Retroperitoneal Fibrosis Research Articles

Increasing Cases of Retroperitoneal Fibrosis After Covid-19: Case Report, Did She Die Retroperitoneal Fibrosis or Cancer?

This case report details the unfortunate journey of a 59-year-old breast cancer survivor who developed secondary Retroperitoneal Fibrosis (RPF) in 2022. Despite diligent treatment efforts, her condition took a devastating turn when, in June 2023, she was diagnosed not only with persistent RPF but also with advanced liver, omental, and duodenal cancer. Tragically, her condition deteriorated rapidly, leading to her passing on day 41 following her presentation. This case underscores the challenges of diagnosing and treating RPF. It highlights the importance of considering cancer development in advanced RPF cases that do not respond to treatment, often leaving limited options for effective intervention.

Cardiovascular manifestations of Erdheim-Chester disease: A narrative review with two cases.

Erdheim-Chester disease (ECD) is a rare 'L' (Langerhans) group histiocytic neoplasm that affects a multitude of organ systems, causing osteosclerotic bone lesions, periaortic encasement ('coated' aorta), retroperitoneal fibrosis involving kidneys and ureters ('hairy kidney'), and infiltration of the central nervous system. Cardiovascular involvement can occur in up to 70% of patients and is usually found during computed tomography/magnetic resonance imaging evaluation. When present, cardiovascular symptoms can have wide variability in presentation from asymptomatic to pericarditis, fatal cardiac tamponade, myocardial infarction, conduction abnormalities, heart failure, renal artery stenosis, and claudication. Cardiac involvement found on imaging includes right atrial pseudotumor, right atrioventricular groove infiltration, and pericardial effusions. ECD can involve the large- and medium-sized arteries, often seen as periarterial thickening (commonly coating the aorta) with stenosis/occlusion. Although more cardiovascular ECD cases have begun to be published in the literature, more data are needed on the outcomes of these patients, as well as how cardiovascular manifestations respond to treatment of ECD.

IgG4-Related Infiltrative Kidney Disease without Extrarenal Organ Involvement: A Case Report

Background: IgG4-related disease (RD) is a systemic condition that can affect nearly all organs. Renal involvement encompasses interstitial nephritis, membranous nephropathy, and renal infiltrative lesions. Symptomatic IgG4-related kidney disease (RKD) without extrarenal organ manifestations is an exceptionally rare occurrence. Case Report: The authors present a case of 48-year-old male with isolated renal IgG4-RD presented with abdominal pain. Laboratory investigations revealed acute kidney injury, proteinuria, and hypoalbuminemia. Whole abdominal computed tomography (CT) scan disclosed infiltrative lesions in both kidneys and ureters, accompanied by hydronephrosis. Kidney biopsy demonstrated lymphocytic and plasma cell infiltration, with 82 plasma cells per high power field (HPF), storiform fibrosis, and the absence of obliterative phlebitis. After receiving corticosteroid treatment, the patient’s renal function improved, and the infiltrative lesions went into remission. Conclusion: Isolated renal IgG4-RD is a rare and challenging diagnosis. The disease can manifest as acute interstitial nephritis, glomerular disease, retroperitoneal fibrosis, or, rarely, as mass or infiltrative lesions. Corticosteroid therapy is likely to yield favorable responses, hence, prompt diagnosis is crucial. Delayed diagnosis may result in organ fibrosis and dysfunction. Keywords: IgG4-related disease; IgG4-related kidney disease; Infiltrative renal disease

IgG4-related retroperitoneal fibrosis mimicking renal pelvis tumor: a case report and literature review

BackgroundRetroperitoneal fibrosis (RPF) is a rare disease characterized by the development of a fibroinflammatory mass in the retroperitoneum. Immunoglobulin-G4 related RPF was suggested as a secondary form of RPF and thought to be part of the spectrum of Immunoglobulin-G4 related diseases (IgG4-RD). Patients often present to the clinic because of flank pain. Ranging from mild to end-stage renal failure can be observed. The main purpose of treatment is to preserve renal function. As it is a rare condition, there is no definite treatment strategy. We report a case of 39-year-old man with left flank pain and diagnosis of IgG4-related RPF mimicking a renal pelvis tumor.Case presentationA 39-year-old male patient presented with left flank pain. MRI suggested solid retroperitoneal mass associated with hydronephrosis in the left kidney collecting system. Upon identifying the retroperitoneal origin of the mass during nephroureterectomy, the procedure was concluded following the acquisition of frozen section and routine pathological samples from the lesion. In the histopathological examination, inflammatory cells were observed and specific immunohistochemistry for IgG-4 was detected focally positive. Following the placement of a DJ stent, immunosuppressive therapy was initiated with Prednol and Azathioprine. After a one-year follow-up period, during which the patient received immunosuppressive treatment and underwent tri-monthly DJ stent replacements, the DJ stent was subsequently removed, revealing complete regression of hydronephrosis.ConclusionsWith the correct diagnosis and treatment of IgG4-related RPF, it is possible to prevent irreversible complications of the disease. Because it is a rare disease, case reports in the literature will be useful for treatment.

Diagnostic value of contrast-enhanced ultrasound in the activity of idiopathic retroperitoneal fibrosis: a retrospective study.

To explore the diagnostic value of contrast-enhanced ultrasound (CEUS) in the disease activity of idiopathic retroperitoneal fibrosis (IRPF). This retrospective study included 148 CEUS examinations from 63 patients with IRPF treated in our hospital from April 2016 to September 2021. They were divided into two groups: IRPF active group (69 examinations) and inactive group (79 examinations). Uni- and multivariable analyses were used to identify independent risk factors for IRPF activity. Receiver operating characteristic (ROC) curves were drawn to establish different diagnostic models to evaluate the diagnostic value of IRPF activity. The z test was used to compare the differences of the area under the curves (AUCs). The value of CEUS in evaluating the variation of disease activity over time was also investigated between repeat patient studies. Univariate and multivariate logistic regression analyses revealed the thickness [odds ratio (OR) = 14.125, 95% confidence interval (CI) = 3.017-66.123] was the most significant independent risk factor for IRPF activity (P < 0.01). The best diagnostic model was model 3, which was established by CEUS score combined with thickness. The AUC was 0.944 (95%CI = 0.912-0.977), and the sensitivity and specificity were 89.86% and 86.08%, respectively. The diagnostic performance was not significantly improved after combining clinical symptom (back pain) and laboratory indicators [erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)]. Compared with before treatment, the CEUS score and thickness were significantly decreased after treatment (x2 = 14.580, P < 0.001 and z = 4.708, P < 0.001, respectively). CEUS has good diagnostic value for IRPF disease activity. Key points •Thickness and contrast-enhanced ultrasound score were significantly higher in the active group than those in inactive group (P < 0.001). •With thickness of 4 mm and contrast-enhanced ultrasound 2 score as optimal cut-off values, the sensitivity and specificity were 89.86%, 81.01% and 52.17%, 100.00%, respectively. •During follow-up, when the disease progressed, the change of CEUS score was earlier than the change of thickness.

Zinner's Syndrome and Retroperitoneal Fibrosis: An Unknown Association.

The value of the Internal Medicine team in the assessment of low prevalence diseases.The importance of multidisciplinary teams.Integration of the internists in the surgical teams.

Renal Manifestations of IgG4‐Related Disease: A Concise Review

IgG4‐related disease (IgG4‐RD) is an immune‐mediated disorder marked by fibro‐inflammatory masses that can infiltrate multiple organ systems. Due to its relatively recent discovery and limited understanding of its pathophysiology, IgG4‐related disease may be difficult to recognize and is consequently potentially underdiagnosed. Renal involvement is becoming regarded as one of the key features of this disease. To date, the most well‐recognized renal complication of IgG4‐related disease is tubulointerstitial nephritis, but membranous glomerulonephritis, renal masses, and retroperitoneal fibrosis have also been reported. This concise review has two objectives. First, it will briefly encapsulate the history, epidemiology, and presentation of IgG4‐related disease. Second, it will examine the reported renal manifestations of IgG4‐related disease, exploring the relevant histology, imaging, clinical features, and treatment considerations. This synthesis will be highly relevant for nephrologists, rheumatologists, general internists, and renal pathologists to raise awareness and help improve early recognition of IgG4‐related kidney disease (IgG4‐RKD).

Epidemiological, Clinical, and Evolutionary Aspects of Severe Obstructive Acute Renal Failure Requiring Dialysis in the Nephrology Department of the University Hospital of Brazzaville

Introduction: Obstructive anuria represents a medical and surgical emergency, sometimes necessitating immediate extrarenal replacement therapy. Our study aims to delineate the profile of hemodialyzed patients with obstructive acute renal failure (OARF), detailing indications for hemodialysis and observing the course of the condition. Patients and Methods: This retrospective study covers the period from 2019 to 2022, encompassing all patients with OARF requiring emergency dialysis at the Nephrology Department of the University Hospital of Brazzaville. Data analysis was performed using EPI Info software version 3.3.2 (CDC, Atlanta, USA). Results: The study involved 39 patients, with 69.23% being male. The average age was 58.10 years, and oliguria was the primary presenting symptom (82%). Tumoral obstruction accounted for 76%, while lithiasic obstruction constituted 14%. Two cases of retroperitoneal fibrosis were observed (2.8%). Neoplastic origins included prostate, bladder, cervical, and rectal cancers in 46.67%, 33.33%, 16.67%, and 3.33% of cases, respectively. Indications for hemodialysis were poorly tolerated uremia, acidosis, hyperkalemia, and pulmonary edema in 79.1%, 38.57%, 19.49%, and 13.21% of cases, respectively. The mortality rate was 25.64%. Conclusion: The reliance on hemodialysis in obstructive anuria underscores a delay in patient management. Emergency treatment revolves around urinary tract drainage, followed by addressing the underlying cause. Prevention hinges on early and adequate management of various causal pathologies.

Üreterde Cut-off İşareti

Obstructive uropathy occurs due to common causes such as kidney stones, vesicoureteral reflux, posterior urethral valve and urothelial tumors. In addition, there are rare causes such as solid tumors, retroperitoneal fibrosis, primary lymphomas (Non-Hodgkin lymphomas etc.). A 20-year old male with lower back pain underwent a bone scan for differential diagnosis of back pain. The bone scan findings were normal, except for the presence of unilateral renal stasis accompanied by a short segment ureteral stasis. Due to the abrupt termination of the ureteral activity, namely the “cutoff sign”, a subsequent SPECT/CT imaging was focused for differential diagnosis of ureteral stasis. Non-diagnostic CT sections revealed a para-aortic soft tissue mass compressing the ureter. This case underlines the added value of SPECT/CT imaging in subtle scintigraphic findings. The abrupt termination of stasis in the ureter may warrant the application of SPECT/CT in cases with no previous history of urinary tract pathology. SPECT / CT may provide additional benefits of clarifying the etiology.

Renal and Urinary Tract Involvement in Fibrosclerosing or Fibroinflammatory Diseases: A Narrative Review

Fibroinflammatory diseases are a group of rare pathologies in which the hallmark is the exuberant deposition of fibrotic tissue and inflammatory cellular infiltrates, characteristic of the specific disease. A sclerotic mass develops within soft tissues and/or organs, damaging and replacing them, with effects ranging from asymptomatic to life-threatening clinical manifestations. The kidneys and urinary tract can be involved in some of these diseases, which can lead to acute kidney injury, chronic kidney disease, and even end-stage kidney disease. IgG4-related disease, retroperitoneal fibrosis, and Erdheim–Chester disease are the three fibroinflammatory disorders that can involve the kidneys. Only a timely and accurate collection of clinical, radiological, metabolic, laboratory, and histological data allows prompt diagnosis and targeted treatment of these pathologies, allowing the stoppage of the evolution of renal and systemic manifestations, which can lead to complete remission. The epidemiology, clinical and histological features, and management of these conditions are herein described in a narrative fashion.

IgG4‐related retroperitoneal fibrosis needs comprehensive treatment and regular long‐term review

IgG4‐related retroperitoneal fibrosis is rare but eminently treatable. Here the authors discuss recently published classification criteria and how the disease is managed, including maintenance therapy once patients are in remission.

IgG4-related disease - a clinical case

Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease affecting one or more organs. Pathomorphologically, a dense infiltrate of lymphocytes and IgG4 plasmocytes, and subsequent fibrosis is detected. Despite the many hypotheses of genetic predisposition, molecular mimicry and autoimmune nature of the disease, the etiology still remains unclear. Both innate and acquired immunity are believed to play a key role in disease pathogenesis. The involvement of occupational risk factors is also discussed. The possible involvement of multiple organs and systems (most often lacrimal and salivary glands, kidneys, lungs, aorta, pancreas, hepatobiliary duct, lymph nodes, as well as retroperitoneal fibrosis) is the reason for the multidisciplinary approach in this disease. In pulmonary involvement, clinical manifestations are nonspecific (cough, dyspnea, chest or back pain, hemoptysis, low-grade fever, weight loss) or patients are asymptomatic and pulmonary changes are an incidental finding on imaging. Up to 60% of cases of autoimmune pancreatitis represent a pancreatic manifestation of IgG4-related disease, with the majority of patients being elderly men. The most common differential diagnosis is a malignant process due to the tumor-like changes of the affected organ. Treatment with corticosteroids, immunosuppressors and symptomatic agents in most cases has a good effect, although relapses of the disease are also observed. We present a clinical case of a 72-year-old patient whose first complaints were nonspecific, for which a chest computed tomography was performed with visualization of four solid nodules in the right lung. After antibiotic therapy, without effect and deterioration of the patient's condition, video-assisted thoracoscopy, biopsy, histology and immunohistochemistry were performed. A diagnosis of IgG4-RD with pleural and lung involvement was confirmed. Anemic syndrome, increased values ​​of amylase, lipase, and acute phase parameters were found from the laboratory tests. Through endoscopic retrograde cholangio-pancreatography, autoimmune pancreatitis was diagnosed in the course of IgG4-RD. A plastic stent was placed on the common bile duct. Treatment with Prednisolone 40 mg/day was started with a subsequent dose reduction. Follow-up computed tomography of the lung after 9 months demonstrated a reduction of the described changes in the lungs, but with persistent lymphadenopathy and fibrotic changes. Azathioprine 100 mg daily was added to prednisone therapy. Against the background of the combined treatment, an improvement of the clinical condition and laboratory parameters was established.

Effectiveness of tofacitinib monotherapy for patients with IgG4-RD or idiopathic retroperitoneal fibrosis.

To explore the effectiveness of tofacitinib for immunoglobulin G4-related disease (IgG4-RD) and idiopathic retroperitoneal fibrosis (IRF), and investigate the expression of JAKs in the lesion of these diseases. Clinical data of patients with IgG4-RD or IRF who were administered with tofacitinib monotherapy were collected. IgG4-RD responder index (IgG4-RD RI) was assessed. The expression of JAK1, JAK2, JAK3, and TYK2 were analysed with immunohistochemistry staining in three salivary glands specimens of IgG4-RD and one retroperitoneal tissue of IRF. Two patients with IRF and two patients with IgG4-RD used tofacitinib monotherapy. Two patients with IRF achieved complete remission with diminished retroperitoneal mass and decreased CRP, as IgG4-RD RI decreased from 6 to 1 in both of them. One with IgG4-RD achieved complete remission with alleviated enlargement of pancreas and IgG4 level decreased from 13.7 g/L to 2.4 g/L, as IgG4-RD RI decreased from 12 to 1. One with IgG4-RD achieved partial response with IgG4 level decreased from 77.1g/L to 25.8g/L as IgG4-RD RI from 18 to 6. JAK1, JAK2, JAK3, and TYK2 expression were detected in biopsy tissues. The staining intensity of the JAK family on the lesion from one IRF patient was similar to those from IgG4-RD patients. Tofacitinib is a potentially effective treatment for IgG4-RD and IRF and it is reasonable to conduct clinical trial to validate its efficacy. The JAKs were expressed in the inflammatory lesions of IgG4-RD and IRF and they may share a common pathogenesis pathway that is independent of IgG4 production.

A case of idiopathic retroperitoneal fibrosis (Ormond’s disease) diagnosed by transduodenal EUS-guided fine-needle aspiration/biopsy

A case of idiopathic retroperitoneal fibrosis (Ormond’s disease) diagnosed by transduodenal EUS-guided fine-needle aspiration/biopsy

Significant association of CX3CR1+CD8 T cells with aging and distinct clinical features in Sjögren's syndrome and IgG4-related disease.

Recent studies have implicated cytotoxic CD4 and CD8 T cells in primary Sjögren's syndrome (pSS) and IgG4-related disease (IgG4-RD), but their association with immune aging and organ-specific clinical features remain unclear. CX3CR1 is expressed on cytotoxic CD4 and CD8 T cells. The aim of this study was to determine associations of peripheral CX3CR1+CD4 and CX3CR1+CD8 T cells with aging and clinical features. Whole blood samples were freshly obtained from consecutive patients with active, treatment-naïve pSS (n=57), IgG4-RD (n=54), and healthy individuals (n=40) and analysed by flow cytometry for CX3CR1+CD4 and CX3CR1+CD8 proportions. Associations of those T cells with aging and clinical features were determined. CX3CR1+CD4 and CX3CR1+CD8 T cells selectively expressed perforin and granzyme B. Proportions of CX3CR1+CD4 and CX3CR1+CD8 T cells were significantly higher in pSS and IgG4-RD than in healthy individuals. Higher proportions of CX3CR1+CD8 T cells were associated with aging in pSS and IgG4-RD but not in healthy individuals. Sex differences were not associated with proportions of CX3CR1+CD8 T cells. Furthermore, patients with pSS with interstitial lung disease showed higher proportions of CX3CR1+CD8 T cells than those without interstitial lung disease. IgG4-RD patients with retroperitoneal fibrosis and/or aortitis exhibited higher proportions of CX3CR1+CD8 T cells compared with those with Mikulicz's disease. Moreover, proportions of CX3CR1+CD8 T cells were decreased following glucocorticoid treatment in paralleled with clinical improvements in IgG4-RD. CX3CR1+CD8 T cells might be involved in immune aging and distinct clinical phenotypes of patients with pSS or IgG4-RD.

Experience of the 193 small intestine reconstructions of ureters

BACKGROUND: The ureteral plastic surgery with their extended constrictions and obliterations remains one of the most difficult problems of reconstructive urology. This is especially true in cases where it is impossible to repair the patency of the ureter due to unchanged urinary tract sections. In such cases, various segments of the gastrointestinal tract and, above all, the ileum proved to be the best plastic material. However, the number of such operations in the world is not enough, which requires further accumulation of experience and study of long-term results of ileoureteroplasty.&#x0D; AIM: The aim of the research is to present the technical features and results of intestinal plastic surgery of ureters.&#x0D; MATERIALS AND METHODS: From 2001 to 2023 ileoureteroplasty was performed in 193 patients of the Clinic of the North-Western State Medical University named after I.I. Mechnikov on the basis of the urological department of City Multidisciplinary Hospital No. 2. The panel consisted of 71 men (36.8%), 122 women (63.2%). The average age was 51 ± 3.2 years. Indications for these operations were extensive defects of the ureters as a result of complications of surgical intervention on the upper urinary tract in 69 (35.8%) of patients, radiation therapy — in 54 (28.0%) and the consequences of iatrogenic damage to the ureters — in 40 (20.7%). Megaureter (6.2%), Ormond’s disease (7.3%) and neoplasms of the ureters (2.0%) were observed less frequently.&#x0D; RESULTS: Unilateral ileoureteroplasty was performed 153 (79.3%), bilateral — 40 (20.7%). The total number of ureteralsurgeries is 235. Since 2013 ileureteroplasty has also been performed with laparoscopic method, and the share of such operations for this period was 35.9%. The early postoperative complications occurred in 18 (9.3%) patients. There were no fatal outcomes. Late complications developed in 16 (11.2%) of 143 examined patients.&#x0D; CONCLUSIONS: This surgical tactics may be recommended in the clinical practice of large urological hospital due to our 22-year experience in using small intestine segments to replace extended ureteral constrictions with a minimum number of postoperative complications, no deaths and good long-term results allows us to recommend.

Retroperitoneal metastasis of breast cancer misdiagnosed as retroperitoneal fibrosis

Retroperitoneal metastasis of breast cancer misdiagnosed as retroperitoneal fibrosis

Retroperitoneal Fibrosis

Retroperitoneal fibrosis (RPF) is a rare condition with an incidence of 1.3 per 100 000 population with a peak age of onset at 40 – 65 years old. The idiopathic form of RPF, also known as Ormond’s disease, is more common and makes up about 75% of the case. Here, we are presenting a case of a woman who was referred to our center due to worsening serum creatinine and was diagnosed with retroperitoneal fibrosis. This case has been taken up due to the rarity of the case and to discuss the radiological appearance specifically the CT appearance of retroperitoneal fibrosis. We have also discussed methods to differentiate between primary and secondary retroperitoneal fibrosis specifically based on CT findings. This is to provide guidance for the diagnosis and prompt management of cases, especially in district hospitals with no advanced diagnostic equipment such as MRI or the ability to biopsy.

Recurrent petit mal seizures in Erdheim-Chester disease mimicking an intra-axial brain tumor: illustrative case.

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis characterized histologically by foamy histiocytes and Touton giant cells in a background of fibrosis. Bone pain with long bone osteosclerosis is highly specific for ECD. Central nervous system involvement is rare, although dural, hypothalamic, cerebellar, brainstem, and sellar region involvement has been described. A 59-year-old man with a history of ureteral obstruction, medically managed petit mal seizures, and a left temporal lesion followed with serial magnetic resonance imaging (MRI) presented with worsening seizure control. Repeat MRI identified bilateral amygdala region lesions. Gradual growth of the left temporal lesion over 1 year with increasing seizure frequency prompted resection. A non-Langerhans cell histiocytosis with a BRAF V600E mutation was identified on pathology. Imaging findings demonstrated retroperitoneal fibrosis and long bone osteosclerosis with increased fluorodeoxyglucose uptake that, together with the neuropathologic findings, were diagnostic of ECD. This case of biopsy-proven ECD is unique in that the singular symptom was seizures well controlled with medical management in the presence of similarly located bilateral anterior mesial temporal lobe lesions. Although ECD is rare intracranially, its variable imaging presentation, including the potential to mimic seizure-associated medial temporal lobe tumors, emphasizes the need for a wide differential diagnosis.

Clinical Characteristics of Retroperitoneal Fibrosis Patients at a Tertiary Hospital in Japan.

Retroperitoneal fibrosis (RPF) is a rare cause of hydronephrosis and progressive renal dysfunction with unidentified origin. RPF is categorized into idiopathic RPF with/without immunoglobulin G4 (IgG4)-related disease (IgG4-RD), and secondary RPF. Identifying the underlying cause is challenging and often associated with delayed diagnosis or therapeutic interventions. We investigated RPF's clinical characteristics based on different etiologies and factors that may help distinguish the underlying causes. We analyzed the cases of 49 patients with RPF that was radiographically diagnosed at our institution (2008-2022). The cohort was 77.6% males; 75.5% had idiopathic RPF and 24.5% had secondary RPF. Among the idiopathic patients, 54.1% had IgG4-RD. The patients were likely to have abdominal pain, lower back pain/lumbago, and constitutional symptoms including generalized fatigue and fever. The idiopathic patients were likely to have higher serum IgG4 and IgG levels and lower serum C3 levels compared to secondary RPF. The IgG4-RPF patients were likely to have higher serum IgG4 levels and lower serum C-reactive protein, ferritin, and C3 levels compared to the idiopathic RPF patients without IgG4-RD. These findings might reflect underlying systemic inflammatory responses. Comprehensive laboratory testing, including serum inflammatory markers and immunological panels, is recommended for radiologically diagnosed RPF patients.