Idiopathic retroperitoneal fibrosis: an update for nephrologists

2020 ◽  
Author(s):  
Valentina Raglianti ◽  
Giovanni M Rossi ◽  
Augusto Vaglio
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Obstructive Uropathy ◽  
Human Leukocyte ◽  
Rare Condition ◽  
Biologic Drugs ◽  
Idiopathic Retroperitoneal Fibrosis ◽  
Immunoglobulin G4 ◽  
Increased Risk ◽  
Environmental Agents ◽  
Stage Renal Disease

Abstract Idiopathic retroperitoneal fibrosis (IRF) is a rare condition characterized by the development of a peri-aortic and peri-iliac tissue showing chronic inflammatory infiltrates and pronounced fibrosis. Ureteral entrapment with consequent obstructive uropathy is one of the most common complications of IRF, which can lead to acute renal failure and, in the long term, to varying degrees of chronic kidney disease. IRF may be isolated or develop in association with autoimmune diseases (e.g. Hashimoto’s thyroiditis and psoriasis) and other fibro-inflammatory disorders (often within the spectrum of immunoglobulin G4-related disease), which suggests that it should be considered as a potentially systemic condition. IRF is an immune-mediated disease: genetic variants (e.g. human leukocyte antigen (HLA)-DRB1*03) and environmental agents (mainly exposure to asbestos and smoking) are strongly associated with an increased risk of developing the disease, while a complex network of chemokines (e.g. CXCL12 and C-C moti chemokine 11 (CCL11)) and cytokines [e.g. interleukin (IL)-6, IL-12 and IL-13] is likely to orchestrate the inflammatory response and simultaneously promote fibrosis. Glucocorticoids, alone or in combination with traditional immunosuppressants such as methotrexate and mycophenolate mofetil, are usually efficacious and promptly induce disease remission; however, up to 50% of patients relapse, thus requiring repeat immunosuppressive courses. Biologic drugs, namely rituximab, are being explored for the treatment of IRF. In addition to medical therapies, interventional procedures (mainly ureteral stenting) are required to relieve ureteral obstruction, whereas surgical ureterolysis is generally reserved to refractory cases. If appropriately treated, then the overall and renal prognosis of IRF are good, with <5% patients developing end-stage renal disease.

scholarly journals IDIOPATHIC RETROPERITONEAL FIBROSIS: A RARE CAUSE OF ABDOMINAL PAIN AND OBSTRUCTIVE UROPATHY

CHEST Journal ◽  
2019 ◽  
Vol 156 (4) ◽  
pp. A1988-A1989
Author(s):  
Mariam Saeed ◽  
Muneer Khan ◽  
Moses Bachan ◽  
Zinobia Khan
Keyword(s):  
Abdominal Pain ◽  
Retroperitoneal Fibrosis ◽  
Obstructive Uropathy ◽  
Idiopathic Retroperitoneal Fibrosis

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 Associations between human leukocyte antigens and renal function

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Marcus Lowe ◽  
Antony Payton ◽  
Arpana Verma ◽  
Judith Worthington ◽  
Isla Gemmell ◽  
...  
Keyword(s):  
Renal Function ◽  
Renal Disease ◽  
Human Leukocyte ◽  
Secondary Outcome ◽  
Exact Nature ◽  
Human Leukocyte Antigens ◽  
Leukocyte Antigens ◽  
Increased Risk ◽  
Stage Renal Disease ◽  
End Stage

AbstractHuman leukocyte antigens (HLA) have been associated with renal function, but previous studies report contradictory findings with little consensus on the exact nature or impact of this observation. This study included 401,307 white British subjects aged 39–73 when they were recruited by UK Biobank. Subjects’ HLA types were imputed using HLA*IMP:02 software. Regression analysis was used to compare 362 imputed HLA types with estimated glomerular filtration rate (eGFR) as a primary outcome and clinical indications as secondary outcome measures. 22 imputed HLA types were associated with increased eGFR (and therefore increased renal function). Decreased eGFR (decreased renal function) was associated with 11 imputed HLA types, seven of which were also associated with increased risk of end-stage renal disease and/or chronic kidney disease. Many of these HLA types are commonly inherited together in established haplotypes, for example: HLA-A*01:01, B*08:01, C*07:01, DRB1*03:01, DQB1*02:01. This haplotype has a population frequency of 9.5% in England and each allele was associated with decreased renal function. 33 imputed HLA types were associated with kidney function in white British subjects. Linkage disequilibrium in HLA heritance suggests that this is not random and particularly affects carriers of established haplotypes. This could have important applications for the diagnosis and treatment of renal disease and global population health.

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.

scholarly journals A Rare Case of Erdheim-Chester Disease and Langerhans Cell Histiocytosis Overlap Syndrome

2015 ◽  
Vol 2015 ◽  
pp. 1-7
Author(s):  
Shahzaib Nabi ◽  
Adeel Arshad ◽  
Tarun Jain ◽  
Fawad Virk ◽  
Rohit Gulati ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Retroperitoneal Fibrosis ◽  
Obstructive Uropathy ◽  
Altered Mental Status ◽  
Langerhans Cell ◽  
Erdheim Chester Disease ◽  
Stage Renal Disease ◽  
End Stage ◽  
Erdheim Chester ◽  
Chester Disease

A 48-year-old woman with a past medical history of seizures and end-stage renal disease secondary to obstructive uropathy from retroperitoneal fibrosis presented to the emergency department with seizures and altered mental status. A Glasgow Coma Scale of 4 prompted intubation, and she was subsequently admitted to the intensive care unit. Magnetic resonance imaging of the brain performed to elucidate the aetiology of her seizure showed a dural-based mass within the left temporoparietal lobe as well as mass lesions within the orbits. Further imaging showed extensive retroperitoneal fibrosis extending to the mediastinum with involvement of aorta and posterior pleural space. Imaging of the long bones showed bilateral sclerosis and cortical thickening of the diaphyses. Imaging of the maxillofacial structures showed osseous destructive lesions involving the mandible. These clinical and radiological features were consistent with a diagnosis of Erdheim-Chester disease; however, the patient’s skin biopsy was consistent with Langerhans cell histiocytosis.

A case of idiopathic retroperitoneal fibrosis

2020 ◽  
Vol 8 (1) ◽  
pp. 101-107
Author(s):  
S.V. Shchekaturov ◽  
◽  
M.M. Kaabak ◽  
A.K. Zokoev ◽  
E.R. Charchyan ◽  
...  
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Idiopathic Retroperitoneal Fibrosis

Could Antioxidant Supplementation Delay Progression of Cardiovascular Disease in End-Stage Renal Disease Patients?

2020 ◽  
Vol 19 (1) ◽  
pp. 41-54 ◽  
Author(s):  
Stefanos Roumeliotis ◽  
Athanasios Roumeliotis ◽  
Xenia Gorny ◽  
Peter R. Mertens
Keyword(s):  
Oxidative Stress ◽  
Renal Disease ◽  
End Stage Renal Disease ◽  
Close Association ◽  
Omega 3 ◽  
Endstage Renal Disease ◽  
Increased Risk ◽  
Underlying Mechanisms ◽  
Stage Renal Disease ◽  
End Stage

In end-stage renal disease patients, the leading causes of mortality are of cardiovascular (CV) origin. The underlying mechanisms are complex, given that sudden heart failure is more common than acute myocardial infarction. A contributing role of oxidative stress is postulated, which is increased even at early stages of chronic kidney disease, is gradually augmented in parallel to progression to endstage renal disease and is further accelerated by renal replacement therapy. Oxidative stress ensues when there is an imbalance between reactive pro-oxidants and physiologically occurring electron donating antioxidant defence systems. During the last decade, a close association of oxidative stress with accelerated atherosclerosis and increased risk for CV and all-cause mortality has been established. Lipid peroxidation has been identified as a trigger for endothelial dysfunction, the first step towards atherogenesis. In order to counteract the deleterious effects of free radicals and thereby ameliorate, or delay, CV disease, exogenous administration of antioxidants has been proposed. Here, we attempt to summarize existing data from studies that test antioxidants for CV protection, such as vitamins E and C, statins, omega-3 fatty acids and N-acetylcysteine.

scholarly journals Uremic Toxins and Their Relation with Oxidative Stress Induced in Patients with CKD

2021 ◽  
Vol 22 (12) ◽  
pp. 6196
Author(s):  
Anna Pieniazek ◽  
Joanna Bernasinska-Slomczewska ◽  
Lukasz Gwozdzinski
Keyword(s):  
Oxidative Stress ◽  
Uremic Toxins ◽  
Water Medium ◽  
Induce Insulin Resistance ◽  
Risk Of Mortality ◽  
Increased Risk ◽  
Stage Renal Disease ◽  
End Stage

The presence of toxins is believed to be a major factor in the development of uremia in patients with chronic kidney disease (CKD) and end-stage renal disease (ESRD). Uremic toxins have been divided into 3 groups: small substances dissolved in water, medium molecules: peptides and low molecular weight proteins, and protein-bound toxins. One of the earliest known toxins is urea, the concentration of which was considered negligible in CKD patients. However, subsequent studies have shown that it can lead to increased production of reactive oxygen species (ROS), and induce insulin resistance in vitro and in vivo, as well as cause carbamylation of proteins, peptides, and amino acids. Other uremic toxins and their participation in the damage caused by oxidative stress to biological material are also presented. Macromolecules and molecules modified as a result of carbamylation, oxidative stress, and their adducts with uremic toxins, may lead to cardiovascular diseases, and increased risk of mortality in patients with CKD.

scholarly journals Variable Symptomatology in Idiopathic Retroperitoneal Fibrosis

1983 ◽  
Vol 76 (12) ◽  
pp. 1023-1025 ◽  
Author(s):  
C D M Fletcher ◽  
P E M Jarrett
Keyword(s):  
Retroperitoneal Fibrosis ◽  
District Hospital ◽  
Differential Diagnoses ◽  
General Surgeon ◽  
Idiopathic Retroperitoneal Fibrosis

Idiopathic retroperitoneal fibrosis is uncommon enough to be placed low down on a list of differential diagnoses of any problem facing a general surgeon in a district hospital. In this paper, 4 cases of retroperitoneal fibrosis are described, all of whom presented within a 5-year period to the same surgeon; each described a different symptomatology. This paper serves to reiterate the diversity of presenting features in retroperitoneal fibrosis and to demonstrate that this condition may not be as rare as is widely believed.

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