Current approach to diagnosis and management of retroperitoneal fibrosis.

Published on Mar 12, 2020in International Journal of Urology2.445
· DOI :10.1111/IJU.14218
Toshiaki Tanaka16
Estimated H-index: 16
(Sapporo Medical University),
Naoya Masumori37
Estimated H-index: 37
(Sapporo Medical University)
Retroperitoneal fibrosis is characterized by fibrotic lesions around the abdominal aorta and common ileac artery causing ureteral obstruction. Secondary retroperitoneal fibrosis is associated with malignant disease, drugs, exposure to radiation and surgery. In contrast, the majority of retroperitoneal fibrosis is classified into idiopathic retroperitoneal fibrosis, for which immunological etiology has been suggested. Recently, idiopathic retroperitoneal fibrosis has been considered to be a spectrum of immunoglobulin G4-related disease, a systemic inflammatory disease, the concept of which has been developed during the past decade. In the management of retroperitoneal fibrosis, assessment of systemic lesions associated with immunoglobulin G4-related disease and the exclusion of secondary retroperitoneal fibrosis is mandatory. Histological examination of retroperitoneal lesions is desired for accurate diagnosis and management. Laparoscopic or open biopsy is often beneficial, although it is more invasive than needle biopsy. Treatment for idiopathic retroperitoneal fibrosis consists of meticulous glucocorticoid therapy based on that for immunoglobulin G4-related disease, which is expected to be highly effective. Ureteral obstruction is usually managed with conservative procedures, such as ureteral stenting or percutaneous nephrostomy. The goal of treatment for retroperitoneal fibrosis should be freedom from the stent/nephrostomy with withdrawal of the glucocorticoid in addition to salvage of renal function; however, conservative management does not always provide favorable outcomes. In contrast, aggressive surgical treatment, such as ureterolysis, can achieve the goal; however, the procedure is associated with high morbidity. Establishment of a consensus about treatment for idiopathic retroperitoneal fibrosis, including the optimal indications for the invasive surgical procedure and conservative management, is desired.
#1Jungen Tang (HUST: Huazhong University of Science and Technology)H-Index: 3
#2Shaozhe Cai (HUST: Huazhong University of Science and Technology)H-Index: 3
Last. Lingli Dong (HUST: Huazhong University of Science and Technology)H-Index: 16
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7 CitationsSource
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#2Federica Maritati (UniFI: University of Florence)H-Index: 13
Last. Augusto Vaglio (UniFI: University of Florence)H-Index: 42
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#1Tong Chen (SJTU: Shanghai Jiao Tong University)H-Index: 1
#1T. Chen (SJTU: Shanghai Jiao Tong University)H-Index: 1
Last. S. Ding (SDU: Shandong University)H-Index: 1
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#2Zachary S. WallaceH-Index: 25
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AbstractIgG4-related disease (IgG4-RD) is a fascinating clinical entity proposed by Japanese investigators, and includes a wide variety of diseases, formerly diagnosed as Mikulicz's disease (MD), autoimmune pancreatitis (AIP), interstitial nephritis, prostatitis, retroperitoneal fibrosis, etc. Although all clinicians in every field of medicine may encounter this new disease, a unifying diagnostic criterion has not been established. In 2011, the Japanese IgG4 team, organized by the Ministry of He...
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