differentiation of retroperitoneal fibrosis and lymphoma. AJR Am J Roentgenol. 199(1):118-26, 2012
                                                                                          12. Li KP et al: Idiopathic retroperitoneal fibrosis (RPF): clinical features of 61 cases and literature review. Clin Rheumatol. 30(5):601-5, 2011
                                                                                          13. Cronin CG et al: Retroperitoneal fibrosis: a review of clinical features and imaging findings. AJR Am J Roentgenol. 191(2):423-31, 2008
                                                                                          14. Vaglio A et al: Retroperitoneal fibrosis. Lancet. 367(9506):241-51, 2006
                                                                                          15. Hamano H et al: Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis. Lancet. 359(9315):1403-4, 2002
                                                                                          16. Vivas I et al: Retroperitoneal fibrosis: typical and atypical manifestations. Br J Radiol. 73(866):214-22, 2000