Medication Summary
The goals of pharmacotherapy are to reduce morbidity and to prevent complications. Medications used include corticosteroids, immunosuppressives, tamoxifen, and rituximab.
Glucocorticoids
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)
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)
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.
Immunosuppressives
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)
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)
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.
Estrogen receptor antagonists
Class Summary
These agents bind to estrogen receptors, preventing stimulating effects of estrogen on nucleic acid synthesis.
Tamoxifen (Nolvadex, Tamofen-10, Tamofen-20)
Nonsteroidal antiestrogen agent. Competitively binds to estrogen receptor, producing nuclear complex that decreases DNA synthesis and inhibits estrogen effects.
DMARDs, Other
Rituximab (Riabni, Rituxan, Rituximab-abbs)
Genetically engineered chimeric murine/human monoclonal IgG1 kappa antibody directed against CD20 antigen; used off-label in retroperitoneal fibrosis
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Intravenous urogram in a patient with retroperitoneal fibrosis shows medial deviation of the middle part of both ureters.
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Retrograde ureterogram in a patient with retroperitoneal fibrosis reveals smooth narrowing and medial shift of the ureter.
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Retroperitoneal fibrosis. Retrograde pyelogram demonstrates hydronephrosis.
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Retroperitoneal fibrosis. Contrast-enhanced CT scan demonstrates a periaortic soft tissue attenuating mass.
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Retroperitoneal fibrosis. Noncontrast CT scan shows periaortic fibrotic reaction associated with an inflammatory aortic aneurysm. Note bilateral ureteric stents.
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Management algorithm of retroperitoneal fibrosis.
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Postureterolysis intravenous urogram in a patient with retroperitoneal fibrosis demonstrates lateral displacement of both ureters and a double J stent on the right side.
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Retroperitoneal fibrosis. Retrograde pyelogram shows satisfactory positioning of a wall stent in a patient with postureterolysis obstruction.
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Retroperitoneal fibrosis. Abdominal radiograph demonstrates a wall stent on the right side.
Tables
|
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 |
