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Journal of the American Society of... Jul 2016Idiopathic retroperitoneal fibrosis (RPF), reviewed herein, is a rare fibro-inflammatory disease that develops around the abdominal aorta and the iliac arteries, and... (Review)
Review
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.
Topics: Algorithms; Autoimmune Diseases; Humans; Inflammation; Retroperitoneal Fibrosis; Treatment Outcome
PubMed: 26860343
DOI: 10.1681/ASN.2015101110 -
Archivos Espanoles de Urologia Jan 2020Retroperitoneal fibrosis (RPF) is an uncommon disease due an inflammatory condition and deposit of fibrotic tissue that involves the retroperitoneal area over the lower... (Review)
Review
OBJECTIVES
Retroperitoneal fibrosis (RPF) is an uncommon disease due an inflammatory condition and deposit of fibrotic tissue that involves the retroperitoneal area over the lower four lumbar vertebrae. Very few epidemiologic studies exist that accurately characterize the incidence and prevalence of the disease.
MATERIALS AND METHODS
A review of the English language literature was performed using the MEDLINE combining the keywords: "retroperitoneal fibrosis", "Ormond´ disease", "IG4 related disease". Additionally, hand search of bibliographies of included studies and previous reviews was also performed to include additional information.
RESULTS
RPF develops insidiously, because the initial symptoms are non-specific. Pain is the most common presenting symptom. Various radiological diagnostic methods are used in the diagnosis of retroperitoneal fibrosis. Contrast-enhanced computerized tomography (CT) is a useful method for diagnosing retroperitoneal pathologies. Magnetic resonance imaging (MRI) is an important radiological method especially in the diagnosis of fibrotic tissue and in the examination of the retroperitoneal organ relation with fibrous tissue. Nuclear imaging is also a method used in renal function evaluation and patient follow-up. Various medical and surgical treatments would be used in the treatment of retroperitoneal fibrosis. In general, immunosuppressive agents such as corticosteroids, tamoxifen, azothiopurine, cyclophosphamide, cyclosporine, progesterone, mycophenolate mofetil are used in medical treatment. Surgical treatment methods are recommended in cases where medical treatment is not efficient.
CONCLUSION
Unfortunately, despite a recent surge in the number of publications on this topic, a few progress has been made in our understanding of the classification, pathophysiology, and, most importantly, the most appropriate treatment for this disease.
Topics: Fibrosis; Humans; Magnetic Resonance Imaging; Retroperitoneal Fibrosis; Retroperitoneal Space; Tomography, X-Ray Computed
PubMed: 31950925
DOI: No ID Found -
Lancet (London, England) Jan 2006Retroperitoneal fibrosis encompasses a range of diseases characterised by the presence of a fibro-inflammatory tissue, which usually surrounds the abdominal aorta and... (Review)
Review
Retroperitoneal fibrosis encompasses a range of diseases characterised by the presence of a fibro-inflammatory tissue, which usually surrounds the abdominal aorta and the iliac arteries and extends into the retroperitoneum to envelop neighbouring structures--eg, ureters. Retroperitoneal fibrosis is generally idiopathic, but can also be secondary to the use of certain drugs, malignant diseases, infections, and surgery. Idiopathic disease was thought to result from a local inflammatory reaction to antigens in the atherosclerotic plaques of the abdominal aorta, but clinicolaboratory findings--namely, the presence of constitutional symptoms and the high concentrations of acute-phase reactants--and the frequent association of the disease with autoimmune diseases that involve other organs suggest that it might be a manifestation of a systemic autoimmune or inflammatory disease. Steroids are normally used to treat idiopathic retroperitoneal fibrosis, although other options--eg, immunosuppressants, tamoxifen--are available. The outlook is usually good, but, if not appropriately diagnosed or treated, the disease can cause severe complications, such as end-stage renal failure. Here, we review the different aspects of retroperitoneal fibrosis, focusing on idiopathic retroperitoneal fibrosis and on the differential diagnosis associated with the secondary forms.
Topics: Adrenal Cortex Hormones; Diagnosis, Differential; Humans; Magnetic Resonance Imaging; Retroperitoneal Fibrosis; Tomography, X-Ray Computed
PubMed: 16427494
DOI: 10.1016/S0140-6736(06)68035-5 -
Journal of Nephrology Aug 2016IgG4-related disease (IgG4-RD) is a recently recognized disorder, often with multiple organ involvement, characterized by dense tissue infiltration of IgG4-positive... (Review)
Review
IgG4-related disease (IgG4-RD) is a recently recognized disorder, often with multiple organ involvement, characterized by dense tissue infiltration of IgG4-positive plasma cells, storiform fibrosis, obliterative phlebitis and frequently elevated serum IgG4 concentration. The kidney can be involved either directly or indirectly. The most frequent direct renal manifestations of IgG4-RD are IgG4-related tubulointerstitial nephritis (TIN) and membranous glomerulonephropathy. Retroperitoneal fibrosis (RPF) is another condition that is frequently IgG4-related and that can indirectly affect the kidney causing ureteral obstruction and hydronephrosis. Contrast-enhanced computerized tomography, magnetic resonance imaging and (18)F-fluorodeoxyglucose positron emission tomography/computed tomography show different imaging findings and are useful tools for monitoring therapeutic response. Steroid treatment is the first line of therapy, but relapsing or refractory forms of the disease are frequently observed and require more aggressive therapeutic approaches. At our centre, we treated three cases of aggressive IgG4-related TIN and two cases of IgG4-related RPF with an intensified, immune suppressive protocol, obtaining good results without severe adverse effects.
Topics: Glomerulonephritis, Membranous; Humans; Immunoglobulin G; Kidney; Nephritis, Interstitial; Retroperitoneal Fibrosis
PubMed: 26972314
DOI: 10.1007/s40620-016-0279-4 -
Rheumatic Diseases Clinics of North... May 2013Retroperitoneal fibrosis (RPF) is a condition characterized by the presence of inflammation and fibrosis in the retroperitoneal space, for which no standard diagnostic... (Review)
Review
Retroperitoneal fibrosis (RPF) is a condition characterized by the presence of inflammation and fibrosis in the retroperitoneal space, for which no standard diagnostic criteria exist. Historically, treatment has focused on relieving the obstruction with percutaneous or cystoscopic assisted placement of ureteral stents followed by more definitive resolution of ureteric obstruction with open or laparoscopic ureterolysis. However, over the past several years management has shifted from primarily a surgical approach to an immunosuppressive-based therapy aimed at modulation of the immune system. This review focuses on the recent advances in the classification, epidemiology, pathophysiology, pathology, imaging, and treatment of RPF.
Topics: Aortic Diseases; Comorbidity; Disease Management; Humans; Immunosuppressive Agents; Retroperitoneal Fibrosis; Stents; Ureter
PubMed: 23597969
DOI: 10.1016/j.rdc.2013.02.004 -
Best Practice & Research. Clinical... Aug 2012Retroperitoneal fibrosis is a rare syndrome hallmarked by a fibrosclerotic tissue in the retroperitoneum, often leading to encasement of the ureters. About two-thirds of... (Review)
Review
Retroperitoneal fibrosis is a rare syndrome hallmarked by a fibrosclerotic tissue in the retroperitoneum, often leading to encasement of the ureters. About two-thirds of cases of retroperitoneal fibrosis are idiopathic, while the remaining cases are secondary to a variety of different causes, including drugs, tumors and infections. Idiopathic retroperitoneal fibrosis may be associated with abdominal aorta aneurysms, with vasculitis of the thoracic aorta and of epi-aortic vessels, or both. Most patients present with abdominal and/or low back pain. Serum markers of inflammation are usually, but not invariably, elevated. The diagnosis is secured by computed tomography or magnetic resonance imaging, which typically show an enhancing retroperitoneal mass medially dislodging the ureters. Positron emission tomography can be useful to document the extent and metabolic activity of the inflammatory process. Treatment rests on glucocorticoids with a tapering scheme variably combined with immunosuppressive agents. In cases of ureter obstruction, relief of obstruction by stenting or ureterolysis is required.
Topics: Biomarkers; Flank Pain; Glucocorticoids; Humans; Immunosuppressive Agents; Low Back Pain; Magnetic Resonance Imaging; Positron-Emission Tomography; Rare Diseases; Retroperitoneal Fibrosis; Tomography, X-Ray Computed; Ureteral Obstruction
PubMed: 23040359
DOI: 10.1016/j.berh.2012.07.004 -
Journal of the Royal Society of Medicine Jul 1979
Topics: Diagnosis, Differential; Humans; Hypertension; Retroperitoneal Fibrosis; Retroperitoneal Neoplasms; Steroids; Urography
PubMed: 552544
DOI: 10.1177/014107687907200703 -
Annals of the Rheumatic Diseases Mar 2019IgG4-related disease (IgG4-RD) is a heterogeneous, multiorgan condition of unclear aetiology that can cause organ failure. Difficulty recognising IgG4-RD contributes to...
OBJECTIVE
IgG4-related disease (IgG4-RD) is a heterogeneous, multiorgan condition of unclear aetiology that can cause organ failure. Difficulty recognising IgG4-RD contributes to diagnostic delays. We sought to identify key IgG4-RD phenotypes.
METHODS
We used two cross-sectional studies assembled by an international, multispecialty network of IgG4-RD specialists who submitted 765 cases to derive and replicate phenotypic groups. Phenotype groups of disease manifestations and key covariate distributions across the identified groups were measured using latent class analysis.
RESULTS
In the derivation cohort (n=493), we identified four groups with distinct manifestations: Group 1 (31%), Pancreato-Hepato-Biliary disease; Group 2 (24%), Retroperitoneal Fibrosis and/or Aortitis; Group 3 (24%), Head and Neck-Limited disease and Group 4 (22%), classic Mikulicz syndrome with systemic involvement. We replicated the identification of four phenotype groups in the replication cohort. Compared with cases in Groups 1, 2 and 4, respectively, cases in Group 3 were more likely to be female (OR 11.60 (95% CI 5.39 to 24.98), 10.35 (95% CI 4.63 to 23.15) and 9.24 (95% CI 3.53 to 24.20)) and Asian (OR 6.68 (95% CI 2.82 to 15.79), 7.43 (95% CI 2.97 to 18.56) and 6.27 (95% CI 2.27 to 17.29)). Cases in Group 4 had a higher median serum IgG4 concentration (1170 mg/dL) compared with groups 1-3 (316, 178 and 445 mg/dL, respectively, p<0.001).
CONCLUSION
We identified four distinctive IgG4-RD phenotypes according to organ involvement. Being Asian or female may predispose individuals to head and neck-limited disease. These phenotypes serve as a framework for identifying IgG4-RD and studying its aetiology and optimal treatment.
Topics: Adult; Americas; Aortitis; Asia; Asian People; Cross-Sectional Studies; Digestive System Diseases; Europe; Female; Humans; Immunoglobulin G; Immunoglobulin G4-Related Disease; Male; Middle Aged; Mikulicz' Disease; Otorhinolaryngologic Diseases; Phenotype; Racial Groups; Retroperitoneal Fibrosis
PubMed: 30612117
DOI: 10.1136/annrheumdis-2018-214603 -
Medicina 2022Some patients diagnosed with idiopathic retroperitoneal fibrosis could be reclassified as IgG4-related disease (IgG4-RD). Classification criteria have not been uniform...
Some patients diagnosed with idiopathic retroperitoneal fibrosis could be reclassified as IgG4-related disease (IgG4-RD). Classification criteria have not been uniform and prevalence of IgG4-related retroperitoneal fibrosis (IgG4-RPF) is unknown in our region. We aimed to describe IgG4-RPF frequency relying on criteria published recently and comparing clinical, histopathologic and radiologic features with non-IgG4-RPF. From January, 2005 to December, 2020, nineteen adults with histopathologic diagnosis of idiopathic retroperitoneal fibrosis were included in a dynamic retrospective cohort at Hospital Italiano de Buenos Aires. Pathology slides were reviewed and immunohistochemistry was performed and assessed for each case. We used classification criteria described in 2019 American College of Rheumatology/European League Against Rheumatism to identify IgG4-RD cases. Ten of 19 patients met criteria for IgG4-RPF. Median age was similar in two subsets (61 versus 55, p = 0.2) and both had male predominance. Three out of 10 patients (p = 0.2) featured other manifestations of IgG4-RD in the IgG4-RPF group, and periaortic fibrosis was the most significant finding in images (p = 0.01). Corticosteroids were mostly used as therapy, followed by azathioprine and rituximab. Most patients did not receive specific treatment. IgG4-RPF patients had dense lymphocytic infiltrate and 8 out of 10 showed storiform fibrosis (p = 0.02). IgG4+ cells/hpf and IgG4/IgG ratio were significantly higher (p = 0.01). Over half of the patients in our cohort met the criteria of IgG4-RPF. New criteria may harmonize the identification of IgG4-RD. As IgG4-RD may be reversible at initial stages, these findings may lead to early recognition, treatment and integral follow-up.
Topics: Humans; Immunoglobulin G; Immunoglobulin G4-Related Disease; Male; Retroperitoneal Fibrosis; Retrospective Studies; Rituximab
PubMed: 35037866
DOI: No ID Found -
Prague Medical Report 2015Ormond's disease is a relatively rare disease with unclear etiology, characterized by chronic periaortitis and retroperitoneal fibrosis. The inflammatory process affects... (Review)
Review
Ormond's disease is a relatively rare disease with unclear etiology, characterized by chronic periaortitis and retroperitoneal fibrosis. The inflammatory process affects the infrarenal part of the abdominal aorta and the iliac arteries, and the presence of infiltrates encasing the ureters and inferior vena cava. This disease is currently classed as an IgG4-related disease. In our review we analyse the clinical history, diagnostic approaches, surgical and immunosuppressive therapies.
Topics: Autoimmune Diseases; Immunoglobulin G; Immunosuppressive Agents; Retroperitoneal Fibrosis; Urologic Surgical Procedures
PubMed: 26445389
DOI: 10.14712/23362936.2015.57