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Augusto Vaglio - One of the best experts on this subject based on the ideXlab platform.

  • into clinical practice diagnosis and therapy of Retroperitoneal Fibrosis
    Current Rheumatology Reports, 2021
    Co-Authors: Paride Fenaroli, Augusto Vaglio, Federica Maritati
    Abstract:

    We aim to review the most relevant diagnostic features and treatment options of Retroperitoneal Fibrosis, in order to provide a useful guide for clinical practice. The recent literature highlights the role of imaging studies such as computed tomography, magnetic resonance imaging and positron emission tomography as useful tools for the diagnosis of Retroperitoneal Fibrosis, with Retroperitoneal biopsy being reserved to atypical cases. The treatment approach is mainly conservative and is based on the use of medical therapies plus urological interventions. Medical therapies essentially comprise glucocorticoids and immunosuppressants—either traditional or biological agents such as rituximab. Surgical ureterolysis is only left for refractory cases. Recent findings in Retroperitoneal Fibrosis highlight the possibility of a non-invasive diagnostic approach and a conservative treatment strategy.

  • Idiopathic Retroperitoneal Fibrosis and its overlap with IgG4-related disease
    Internal and Emergency Medicine, 2017
    Co-Authors: Giovanni Maria Rossi, Rossana Rocco, Eugenia Accorsi Buttini, Chiara Marvisi, Augusto Vaglio
    Abstract:

    Retroperitoneal Fibrosis (RPF) is a rare disease characterised by fibrous tissue proliferation in the retroperitoneum, with encasement of the ureters and large vessels of the abdomen as the most destructive of potentially severe complications. It can either be idiopathic, or secondary to infections, malignancies, or the use of certain drugs. The idiopathic form accounts for approximately 75% of the cases, and is usually responsive to immunosuppressive therapy. In recent years, the emergence of a new clinical entity, IgG4-related disease (IgG4-RD), shed light on many fibro-inflammatory disorders once thought to be separate clinical entities, although frequently associated in the so-called multifocal fibrosclerosis. Among these, together with sclerosing pancreatitis and cholangitis, pseudotumour of the orbit, idiopathic mediastinal Fibrosis and other conditions, is idiopathic Retroperitoneal Fibrosis (IRF). Both IRF and IgG4-RD can be associated with a wide variety of disorders, usually governed by immune-mediated (and particularly auto-immune) mechanisms. In our review, we discuss the clinical and therapeutic challenges IRF presents to the internist, as well as the meaning of its recent inclusion in the IgG4-RD spectrum from a clinical practice standpoint.

  • idiopathic Retroperitoneal Fibrosis
    Journal of The American Society of Nephrology, 2016
    Co-Authors: Augusto Vaglio, Federica Maritati
    Abstract:

    Idiopathic Retroperitoneal Fibrosis (RPF), reviewed herein, is a rare fibro-inflammatory disease that develops around the abdominal aorta and the iliac arteries, and spreads into the adjacent retroperitoneum, where it frequently causes ureteral obstruction and renal failure. The clinical phenotype of RPF is complex, because it can be associated with fibro-inflammatory disorders involving other organs, is considered part of the spectrum of IgG4-related disease, and often arises in patients with other autoimmune conditions. Obstructive uropathy is the most common complication, although other types of renal involvement may occur, including stenosis of the renal arteries and veins, renal atrophy, and different types of associated GN. Environmental and genetic factors contribute to disease susceptibility, whereas the immunopathogenesis of RPF is mediated by different immune cell types that eventually promote fibroblast activation. The diagnosis is made on the basis of computed tomography or magnetic resonance imaging, and positron emission tomography is a useful tool in disease staging and follow-up. Treatment of idiopathic RPF aims at relieving ureteral obstruction and inducing disease regression, and includes the use of glucocorticoids, combined or not with other traditional immunosuppressants. However, biologic therapies such as the B cell–depleting agent rituximab are emerging as potentially efficacious agents in difficult-to-treat cases.

  • idiopathic and secondary forms of Retroperitoneal Fibrosis a diagnostic approach
    Revue de Médecine Interne, 2015
    Co-Authors: M L Urban, Alessandra Palmisano, Domenico Corradi, Carlo Buzio, Maria Nicastro, Augusto Vaglio
    Abstract:

    Retroperitoneal Fibrosis (RPF) is an uncommon disease characterized by a fibrous reaction that takes place in the peri-aortic retroperitoneum and often entraps the ureters causing obstructive uropathy. RPF is idiopathic in the majority of cases, but can also be secondary to malignancies, infections, drugs, radiotherapy, and rare histiocytic disorders such as Erdheim-Chester disease. Idiopathic RPF is an immune-mediated disease, which can either be isolated, associated with other autoimmune diseases, or arise in the context of a multifocal fibro-inflammatory disorder recently renamed as IgG4-related disease. The differential diagnosis between idiopathic, IgG4-related and secondary RPF is crucial, essentially because the therapeutic approaches - especially of idiopathic vs. secondary RPF - can be dramatically different. This review focuses on the clinical, laboratory and imaging features of the different RPF forms, and also provides an overview of the available treatment options.

  • prednisone versus tamoxifen in patients with idiopathic Retroperitoneal Fibrosis an open label randomised controlled trial
    The Lancet, 2011
    Co-Authors: Augusto Vaglio, Carlo Salvarani, Alessandra Palmisano, Domenico Corradi, Federico Alberici, Umberto Maggiore, S Ferretti, Rocco Cobelli, F Ferrozzi, Carlo Buzio
    Abstract:

    Summary Background Glucocorticoids are the mainstay of treatment of idiopathic Retroperitoneal Fibrosis, but they often have substantial toxic effects. Several reports have suggested tamoxifen as an alternative to glucocorticoids. We compared the efficacy of prednisone with that of tamoxifen in maintainance of remission in patients with idiopathic Retroperitoneal Fibrosis. Methods In this open-label, randomised controlled trial, we enrolled patients aged 18–85 years with newly diagnosed idiopathic Retroperitoneal Fibrosis at the Parma Hospital, Parma, Italy, between Oct 1, 2000, and June 30, 2006. After induction therapy with 1 mg/kg daily of prednisone for 1 month, the patients who achieved remission were randomly assigned to receive tapering prednisone (initial dose 0·5 mg/kg daily) for 8 months or tamoxifen (fixed dose 0·5 mg/kg daily) for 8 months. The sequence of randomisation (1:1), blocked in groups of two and four (with block size randomly selected), was generated by the trial statistician with a computer programme. After the end of treatment, the patients were followed up for an additional 18 months. Neither patients nor those giving interventions or analysing the data were masked to group assignment. The two radiologists who assessed CT and MRI scans were masked. The primary endpoint was the relapse rate by the end of treatment (month 8), which was analysed by intention to treat. This trial is registered with ClinicalTrials.gov, number NCT00440349. Findings After induction therapy, 36 of the 40 enrolled patients achieved remission and were randomly assigned to treatment (18 per group). One patient (6%) in the prednisone group and seven patients (39%) in the tamoxifen group relapsed by the end of treatment (difference −33% [95% CI −58 to −8, p=0·0408]. The difference in relapse rate between the groups was sustained after the additional 18-month follow-up: the 26-month estimated cumulative relapse probability was 17% with prednisone and 50% with tamoxifen (difference −33% [−62 to −3, p=0·0372]). Cushingoid changes and grade 2 hypercholesterolaemia were more common in the prednisone group than in the tamoxifen group (p=0·0116 and p=0·0408, respectively). Interpretation Prednisone is more effective in prevention of relapses than is tamoxifen in patients with idiopathic Retroperitoneal Fibrosis. Therefore, prednisone should be considered as first-line treatment for patients with newly diagnosed idiopathic Retroperitoneal Fibrosis. Funding Parma University Hospital.

Carlo Buzio - One of the best experts on this subject based on the ideXlab platform.

  • idiopathic and secondary forms of Retroperitoneal Fibrosis a diagnostic approach
    Revue de Médecine Interne, 2015
    Co-Authors: M L Urban, Alessandra Palmisano, Domenico Corradi, Carlo Buzio, Maria Nicastro, Augusto Vaglio
    Abstract:

    Retroperitoneal Fibrosis (RPF) is an uncommon disease characterized by a fibrous reaction that takes place in the peri-aortic retroperitoneum and often entraps the ureters causing obstructive uropathy. RPF is idiopathic in the majority of cases, but can also be secondary to malignancies, infections, drugs, radiotherapy, and rare histiocytic disorders such as Erdheim-Chester disease. Idiopathic RPF is an immune-mediated disease, which can either be isolated, associated with other autoimmune diseases, or arise in the context of a multifocal fibro-inflammatory disorder recently renamed as IgG4-related disease. The differential diagnosis between idiopathic, IgG4-related and secondary RPF is crucial, essentially because the therapeutic approaches - especially of idiopathic vs. secondary RPF - can be dramatically different. This review focuses on the clinical, laboratory and imaging features of the different RPF forms, and also provides an overview of the available treatment options.

  • prednisone versus tamoxifen in patients with idiopathic Retroperitoneal Fibrosis an open label randomised controlled trial
    The Lancet, 2011
    Co-Authors: Augusto Vaglio, Carlo Salvarani, Alessandra Palmisano, Domenico Corradi, Federico Alberici, Umberto Maggiore, S Ferretti, Rocco Cobelli, F Ferrozzi, Carlo Buzio
    Abstract:

    Summary Background Glucocorticoids are the mainstay of treatment of idiopathic Retroperitoneal Fibrosis, but they often have substantial toxic effects. Several reports have suggested tamoxifen as an alternative to glucocorticoids. We compared the efficacy of prednisone with that of tamoxifen in maintainance of remission in patients with idiopathic Retroperitoneal Fibrosis. Methods In this open-label, randomised controlled trial, we enrolled patients aged 18–85 years with newly diagnosed idiopathic Retroperitoneal Fibrosis at the Parma Hospital, Parma, Italy, between Oct 1, 2000, and June 30, 2006. After induction therapy with 1 mg/kg daily of prednisone for 1 month, the patients who achieved remission were randomly assigned to receive tapering prednisone (initial dose 0·5 mg/kg daily) for 8 months or tamoxifen (fixed dose 0·5 mg/kg daily) for 8 months. The sequence of randomisation (1:1), blocked in groups of two and four (with block size randomly selected), was generated by the trial statistician with a computer programme. After the end of treatment, the patients were followed up for an additional 18 months. Neither patients nor those giving interventions or analysing the data were masked to group assignment. The two radiologists who assessed CT and MRI scans were masked. The primary endpoint was the relapse rate by the end of treatment (month 8), which was analysed by intention to treat. This trial is registered with ClinicalTrials.gov, number NCT00440349. Findings After induction therapy, 36 of the 40 enrolled patients achieved remission and were randomly assigned to treatment (18 per group). One patient (6%) in the prednisone group and seven patients (39%) in the tamoxifen group relapsed by the end of treatment (difference −33% [95% CI −58 to −8, p=0·0408]. The difference in relapse rate between the groups was sustained after the additional 18-month follow-up: the 26-month estimated cumulative relapse probability was 17% with prednisone and 50% with tamoxifen (difference −33% [−62 to −3, p=0·0372]). Cushingoid changes and grade 2 hypercholesterolaemia were more common in the prednisone group than in the tamoxifen group (p=0·0116 and p=0·0408, respectively). Interpretation Prednisone is more effective in prevention of relapses than is tamoxifen in patients with idiopathic Retroperitoneal Fibrosis. Therefore, prednisone should be considered as first-line treatment for patients with newly diagnosed idiopathic Retroperitoneal Fibrosis. Funding Parma University Hospital.

  • Retroperitoneal Fibrosis evolving concepts
    Rheumatic Diseases Clinics of North America, 2007
    Co-Authors: Augusto Vaglio, Carlo Salvarani, Alessandra Palmisano, Domenico Corradi, Carlo Buzio
    Abstract:

    Retroperitoneal Fibrosis (RPF) is a rare fibro-inflammatory condition that is idiopathic in most cases, but may be secondary to various causes. Although the cause and pathogenesis of the idiopathic form are unknown, immunogenetic factors and immunopathologic/autoimmune mechanisms are probably involved. Idiopathic RPF usually develops around the abdominal aorta and iliac arteries but in some cases may also involve the thoracic aorta and the origin of its major branches, with a pattern similar to that of other forms of large-vessel vasculitis. In addition, the disease is frequently associated with autoimmune conditions affecting other organs. Glucocorticoids alone or in combination with immunosuppressive agents are usually effective treatment options, but the disease frequently has a chronic relapsing course.

  • idiopathic Retroperitoneal Fibrosis clinicopathologic features and differential diagnosis
    Kidney International, 2007
    Co-Authors: Domenico Corradi, Alessandra Palmisano, S Ferretti, Rocco Cobelli, Gabriella Moroni, Roberta Maestri, Silvia Bosio, Paolo Greco, Lucio Manenti, Carlo Buzio
    Abstract:

    Idiopathic Retroperitoneal Fibrosis (IRF) is a rare disease often causing obstructive uropathy. We evaluated the clinicopathologic features of 24 patients with IRF to characterize the histopathology of the disease and to provide a framework for the differential diagnosis with other Retroperitoneal fibrosing conditions. Retroperitoneal specimens were analyzed by light and electron microscopy and by immunohistochemistry. Most patients presented with abdominal/lumbar pain, constitutional symptoms, and high acute-phase reactants. Overall, 20 had ureteral involvement and 13 developed acute renal failure. The Retroperitoneal tissue consisted of a fibrous component and a chronic inflammatory infiltrate with the former characterized by myofibroblasts within a type-I collagen matrix. The infiltrate displayed perivascular and diffuse patterns containing lymphocytes, macrophages, plasma cells, and eosinophils. The perivascular aggregates had a central core of CD20 + cells and a mantle of CD3 + cells in equal proportions. In the areas of diffuse infiltrate, CD3 + cells outnumbered the CD20 + cells. Most plasma cells were positive for the IgG4 isotype. Small vessel vasculitis was found in the specimens of 11 patients. Our study indicates that a sclerotic background with myofibroblasts associated with a diffuse and perivascular infiltrate mainly consisting of T and B lymphocytes may be a pathological hallmark of IRF.

Gabriella Moroni - One of the best experts on this subject based on the ideXlab platform.

  • the value of 18 f fdg pet ct in the assessment of active idiopathic Retroperitoneal Fibrosis
    European Journal of Nuclear Medicine and Molecular Imaging, 2012
    Co-Authors: Gabriella Moroni, Massimo Castellani, Aurora Balzani, R Dore, Nicola Bonelli, Selena Longhi, Isabella Martinelli, Piergiorgio Messa, Paolo Gerundini
    Abstract:

    Purpose The different stages in idiopathic Retroperitoneal Fibrosis (IRF) are generally assessed by assay of inflammatory markers and analysis of contrast-enhanced CT images of the Retroperitoneal mass. We investigated the potential role of 18F-FDG PET/CT in this clinical setting.

  • idiopathic Retroperitoneal Fibrosis clinicopathologic features and differential diagnosis
    Kidney International, 2007
    Co-Authors: Domenico Corradi, Alessandra Palmisano, S Ferretti, Rocco Cobelli, Gabriella Moroni, Roberta Maestri, Silvia Bosio, Paolo Greco, Lucio Manenti, Carlo Buzio
    Abstract:

    Idiopathic Retroperitoneal Fibrosis (IRF) is a rare disease often causing obstructive uropathy. We evaluated the clinicopathologic features of 24 patients with IRF to characterize the histopathology of the disease and to provide a framework for the differential diagnosis with other Retroperitoneal fibrosing conditions. Retroperitoneal specimens were analyzed by light and electron microscopy and by immunohistochemistry. Most patients presented with abdominal/lumbar pain, constitutional symptoms, and high acute-phase reactants. Overall, 20 had ureteral involvement and 13 developed acute renal failure. The Retroperitoneal tissue consisted of a fibrous component and a chronic inflammatory infiltrate with the former characterized by myofibroblasts within a type-I collagen matrix. The infiltrate displayed perivascular and diffuse patterns containing lymphocytes, macrophages, plasma cells, and eosinophils. The perivascular aggregates had a central core of CD20 + cells and a mantle of CD3 + cells in equal proportions. In the areas of diffuse infiltrate, CD3 + cells outnumbered the CD20 + cells. Most plasma cells were positive for the IgG4 isotype. Small vessel vasculitis was found in the specimens of 11 patients. Our study indicates that a sclerotic background with myofibroblasts associated with a diffuse and perivascular infiltrate mainly consisting of T and B lymphocytes may be a pathological hallmark of IRF.

  • long term outcome of idiopathic Retroperitoneal Fibrosis treated with surgical and or medical approaches
    Nephrology Dialysis Transplantation, 2006
    Co-Authors: Gabriella Moroni, Piergiorgio Messa, Beniamina Gallelli, Giovanni Banfi, Sandro Sandri, Claudio Ponticelli
    Abstract:

    Background. Retroperitoneal Fibrosis is a severe disease that affects the ureters, causing renal insufficiency in three-quarters of patients. The optimal treatment is far from being established. Methods. Seventeen patients with idiopathic Retroperitoneal Fibrosis and ureteral entrapment followed in our unit for at least 1 year were selected for this study. At presentation 13 patients had renal insufficiency. All patients received steroids, associated with ureterolysis in five (group 1), with azathioprine in six (group 2) and with tamoxifen in six (group 3). Four patients of group 2 and five of group 3 received ureteral stenting or nephrostomy. There were no significant differences among the three groups or the clinical and biochemical characteristics at presentation. Results. All patients of groups 1 and 2 entered remission after therapy. One patient from group 3 did not respond to therapy. During a mean follow-up of 56 � 41 months, three patients (two from group 1, one from group 2, 18%) had a recurrence of the disease, which fully responded to retreatment in all three cases. At the last observation, all patients were alive; three patients (18%) had renal insufficiency, of them one from group 1 had to start dialysis 6 years after ureterolysis, one patient from group 2 and one from group 3 had serum creatinine of 1.5 mg/dl. Renal survival was 100% at 5 years and 80% at 10 years. Conclusions. In most patients, each of the three different therapeutic approaches restored renal function and significantly reduced the fibrotic mass in the short-term and maintained stable serum creatinine in the long-term.

Michael D Stifelman - One of the best experts on this subject based on the ideXlab platform.

  • utility of mri features for differentiation of Retroperitoneal Fibrosis and lymphoma
    American Journal of Roentgenology, 2012
    Co-Authors: Andrew B Rosenkrantz, Michael D Stifelman, Bradley Spieler, Claudia R Seuss, Sooah Kim
    Abstract:

    OBJECTIVE. The objective of our study was to retrospectively compare the MRI features of Retroperitoneal Fibrosis (RPF) and lymphoma presenting as confluent Retroperitoneal soft tissue. MATERIALS AND METHODS. MRI studies of 31 patients (18 men, 13 women; mean age, 58.4 ± 15.8 [SD] years; 22 with RPF and nine with lymphoma) were evaluated. Two radiologists independently and in consensus evaluated all cases for an array of subjective imaging features. A third radiologist measured the size (i.e., the greatest dimension in the transverse plane) and apparent diffusion coefficient (ADC) value of the tissue. Features of RPF and lymphoma were compared using the Fisher exact test, Mann-Whitney test, and receiver operating characteristic (ROC) curve analysis. Interreader concordance was also calculated. RESULTS. The mean age of patients with lymphoma was significantly greater than that incases of RPF (72.4 ± 13.3 [SD] vs 52.7 ± 13.2 years, respectively; p = 0.003). The MRI features significantly more common in pati...

  • robotic ureterolysis for relief of ureteral obstruction from Retroperitoneal Fibrosis
    Urology, 2011
    Co-Authors: Aryeh Y Keehn, Patrick W Mufarrij, Michael D Stifelman
    Abstract:

    Objective To review our experience with robotic surgery for the management of Retroperitoneal Fibrosis (RPF) with ureteral obstruction. Ureteral obstruction is common in Retroperitoneal Fibrosis RPF. Methods Since April 2006, 21 patients have presented to our institution with ureteral obstruction, apparently from RPF. All underwent robotic biopsy. If frozen pathology reveals malignancy, is equivocal, and/or the fibrotic reaction is extensive, we stent the obstructed side(s) and await final pathology. If RPF is confirmed, medical therapy is initiated to relieve obstruction; failures receive salvage ureterolysis. Lymphomas are referred to medical oncology. If frozen pathology demonstrates RPF, immediate ureterolysis is performed, if technically feasible. Ureterolysis is not performed for uninvolved contralateral systems. We reviewed data with institutional review board approval. Results Of 21 patients, 3 were diagnosed with lymphoma and 18 with RPF. Seventeen patients (21 renal units) with RPF received robotic ureterolysis (11 primary, 6 salvage); the other patient died of trauma before intervention. The only perioperative complication, an enterocutaneous fistula, required bowel resection. Three patients required a secondary procedure to relieve obstruction. At a mean follow-up of 20.5 months, no renal unit has evidence of obstruction, and all patients have improved or resolved symptoms. Furthermore, none of the 13 patients who underwent a unilateral ureterolysis have had disease progression to the contralateral side. Conclusions Robotic ureterolysis can be performed with minimal morbidity and provides durable success rates for relief of symptoms and obstruction in RPF. Biopsy remains integral to ruling out lymphoma. Empiric contralateral ureterolysis may not be necessary.

  • minimally invasive management of Retroperitoneal Fibrosis
    Urology, 2008
    Co-Authors: Michael D Stifelman, Patrick W Mufarrij, Ojas Shah, Michael E Lipkin
    Abstract:

    OBJECTIVES Ureteral obstruction is a common finding in Retroperitoneal Fibrosis (RPF). The management of ureteral obstruction in patients with RPF is challenging and controversial. To our knowledge we are the first to report on laparoscopic ureterolysis (LU) and robotic ureterolysis (RU) for the management of RPF to determine feasibility and success. METHODS We reviewed the charts of all patients who have undergone laparoscopic ureterolysis with or without robotic assistance at our institution. RESULTS Between 2001 and 2006, one surgical team performed laparoscopic ureterolysis with or without robotic assistance on 15 renal units in 10 patients. Mean age was 50.9 years (range, 28 to 71 years). Eight patients presented with back pain and all 15 renal units had radiographic evidence of obstruction. Five patients underwent LU, and 5 underwent RU. Mean operative times for bilateral and unilateral LU were 509.0 and 110 minutes, and the mean estimated blood loss was 362.5 and 50 mL. Mean operative times for bilateral and unilateral RU were 390 and 220.5 minutes, and the mean estimated blood loss was 25 and 35.5 mL. With a mean follow-up of 15.6 months, 90% of all patients were asymptomatic and 86.7% renal units had no signs of obstruction on imaging. CONCLUSIONS Laparoscopic ureterolysis with or without robotics may be performed with minimal perioperative morbidity and provides excellent success rates for relief of symptoms and obstruction in RPF. RU appears to have better short-term outcomes and is now our technique of choice.

Richard D Swartz - One of the best experts on this subject based on the ideXlab platform.

  • coronary artery involvement in idiopathic Retroperitoneal Fibrosis computed tomographic findings
    Journal of Thoracic Imaging, 2012
    Co-Authors: Katherine E Maturen, Baskaran Sundaram, Wendy Marder, Richard D Swartz
    Abstract:

    We describe 2 patients with perivascular low-attenuation soft tissue and inflammatory changes surrounding the coronary arteries in the clinical setting of idiopathic Retroperitoneal Fibrosis. Neither patient had inducible ischemia on cardiac stress testing. The coronary arteries present an additional site of interest as connections between sclerosing diseases--including Retroperitoneal Fibrosis, fibrosing mediastinitis, autoimmune pancreatitis, sclerosing cholangitis, and other entities--continue to emerge.

  • idiopathic Retroperitoneal Fibrosis a review of the pathogenesis and approaches to treatment
    American Journal of Kidney Diseases, 2009
    Co-Authors: Richard D Swartz
    Abstract:

    Idiopathic Retroperitoneal Fibrosis (IRPF) is an increasingly recognized syndrome. The development of inflammation and Fibrosis in the retroperitoneum most often results in a periaortic mass on computed tomography or magnetic resonance imaging that causes pain and constitutional symptoms. Its organ involvement results in urinary tract obstruction, bowel dysfunction, and venous compression with leg swelling, or thrombosis. The syndrome appears autoimmune in nature, but has no specific immunologic markers. However, nonspecific inflammatory indicators, such as sedimentation rate and C-reactive protein level, reflect disease activity and therapeutic response. Retroperitoneal Fibrosis also can arise secondary to inflammatory, infectious, or malignant disease in Retroperitoneal organs, in which case treatment is directed at the primary process. However, in patients with IRPF, initial treatment of the local mechanical complications must be followed by medical therapy with corticosteroids or, more recently, the addition of steroid-sparing agents. Although there are no controlled therapeutic trials, a number of reports with as few as 3 or as many as 28 cases describe sustained and effective steroid-sparing treatment with cyclophosphamide, azathioprine or colchicine, or such newer agents as mycophenolate mofetil or tamoxifen. Overall, IRPF responds to corticosteroid therapy initially but recurs without prolonged treatment. Sustained remission can be attained with steroid-sparing treatment. Kidney function can be preserved, and local organ dysfunction can remit for periods of 10 years or more. Although not randomized or controlled, the evidence convincingly supports a combination of initial surgical or urological intervention, along with early corticosteroid therapy for up to 6 months followed by either mycophenolate or tamoxifen for 1 to 3 years. What was previously believed to be an uncommon and challenging syndrome can be treated successfully when recognized by its characteristic presentation.

  • idiopathic Retroperitoneal Fibrosis a role for mycophenolate mofetil
    Clinical Nephrology, 2008
    Co-Authors: Richard D Swartz, A M Lake, William W Roberts, Gary J Faerber, Jr Js Wolf
    Abstract:

    Purpose Idiopathic Retroperitoneal Fibrosis (IRPF) is an unusual progressive illness for which consistent therapeutic recommendations have not been devised. The present report describes a collaborative nephrology and urology approach to distinguish IRPF from secondary disease and then combine necessary acute surgical or radiological intervention with short-term corticosteroid and with mycophenolate mofetil (MM) to facilitate steroid tapering and long-term management. Materials and methods 21 patients have been evaluated and followed over a 7-year period, 16 with characteristic IRPF and 5 with secondary Retroperitoneal disease. IRPF patients initially received high-dose corticosteroid and MM. We report clinical follow-up along with imaging studies of the retroperitoneum and related organs, serologic markers for systemic disease, and nonspecific acute-phase reactants as indicators of ongoing disease activity. Results Among IRPF patients, uniform success in stabilizing clinical signs and symptoms, radiological disease in the retroperitoneum and associated organs, and inflammatory indicators have been observed. Corticosteroid therapy can be limited to 6 months or less and MM to approximately 2 years, all with substantial impact on the natural history of IRPF. Conclusions This is not a randomized, controlled trial, and patients were often referred with prior complications and/or treatments, however, the systematic approach and consistent results support the utility of MM as a safe and effective choice for long-term stabilization in IRPF.