PMID- 33005943
OWN - NLM
STAT- MEDLINE
DCOM- 20211125
LR  - 20220531
IS  - 1460-2385 (Electronic)
IS  - 0931-0509 (Linking)
VI  - 36
IP  - 10
DP  - 2021 Sep 27
TI  - Idiopathic retroperitoneal fibrosis: an update for nephrologists.
PG  - 1773-1781
LID - 10.1093/ndt/gfaa083 [doi]
AB  - 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 of IRF, 
      which can lead to acute renal failure and, in the long term, to varying degrees 
      of chronic kidney disease. IRF may be isolated or develop in association with 
      autoimmune diseases (e.g. Hashimoto's thyroiditis and psoriasis) and other 
      fibro-inflammatory disorders (often within the spectrum of immunoglobulin 
      G4-related disease), which suggests that it should be considered as a potentially 
      systemic condition. IRF is an immune-mediated disease: genetic variants (e.g. 
      human leukocyte antigen (HLA)-DRB1*03) and environmental agents (mainly exposure 
      to asbestos and smoking) are strongly associated with an increased risk of 
      developing the disease, while a complex network of chemokines (e.g. CXCL12 and 
      C-C moti chemokine 11 (CCL11)) and cytokines [e.g. interleukin (IL)-6, IL-12 and 
      IL-13] is likely to orchestrate the inflammatory response and simultaneously 
      promote fibrosis. Glucocorticoids, alone or in combination with traditional 
      immunosuppressants such as methotrexate and mycophenolate mofetil, are usually 
      efficacious and promptly induce disease remission; however, up to 50% of patients 
      relapse, thus requiring repeat immunosuppressive courses. Biologic drugs, namely 
      rituximab, are being explored for the treatment of IRF. In addition to medical 
      therapies, interventional procedures (mainly ureteral stenting) are required to 
      relieve ureteral obstruction, whereas surgical ureterolysis is generally reserved 
      to refractory cases. If appropriately treated, then the overall and renal 
      prognosis of IRF are good, with <5% patients developing end-stage renal disease.
CI  - (c) The Author(s) 2020. Published by Oxford University Press on behalf of ERA-EDTA. 
      All rights reserved.
FAU - Raglianti, Valentina
AU  - Raglianti V
AD  - Deptartment of Biomedical, Experimental and Clinical Sciences "Mario Serio", 
      University of Firenze, Firenze, Italy.
FAU - Rossi, Giovanni M
AU  - Rossi GM
AD  - Nephrology Unit, Parma University Hospital, Parma, Italy.
AD  - Department of Medicine and Surgery, University of Parma, Parma, Italy.
FAU - Vaglio, Augusto
AU  - Vaglio A
AD  - Deptartment of Biomedical, Experimental and Clinical Sciences "Mario Serio", 
      University of Firenze, Firenze, Italy.
AD  - Nephrology and Dialysis Unit, Meyer Children's Hospital, Firenze, Italy.
LA  - eng
PT  - Journal Article
PT  - Review
PL  - England
TA  - Nephrol Dial Transplant
JT  - Nephrology, dialysis, transplantation : official publication of the European 
      Dialysis and Transplant Association - European Renal Association
JID - 8706402
RN  - 0 (Chemokines)
RN  - 0 (Cytokines)
RN  - 0 (Glucocorticoids)
RN  - 4F4X42SYQ6 (Rituximab)
RN  - HU9DX48N0T (Mycophenolic Acid)
RN  - YL5FZ2Y5U1 (Methotrexate)
SB  - IM
MH  - Chemokines
MH  - Cytokines
MH  - Glucocorticoids
MH  - Humans
MH  - Methotrexate
MH  - Mycophenolic Acid
MH  - Nephrologists
MH  - *Retroperitoneal Fibrosis/diagnosis/etiology/therapy
MH  - Rituximab
MH  - *Ureteral Obstruction/etiology/therapy
OTO - NOTNLM
OT  - IgG4
OT  - autoimmunity
OT  - corticosteroids
OT  - hydronephrosis
OT  - idiopathic retroperitoneal fibrosis
OT  - rituximab
EDAT- 2020/10/03 06:00
MHDA- 2021/11/26 06:00
CRDT- 2020/10/02 05:47
PHST- 2019/12/02 00:00 [received]
PHST- 2020/10/03 06:00 [pubmed]
PHST- 2021/11/26 06:00 [medline]
PHST- 2020/10/02 05:47 [entrez]
AID - 5917177 [pii]
AID - 10.1093/ndt/gfaa083 [doi]
PST - ppublish
SO  - Nephrol Dial Transplant. 2021 Sep 27;36(10):1773-1781. doi: 10.1093/ndt/gfaa083.