Follow-up checks during and after therapy
During therapy and after discontinuation of immunosuppression, regular laboratory checks and abdominal ultrasound examinations were carried out.
The laboratory tests included:
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Blood count
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Electrolytes
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Blood-lowering rate (BSG)
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C-reactive protein (CRP)
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Serum creatinine
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Serumurea
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Blood glucose
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Lipase
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OLD and AST
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Urine Status
In the first six months after the start of therapy, the examinations were initially carried out at intervals of two weeks. After a stable state had been reached, the control intervals could be extended to three months.
Radiological follow-up checks by means of computed tomography (CT) or magnetic resonance imaging (MRI) were carried out approximately every four to six months during therapy. After the end of immunosuppression, the examination intervals were extended to twelve months.
Removal of the ureter rails
The time of removal of the ureter splints was dependent on the extent of the radiologically visible regression of the retroperitoneal fibrosis. Once again, hydronephrosis, i.e. a urinary congestion of the kidney, was used again by stents or ureter rails.
As a successful response to drug therapy, both an improvement in laboratory values and a regression of fibrosis detectable in CT or MRI were evaluated. In addition, the subjective improvement of the complaints was taken into account.
A recurrence was defined as a renewed increase in fibrosis after discontinuation of the MMF medication.
Results of treatment
In all patients, the CT or MRI showed a regression of the retroperitoneal tissue mass. In five patients, the primary aperation could be finally removed.
The average duration of the stents was 5.6 months and was between three and eight months.
Shortly after the start of treatment, the patients reported a significant improvement in their symptoms. Significant changes in laboratory values were also observed in the course of the therapy.
Creatinine decreased on average from 2.8 to 1.3 mg/dl. The CRP value decreased on average from 5.5 mg/dL to 0.8 mg/dL. The blood-lowering speed also decreased on average from 36 to 9 mm in the first hour.
Treatment with mycophenolate mofetil (MMF) was stopped at an average of 27 months. The duration of treatment was between 11 and 96 months.
Treatment with corticosteroids, on the other hand, was completed on average after seven months. The duration of treatment was between three and 24 months.
Therapy regimen for idiopathic retroperitoneal fibrosis
After the diagnosis of idiopathic retroperitoneal fibrosis, a distinction was first made as to whether a ureteral obstruction, i.e. a narrowing or blocking of the ureter, was present.
In ureteral obstruction
In the event of obstruction of the urinary outflow, a ureter stent was first inserted.
In addition, drug therapy with corticosteroids and mycophenolate mofetil was started.
The treatment provided:
• Corticosteroids with about 1 mg/kg body weight
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MMF with 2-3 g daily
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regular checks
• step by step reduction of cortisondosis
After about six months, cortisone therapy should be terminated and the course should be controlled by MRI.
In the absence of hydronephrosis, declining creatinine and a regression of fibrosis, the ureter splint could be removed.
Depending on the course, the MMF therapy could also be terminated.
If, on the other hand, hydronephrosis remained, further stent changes or surgical treatment were possible.
In case of lack of ureteral obstruction
Even without ureter obstruction, immunosuppressive therapy with corticosteroids and MMF was carried out.
The first few months were marked by close checks. Subsequently, the distances between the investigations could be extended.
The decisive factor for the further course were:
• the existence or absence of hydronephrosis,
• the development of the creatinine value,
• the regression of fibrosis in CT or MRI.
Special courses of illness
In one patient, surgical ureterlateralization was provided due to severe hydronephrosis. However, the patient permanently refused this procedure.
Under continuous MMF therapy and repeated stent inlays, their kidney function could be stabilized with a creatinine level of about 1.2 mg/dL. The residual function of the left kidney was only about ten percent.
In order to avoid surgery as much as possible, the patient decided to discontinue MMF therapy over a period of eight years.
Special course in Patient No. 9
Patient No. 9 was admitted to an emergency hospital due to severe abdominal pain and the radiological suspicion of an abdominal aortic aneurysm.
However, no aneurysm could be detected in the subsequent emergency surgery. A histological examination carried out during the procedure instead showed evidence of retroperitoneal fibrosis. The procedure was then terminated.
After wound healing was completed, a drug treatment began. Within four months, the imaging process showed a significant decrease in the mass of tissue around the aorta.
Figure 3: MRI of the abdomen before initiation of therapy
Figure 4: MRI of the abdomen four months after initiation of therapy with MMF and glucocorticoids
Special course in Patient No. 7
Patient No. 7 showed only mild hydronephrosis at the beginning. Therefore, the insert of a ureter stent could be dispensed with.
The treatment was carried out exclusively by medication. In the further course, the kidney congestion completely regressed and was subsequently permanently no longer detectable.