Sections

Medication

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and to prevent complications. Empirical therapy includes corticosteroids, immunosuppressives, and tamoxifen.

Class Summary

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli. Doses of up to 10 mg/d of prednisone or prednisolone are typically used, but some patients may require higher doses. Adverse events associated with long-term steroid use make dose reductions and cessation important in due course.

Prednisolone (Pediapred, Delta-Cortef, Articulose-50, AK-Pred)

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Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and suppresses lymphocytes and antibody production.

Prednisone (Deltasone, Meticorten, Orasone)

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Class Summary

Some agents function as antimetabolites to decrease DNA and RNA synthesis and are used to treat a number of autoimmune conditions. These agents can also be used in patients with inadequate response or excessive toxicity to corticosteroids.

Mycophenolate (CellCept, Myfortic)

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Inhibits purine synthesis and proliferation of human lymphocytes. Promising published case report of 3 patients with resistant disease treated with mycophenolate mofetil. Reduced toxicity makes this regimen an attractive alternative.

Azathioprine (Imuran)

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Imidazolyl derivative of 6-mercaptopurine. Many of biological effects are similar to those of parent compound. Both compounds are eliminated rapidly from blood and are oxidized or methylated in erythrocytes and liver. No azathioprine or mercaptopurine is detectable in urine 8 h after administration.

Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. Mechanism whereby azathioprine affects autoimmune diseases unknown. Works primarily on T cells. Suppresses hypersensitivities of cell-mediated type and causes variable alterations in antibody production.

Immunosuppressive, delayed hypersensitivity, and cellular cytotoxicity tests are suppressed to a greater degree than antibody responses. Works very slowly; may require 6-12 mo of trial prior to effect. Up to 10% of patients may have an idiosyncratic reaction that disallows use. Do not allow WBC count to drop to less than 3000/mL or lymphocyte count to drop to less than 1000/mL.

Available in tab form for oral administration or in 100-mg vials for IV injection.

Class Summary

These agents bind to estrogen receptors, preventing stimulating effects of estrogen on nucleic acid synthesis.

Tamoxifen (Nolvadex, Tamofen-10, Tamofen-20)

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Nonsteroidal antiestrogen agent. Competitively binds to estrogen receptor, producing nuclear complex that decreases DNA synthesis and inhibits estrogen effects.

Follow-up

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Media Gallery

Intravenous urogram shows medial deviation of the middle part of both ureters.
Retrograde ureterogram reveals smooth narrowing and medial shift of the ureter.
Retrograde pyelogram demonstrates hydronephrosis.
Contrast-enhanced CT scan demonstrates a periaortic soft tissue attenuating mass.
Noncontrast CT scan shows periaortic fibrotic reaction associated with an inflammatory aortic aneurysm. Note bilateral ureteric stents.
Management algorithm of retroperitoneal fibrosis.
Postureterolysis intravenous urogram demonstrates lateral displacement of both ureters and a double J stent on the right side.
Retrograde pyelogram shows satisfactory positioning of a wall stent in a patient with postureterolysis obstruction.
Abdominal radiograph demonstrates a wall stent on the right side.

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Table. Differential Diagnoses of Retroperitoneal Fibrosis^[43]

Table. Differential Diagnoses of Retroperitoneal Fibrosis ^[43]

	Retroperitoneal Fibrosis	Retroperitoneal Lymphoma	Sclerosing Mesenteritis	Desmoid-Type Fibromatosis	Inflammatory Myofibroblastic Tumor	Well-Differentiated Liposarcoma Sclerosing Variant
Ureteral displacement	Medial	Lateral
Ureteral obstruction	~80%	~50%	Rare	Rare	Rare	Unknown
Aortic displacement	Rare	Anterior
Reactive perivascular lymphoid aggregates	100%	Absent	Variable	Rare	Variable	Present in the inflammatory type
Necrosis	Absent	Variable	Fat necrosis	Rare	Focal	Fat necrosis
Vasculitis	~50%	Absent	Absent	Absent	Absent	Absent
Clonality	Absent	Variable	Absent	Absent	Absent	Present
Β-catenin	Negative	Unknown	Negative	Positive in 90% of cases	Negative	Variable positivity
ALK-1	Negative	Usually negative	Negative	Negative	Positive in 50% of cases	Negative
CD-117	Negative in spindle cell component	Rare	Variable	Negative	Rare	Negative
Desmin	Negative	Negative	Variable	Rare	Usually positive	Rare
S100	Negative	Negative	Negative	Rare	Negative	Usually positive in the adipocytic component

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Author

Chandra Shekhar Biyani, MS, MBBS, DUrol, FRCS(Urol), FEBU Consulting Urologist, Department of Urology, Pinderfields General Hospital, The Mid-Yorkshire Hospitals NHS Trust, UK

Chandra Shekhar Biyani, MS, MBBS, DUrol, FRCS(Urol), FEBU is a member of the following medical societies: British Medical Association, International College of Surgeons, British Association of Urological Surgeons, European Association of Urology

Disclosure: Nothing to disclose.

Coauthor(s)

Joby Taylor, MBChB, FRCS Consultant Urologist, Forth Valley Royal Hospital, UK

Joby Taylor, MBChB, FRCS is a member of the following medical societies: British Association of Urological Surgeons, European Association of Urology, International Continence Society, Royal College of Surgeons of Edinburgh, United Kingdom Continence Society

Disclosure: Nothing to disclose.

Anthony J Browning, MB, ChB, FRCS(Edin) Consultant Urological Surgeon, Mid Yorkshire NHS Trust, Pinderfields General Hospital; Associate Post Graduate Dean, Yorkshire and Humber Deanery, UK

Anthony J Browning, MB, ChB, FRCS(Edin) is a member of the following medical societies: Endourological Society, British Association of Urological Surgeons, European Association of Urology

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Bradley Fields Schwartz, DO, FACS Professor of Urology, Director, Center for Laparoscopy and Endourology, Department of Surgery, Southern Illinois University School of Medicine

Bradley Fields Schwartz, DO, FACS is a member of the following medical societies: American College of Surgeons, American Urological Association, Association of Military Osteopathic Physicians and Surgeons, Endourological Society, Society of Laparoendoscopic Surgeons, Society of University Urologists

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Endourological Society Board of Directors; President Elect North Central Section of the American Urological Association<br/>Serve(d) as a speaker or a member of a speakers bureau for: Cook Medical.

Additional Contributors

Martha K Terris, MD, FACS Professor and Chief of Urology, Witherington Distinguished Chair, Department of Surgery, Section of Urology, Director, Urology Residency Training Program, Medical College of Georgia at Augusta University; Professor, Department of Physician Assistants, Medical College of Georgia School of Allied Health; Chief, Section of Urology, Augusta Veterans Affairs Medical Center

Martha K Terris, MD, FACS is a member of the following medical societies: American Cancer Society, American College of Surgeons, American Institute of Ultrasound in Medicine, American Society of Clinical Oncology, American Urological Association, Association of Women Surgeons, New York Academy of Sciences, Society of Government Service Urologists, Society of University Urologists, Society of Urology Chairpersons and Program Directors, Society of Women in Urology

Disclosure: Nothing to disclose.

Retroperitoneal Fibrosis Medication

Medication Summary

Glucocorticoids

Class Summary

Prednisolone (Pediapred, Delta-Cortef, Articulose-50, AK-Pred)

Prednisolone (Pediapred, Delta-Cortef, Articulose-50, AK-Pred)

Prednisone (Deltasone, Meticorten, Orasone)

Prednisone (Deltasone, Meticorten, Orasone)

Immunosuppressives

Class Summary

Mycophenolate (CellCept, Myfortic)

Mycophenolate (CellCept, Myfortic)

Azathioprine (Imuran)

Azathioprine (Imuran)

Estrogen receptor antagonists

Class Summary

Tamoxifen (Nolvadex, Tamofen-10, Tamofen-20)

Tamoxifen (Nolvadex, Tamofen-10, Tamofen-20)

Retroperitoneal Fibrosis Medication

Medication Summary

Glucocorticoids

Class Summary

Prednisolone (Pediapred, Delta-Cortef, Articulose-50, AK-Pred)

Prednisolone (Pediapred, Delta-Cortef, Articulose-50, AK-Pred)

Prednisone (Deltasone, Meticorten, Orasone)

Prednisone (Deltasone, Meticorten, Orasone)

Immunosuppressives

Class Summary

Mycophenolate (CellCept, Myfortic)

Mycophenolate (CellCept, Myfortic)

Azathioprine (Imuran)

Azathioprine (Imuran)

Estrogen receptor antagonists

Class Summary

Tamoxifen (Nolvadex, Tamofen-10, Tamofen-20)

Tamoxifen (Nolvadex, Tamofen-10, Tamofen-20)

References

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