scholarly journals Mycophenolate mofetil combined with steroids: New experiences in the treatment of idiopathic retroperitoneal fibrosis

2007 ◽  
Vol 64 (6) ◽  
pp. 385-390 ◽  
Author(s):  
Katarina Obrencevic ◽  
Dragan Jovanovic ◽  
Zoran Kovacevic ◽  
Rajko Hrvacevic ◽  
Ljiljana Ignjatovic ◽  
...  
Keyword(s):  
Renal Failure ◽  
Mycophenolate Mofetil ◽  
Normal Level ◽  
Retroperitoneal Fibrosis ◽  
Oral Prednisone ◽  
Immunosuppressive Drugs ◽  
Obstructive Nephropathy ◽  
Idiopathic Retroperitoneal Fibrosis ◽  
Prior Therapy ◽  
Ureteral Catheters

Background/Aim. Idiopathic retroperitoneal fibrosis (IRF) is an uncommon disease characterized by a retroperitoneal fibrotic tissue that often involve the ureters, leading to the obstructive nephropathy and variable impairment of renal function. Findings strongly suggest an autoimmune etiology. Surgery, medical treatment with immunosuppressive drugs, or a combination of both are proposed. The optimal treatment has not been established yet. The aim of this study was to present our experience with combined immunosuppressive therapy of IRF, steroids (S) and mycophenolate mofetil (MMF). Methods. We prospectively followed four patients with IRF from January 2004 to December 2006. Three patients had an active disease with bilateral hydronephrosis. In the two of them acute renal failure was presented, and ureteral catheters were inserted in one in order to manage ureteral obstruction. One patient has came to our unit with a relapse of IRF and incipient chronic renal failure after the prior therapy with ureterolysis and immunosuppressive drugs (azathioprine and tamoxifen). All patients received steroids and MMF. Two patients were treated with intravenous methylprednisolone pulses (250 mg each), for three consecutive days, followed by oral prednisone 0.5 mg/kg/day. The other two patients received oral prednisone at the same dose. Prednisone was gradually tappered to a maintenance dose of 10 mg/kg/day. Simultaneously, all patients received MMF, initially 1 g/day with the increase to 2 g/day. Results. After four weeks of the therapy all symptoms disappeared, as well as a hydronephrosis with a decrease of erythrocyte sedimentation rate and Creactive protein (CRP) to normal level in all patients. Three patients remain in remission untill the end of the follow up. One patient had a relapse because of stopping taking the therapy after six months. He was treated by oral prednisone 0.5 mg/kg/day, which was gradually decreased. After twelve weeks hydronephrosis disappeared and CRP returns to the normal level. Conclusion. The combination of steroids and mycophenolate mofetil led to the remission of IRF with a strong and quick immunosuppressive effect. It also provided avoiding the long-term use of high steroid dose and surgical procedures. .

scholarly journals Successful treatment of idiopathic retroperitoneal fibrosis with combined immunosuppressive therapy

2019 ◽  
Vol 76 (10) ◽  
pp. 1014-1021
Author(s):  
Katarina Obrencevic ◽  
Dejan Petrovic ◽  
Predrag Aleksic ◽  
Marijana Petrovic ◽  
Nemanja Rancic ◽  
...  
Keyword(s):  
Renal Failure ◽  
Renal Function ◽  
Chronic Renal Failure ◽  
Retroperitoneal Fibrosis ◽  
Oral Prednisone ◽  
Obstructive Nephropathy ◽  
Idiopathic Retroperitoneal Fibrosis ◽  
Severe Renal Failure ◽  
The Mean ◽  
Systemic Symptoms

Background/Aim. Idiopathic retroperitoneal fibrosis (IRF) is characterized by the fibroinflammatory periaortic tissue that affects the ureters, causing obstructive nephropathy and variable impairment of renal function. The findings strongly suggest an autoimmune etiology. The optimal treatment has not been established. The aim of this study was to analyze a long-term efficacy of combined corticosteroid therapy with mycophenolate mofetil (MMF) in the patients with IRF. Methods. We retrospectively followed 13 patients (8 males and 5 females) with IRF. All patients received corticosteroids and MMF. For the patients with severe renal failure, an initial ureteral decompression was made and prednisone was started orally 0.5 mg/kg with fast tapering. In cases with a mild renal failure corticosteroids were administrated as intravenous methylprednisolone pulses for 3 days, followed by oral prednisone. The dose of MMF was 1000 mg twice a day. MMF was stopped after 18 months and prednisone after 48 months. Results. Systemic symptoms resolved in all patients. Erythrocyte sedimentation (SE) rate declined from the mean of 67.6 to 26.3 mm/h and C-reactive protein (CRP) from the mean of 18.5 to 6.3 mg/L. In 7 out of 8 patients, the ureteral stents were successfully removed 13 weeks on average. Seven patients had 100% of reduction in the periaortic mass, and the average percent reduction was 76.9%. The kidney function improved and remained normal in 6 treated patients. In 4 patients a mild chronic renal failure remained due to afunction of one kidney. Three patients, with a prior chronic renal failure, did not get worse renal function. The disease recurred in 3 patients. There were no treatment side effects noted. Conclusion. Combination of corticosteroids and MMF is a potentially effective treatment in restoring the renal function and reducing the fibrotic tissue in the patients with idiopathic retroperitoneal fibrosis. It could prevent the need for ureteral stenting and surgery. Longer treatment may reduce a possibility of recurrence.

scholarly journals P0258EVALUATION OF ACUTE - PHASE REACTANTS IN MONITORING DISEASE ACTIVITY AND RESPONSE TO IMMUNOSUPPRESSIVE THERAPY IN IDIOPATHIC RETROPERITONEAL FIBROSIS

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Katarina Obrencevic ◽  
Marijana Petroviä‡ ◽  
Nemanja Rancic ◽  
Jelena Tadiä‡ ◽  
Neven Vavic ◽  
...  
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Case Series ◽  
Immunosuppressive Drugs ◽  
Obstructive Nephropathy ◽  
Mass Reduction ◽  
Retrospective Case ◽  
Idiopathic Retroperitoneal Fibrosis ◽  
Acute Phase Reactants ◽  
Case Series Study ◽  
One Year

Abstract Background and Aims Idiopathic retroperitoneal fibrosis (IRF) is a rare disease characterized by fibroinflammatory periaortic tissue that affects the ureters, causing obstructive nephropathy and impairment of renal function. Findings strongly suggest an autoimmune etiology. Medical treatment includes immunosuppressive drugs, but the optimal treatment has not been established. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) represent markers of active inflammation in the early phase of the disease, and hence could be sensitive predictors of the treatment response. Method This retrospective case series study includes patients with IRF treated with two different protocols: corticosteroids in combination with mycophenolate mofetil (MMF) and corticosteroids combined with azathioprine (AZA). The total number of 28 patients (13 in the first group and 15 in the second group) were treated and followed in the Clinic of nephrology in Military Medical Academy. Results At presentation 92.3% of patients had elevated levels of ESR and CRP, and 67.8% of them had acute renal insufficiency. Systemic symptoms resolved after four weeks in all patients. The kidney function improved significantly in both groups after first moth of treatment, although the creatinine level was higher in the MMF group because of the disease recurrence in 23% of the patients. In MMF group was observed faster reduction in the periaortic mass after six months and 53.8% of patients achieve 100% of the mass reduction at the end of the treatment while 33.3% in AZA group. The levels of ESR and CRP after one month of treatment in AZA group correlated with the fibrotic mass reduction after six months and after one year. In MMF group, only the CRP level after one month correlated with the fibrotic mass reduction achieved after one year. Conclusion MMF had more rapid and complete effect in hydrnephrosis withdrawal and total reduction of the fibrotic tissue, comparing with AZA whose effect was slower and without complete mass reduction. CRP strongly correlated with the remission of the disease in both therapeutic protocols.

scholarly journals Ureteral Strangulation by Fibrosis: A Cold Case Report of Ormand's Disease

2011 ◽  
Vol 2011 ◽  
pp. 1-4
Author(s):  
Amarpreet Sandhu ◽  
Leslea Brickner ◽  
Mark Chen
Keyword(s):  
Renal Failure ◽  
Acute Renal Failure ◽  
Retroperitoneal Fibrosis ◽  
Obstructive Uropathy ◽  
Laboratory Data ◽  
Signs And Symptoms ◽  
Final Diagnosis ◽  
Renal Ultrasound ◽  
Idiopathic Retroperitoneal Fibrosis ◽  
Acute And Chronic Inflammation

Retroperitoneal fibrosis or Ormand's disease is rare in incidence and clinically elusive to diagnosis until obstructive uropathy clinically manifests by the mechanism of ureteral fibrotic strangulation and acute renal failure. We encountered a 50-year-old woman with months of nonspecific abdominal pain and presented with signs and symptoms of acute renal failure. Laboratory data was significant for blood urea nitrogen 47 mg/dL and creatinine of 8.47 mg/dL. Renal ultrasound revealed bilateral hydronephrosis and an abdominal computed tomogram confirmed an abnormal soft tissue retroperitoneal confluence that encased the pelvic vessels. Urologic consultation was requested and bilateral ureteral stents were placed with relief of her obstructive uropathy. Five days after ureteral stenting her creatinine dropped to 1.64 mg/dL. One month later patient underwent ureterolysis with biopsy showing fibroblast proliferation consistent with acute and chronic inflammation. By ruling out infections and malignancy, the final diagnosis was made to be idiopathic retroperitoneal fibrosis.

scholarly journals Severe Complications from an Unexpectedly High Serum Mycophenolic Acid Concentration in a Patient with Renal Failure Secondary to Lupus Nephritis: A Case Report

2019 ◽  
Vol 9 (2) ◽  
pp. 72-78 ◽  
Author(s):  
Yuji Doi ◽  
Hirotsugu Kitayama ◽  
Masayoshi Yamada ◽  
Yudai Miyama
Keyword(s):  
Acute Kidney Injury ◽  
Renal Failure ◽  
Mycophenolate Mofetil ◽  
Lupus Nephritis ◽  
Acid Concentration ◽  
Immunosuppressive Agents ◽  
Kidney Injury ◽  
Immunosuppressive Drugs ◽  
High Serum ◽  
Symptom Relapse

Mycophenolate mofetil (MMF) is used widely to treat lupus nephritis and is considered safer than other immunosuppressive drugs. Reports on severe complications related to MMF are sparse. We report a case of a patient with lupus nephritis in whom severe complications were possibly caused by MMF. The patient was a 17-year-old girl who received a diagnosis of lupus nephritis at the age of 14 years and had been taking steroid and immunosuppressive agents since then. One week after starting MMF 1 g/day instead of mizoribine owing to symptom relapse and serologic data deterioration, she presented with seizure, accompanied by leukopenia, thrombocytopenia, and renal failure. We discontinued MMF because she had extremely high serum mycophenolate acid concentration (88 µg/mL). A few weeks later, she recovered without any complications and was discharged. Although rare, clinicians should be aware that serum mycophenolate acid concentration may become extremely high in the setting of acute kidney injury. In such circumstances, they should perform serum concentration monitoring to avoid possible adverse events.

scholarly journals Characteristics of Adult Patients with Idiopathic Retroperitoneal Fibrosis and Assessment of Risk of Relapse at Diagnosis

2021 ◽  
Vol 10 (7) ◽  
pp. 1380
Author(s):  
Jerome Razanamahery ◽  
Bastien Bouldoires ◽  
Sebastien Humbert ◽  
Philip Bielefeld ◽  
Veronique Fournier ◽  
...  
Keyword(s):  
Renal Failure ◽  
Smoking Cessation ◽  
Chronic Renal Failure ◽  
Retroperitoneal Fibrosis ◽  
Adult Patients ◽  
Idiopathic Retroperitoneal Fibrosis ◽  
Multicentric Study ◽  
Radiological Features ◽  
Presentation Methods

Objectives: To compare adult patients’ characteristics suffering from idiopathic retroperitoneal fibrosis between “relapse-free” and relapsing patients at the diagnosis and identify factors associated with relapse at initial presentation. Methods: We conducted a retrospective multicentric study in four hospitals in Eastern France, from 1993 to 2020, of adult patients suffering from idiopathic retroperitoneal fibrosis. We analyzed clinical, biological, and radiological features at diagnosis and during a forty-month follow-up. Results: Of 47 patients suffering from retroperitoneal fibrosis, 21 patients had idiopathic retroperitoneal fibrosis. Among them, 13 experienced one or more relapses during follow-up. At diagnosis, clinical characteristics, relevant comorbidities, biological and radiological features were similar between groups. Smoking cessation seems associated with decreased relapse risk (p: 0.0624). A total of 8 patients developed chronic renal failure during follow-up. Ureteral infiltration at diagnosis was associated with evolution to chronic renal failure (p: 0.0091). Conclusion: No clinical, biological, or radiological features could predict relapse at retroperitoneal fibrosis diagnosis, but smoking cessation may prevent relapse.

scholarly journals A Rare Cause of Acute Renal Failure: Retroperitoneal Fibrosis

2019 ◽  
Vol 1 (4) ◽  
pp. 1-4
Author(s):  
Yılmaz Omer ◽  
Kizilkan Yunus Emre ◽  
Temel Muhammed Cihan ◽  
Ediz Caner ◽  
Ozcelik Fatih
Keyword(s):  
Renal Failure ◽  
Acute Renal Failure ◽  
Retroperitoneal Fibrosis ◽  
Foley Catheter ◽  
Idiopathic Retroperitoneal Fibrosis ◽  
Fibrotic Tissue ◽  
Abdominal Magnetic Resonance Imaging ◽  
Grade 3 ◽  
Loss Of Appetite

Idiopathic retroperitoneal fibrosis also known as Ormonds disease is a rare disorder characterized by the development of fibrotic tissue in the retroperitoneum. The fibrotic tissue may compress ureters, leading to obstructive nephrouropathy and renal failure. A 58-year-old man with fatigue, loss of appetite and unable to urinate was admitted to our clinic. Because of the serum creatinine value of 5.3 mg/dl, urinary ultrasonography was performed and bilateral grade 3 hydronephrosis with moderate level urine in bladder was detected. Hydronephrosis did not regress by transurethral foley catheter and suspicious appearance in the retroperitoneal area was found in abdominal magnetic resonance imaging. Tru-cut biopsy result of the current lesion was finally reported as a connective tissue. Bilateral double j catheter insertion was performed and started to immunosuppression therapy with corticosteroid. Two months later, double j catheters were removed and hydronephrosis was not detected in follow-up. In this case report, we tried to explain that, retroperitoneal fibrosis should be considered in the differential diagnosis of postrenal acute renal failure, even in patients without a classic symptom such as pain. In addition, early surgical intervention should be avoided in such patients.

Idiopathic Retroperitoneal Fibrosis Presenting with Hypertension and Acute Renal Failure

2017 ◽  
Vol 18 (1) ◽  
pp. 37-39
Author(s):  
Engin Onan ◽  
Saime Paydas ◽  
Merve Erkoc ◽  
Tuba Korkmaz ◽  
Hasan Bilen Onan ◽  
...  
Keyword(s):  
Renal Failure ◽  
Acute Renal Failure ◽  
Retroperitoneal Fibrosis ◽  
Idiopathic Retroperitoneal Fibrosis

scholarly journals Concomitant presentation of IgG4-negative idiopathic retroperitoneal fibrosis and Addison’s disease

2019 ◽  
Vol 12 (10) ◽  
pp. e230767 ◽  
Author(s):  
Mark Riley ◽  
Muhammad Hamza Saad Shaukat ◽  
Mohammed Bari ◽  
Ruben Peredo-Wende
Keyword(s):  
Renal Failure ◽  
Adrenal Insufficiency ◽  
Plasma Cells ◽  
Retroperitoneal Fibrosis ◽  
Obstructive Uropathy ◽  
Addison’S Disease ◽  
Serum Cortisol ◽  
Serum Cortisol Level ◽  
Addison's Disease ◽  
Idiopathic Retroperitoneal Fibrosis

We describe a patient who was admitted to our medical centre with acute renal failure, hyponatraemia and hyperkalaemia. CT of the abdomen and pelvis showed a retroperitoneal mass with bilateral ureteral obstruction. Biopsy revealed fibrosis with inflammatory infiltrate, but rare IgG4-positive plasma cells. After placement of bilateral pigtail nephrostomy catheters, renal failure improved but metabolic derangements remained. Morning serum cortisol level was equivocal, but with blunted response on cosyntropin stimulation testing indicating adrenal insufficiency. Serology for 21-hydroxylase antibodies was strongly positive, supporting the diagnosis of Addison’s disease. In addition to nephrostomy catheters for obstructive uropathy, idiopathic retroperitoneal fibrosis was treated with mycophenolate mofetil. Physiological doses of hydrocortisone and fludrocortisone for Addison’s disease were also initiated. The patient continues to be monitored for regression of the mass. Based on review of the literature, this is the first reported case of IgG4-negative idiopathic retroperitoneal fibrosis presenting with autoimmune primary adrenal insufficiency.

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