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Obstructive nephropathy (ON) is a common and reversible cause of post-renal acute kidney injury (AKI) and may be caused by a variety of conditions. It occurs when both the upper urinary tracts are obstructed, or when one tract is obstructed in patients with a solitary kidney. ON is suspected whenever there is evidence of hydronephrosis at imaging. However, not all patients with obstruction develop hydronephrosis and significant obstruction can be present in the absence of hydronephrosis. This syndrome is called non-dilated obstructive uropathy (NDOU). It accounts for about 5% of cases of urinary obstruction and the diagnosis can be challenging. The current paper provides an overview of the literature aiming to identify the main causes of NDOU and its clinical presentation, in order to clarify when to suspect it among AKI cases. A narrative review was performed due to the overall low quality of the available evidence. Only patients with post-renal AKI and a non-dilated or minimal dilation of the intrarenal collecting system were included. As evidenced by our review, NDOU is most prevalent in the fifth and sixth decades of life and affects mainly the male gender. On hospital admission serum creatinine levels are usually very high. Among the most common clinical presentations are oliguria/anuria, abdominal pain, signs of retention such as oedema or pleural effusion, and nausea/vomiting. About three out of four cases of NDOU are due to an ab-extrinsic compression of the ureters caused by retroperitoneal fibrosis or malignant disease. An effective and minimally invasive urinary diversion is obtained with ureteric stenting or a percutaneous nephrostomy. A correct diagnosis of NDOU may be challenging but it is of paramount importance as it can lead to a prompt management with a potential complete resolution of both obstruction and acute renal failure. [ABSTRACT FROM AUTHOR]

Background: Enfortumab vedotin (EV) is an antibody-drug conjugate (ADC) that has been approved by the FDA for patients with locally advanced or metastatic urothelial carcinoma (UC). This study presents a comprehensive pharmacovigilance analysis of the post-marketing safety profile of EV in the real-world based on the US Food and Drug Administration Adverse Event Reporting System (FAERS). Methods: Adverse event (AE) reports regarding EV between January 2020 and December 2023 were obtained from the FAERS database. The standardized MedDRA query (SMQ) narrow search AEs on the preferred term (PT) level were used. Disproportionality analysis was performed to identify the AE signals for EV with the reporting odds ratio (ROR), proportional reporting ratio (PRR), multiitem gamma Poisson shrinker (MGPS), and Bayesian confidence propagation neural network (BCPNN). Results: A total of 2,216 reports regarding EV were included in the present study. SMQ analysis results indicated that a stronger strength signal was found in severe cutaneous adverse reactions, retroperitoneal fibrosis, and peripheral neuropathy. A total of 116 significant disproportionality PTs referring to 14 system organ classes (SOCs) were retained by disproportionality analysis, with 49 PTs not listed on the EV drug label. Frequently reported EV-related AEs included rash, peripheral neuropathy, decreased appetite, alopecia, and pruritus. The time to onset of the majority of EV-related AEs was within 30 days (66.05%), with only 0.73% events occurring after 1 year. Conclusion: The disproportionality analysis highlights that dermatologic toxicity and peripheral neuropathy were the major AEs induced by EV. The potential AEs not listed on the drug label were mainly related to gastrointestinal, hepatic, and pulmonary events. Further research is needed to confirm and explore the EVrelated AEs in clinical practice. [ABSTRACT FROM AUTHOR]

Abstract Introduction and aims Retroperitoneal fibrosis (RPF) is a fibroinflammatory disease in which patients may suffer obstructive uropathy (OU). The optimum treatment strategy for RPF with secondary OU is currently unclear, and the aim of this literature review is to assess the methods used to treat this patient cohort. Methods Medline, Embase, Cinahl, the Cochrane Library and PubMed were systematically searched to find studies assessing treatment outcomes in this patient cohort. After reviewing the studies' titles, abstracts and full texts, 12 were found that matched our search aims. Data from these publications were analysed and reported. Results The demographic and symptomatic features of patients across the 12 studies were representative of the general RPF population. No randomised control trials (RCTs) were found, and just one study formally compared outcomes between patients who underwent different treatment strategies. Many of the studies concluded that using medical and surgical methods in combination led to positive outcomes; whereas, others found positive outcomes following a variety of regimens. Many studies also highlighted, however, that significant minorities required further treatment after initial therapy. Conclusions regarding optimum treatment methods were limited as most publications did not formally compare outcomes following different strategies and had an observational study design. Conclusion Although positive outcomes were commonly seen following medical, surgical and a combination of treatments, the literature currently lacks research formally comparing outcomes after assigning specific treatment protocols to groups of RPF patients. More research is therefore required to determine how to best manage RPF leading to secondary OU.

A paraganglioma is a neuroendocrine tumor that may secrete catecholamines and present with symptoms of sympathetic overload such as hypertension and diaphoresis. It is important that paragangliomas are identified, as they must often be treated by surgical excision. IgG4-related retroperitoneal fibrosis (IgG4-RPF) is a systemic inflammatory disease that results in the infiltration of IgG4-positive plasma cells in the retroperitoneum. Such fibrosis may adversely affect nearby organs and tissues. Here, we describe a case of combined paraganglioma and IgG4-RPF in a 47-year-old female patient. This case demonstrates the deleterious effect of these two conditions when they occur simultaneously.

Background: Dogs with retroperitoneal hemangiosarcoma (HSA) exhibit variable postoperative median survival times (MST). Objective: To retrospectively evaluate the prognostic value of selected tumour‐related factors, such as tumour size, rupture, invasion into adjacent tissue, involvement of lymph node and distant metastasis, they were analysed in dogs with retroperitoneal HSA. Methods: Ten dogs with retroperitoneal HSA managed solely with surgical excision were reviewed and compared with spleen (71) and liver (9) HSA. The Kaplan–Meier method and log‐rank analysis were used compare MSTs between factors. Multivariable Cox proportional‐hazard analysis was used to compare differences between arising sites. Results: Retroperitoneal HSA showed comparatively longer postoperative MST compared with that of spleen and liver HSA and demonstrated significantly longer MST (p = 0.003) for tumours ≥5 cm (195 days) than <5 cm (70 days). Spleen HSA revealed significantly shorter MSTs in involvement of distant lymph nodes (23 days) and distant metastasis (39 days) than those in negative (83 days, p = 0.002 and 110 days, p < 0.001, respectively). Liver HSA also revealed significantly shorter MST (16.5 days compared with 98 days, p = 0.003) for distant metastasis. Additionally, hazard ratios (HRs) and their forest plot for overall HSA revealed as poor prognostic factors, arising sites (spleen; HR 2.78, p = 0.016 and liver; HR 3.62, p = 0.019), involvement of distant lymph nodes (HR 2.43, p = 0.014), and distant metastasis (HR 2.86, p < 0.001), and as better prognostic factor of tumour size ≥5 cm (HR 0.53, p = 0.037). Conclusion: In combination with overall HSA, retroperitoneal HSA shows comparatively longer postoperative MST compared to spleen and liver HSA, associated with tumour size ≥5 cm suggesting better prognostic factor. [ABSTRACT FROM AUTHOR]

Introduction IgG4-related disease (IgG4-RD) is a progressive and potentially life-threatening condition characterized by immune system activation and tissue fibrosis, affecting various organs such as the pancreas, kidneys, and lungs. Initially recognized in 2003, it often presents as mass-like lesions, which can mimic tumors. Despite advancements in understanding its pathology, epidemiological data are limited, and many patients remain undiagnosed due to unfamiliarity with the disease. Aim of the study The aim of this article wast to summarize the latest knowledge on the diagnosis and clinical manifestations of IgG4-related disease. Materiał and methods This review is based on articles from the PubMed and Google Scholar databases, covering the years 2007-2024, using the keywords: IgG4-related disease; autoimmune pancreatitis; retroperitoneal fibrosis; classification criteria. Results The disease's complex pathogenesis involves B and T cell activity, with genetic and environmental factors contributing. While effective treatments, like B cell depletion, exist, the disease's broad clinical manifestations and multi-organ involvement require a multidisciplinary approach for proper diagnosis and management. Conclusion Diagnosing IgG4-RD is challenging due to its varied symptoms, often mimicking other diseases. Key findings include high IgG4 levels and specific histopathological features. Further research is needed to understand its genetic factors, pathogenesis, and epidemiology.

Median arcuate ligament syndrome (MALS) involves coeliac artery compression, causing a range of symptoms from chronic pain to life-threatening complications. This case features a 52-year-old patient with recurrent retroperitoneal bleeding from MALS-related inferior pancreaticoduodenal artery aneurysms (PDAAs). Emergency interventions, including surgical bleeding control, angioplasty, percutaneous drainage, and median arcuate ligament release, were conducted. The case highlights challenges in diagnosing and managing MALS-related PDAA, emphasizing the importance of early identification and tailored interventions based on clinical symptoms and imaging. Surgical intervention to release the ligament is the primary treatment, with considerations for prophylactic intervention in PDAA cases. Lack of established PDAA management protocols underscores the need for prompt intervention to prevent complications. In conclusion, this report stresses the association between MALS and PDAA, advocating for early identification and tailored management to mitigate complications. [ABSTRACT FROM AUTHOR]

Introduction: Minimal access surgery, encompassing laparoscopy and robotic procedures, has become the preferred choice for a wide range of surgical interventions, receiving widespread acceptance from both surgeons and patients. However, as the utilization of these minimally invasive techniques continues to grow, surgeons are increasingly confronted with unforeseen and puzzling complications. One such rare and perplexing complication is the emergence of ascites following laparoscopic procedures, particularly when no accidental injury to the bowel or urinary system has occurred, posing a challenging and distressing scenario for the operating surgeon. Aims and Objective: We have encountered a case of idiopathic ascites following a laparoscopic appendicectomy in a 10-year-old boy. The primary objective of this study is to conduct a comprehensive literature review to identify similar case reports and publications. By doing so, we aim to enhance our understanding of the causes and principles for managing idiopathic ascites occurring after laparoscopic surgery. Methods: A 10-year-old boy was admitted to the hospital with typical symptoms of acute appendicitis. Following evaluation, a nearly bloodless and uneventful laparoscopic appendicectomy was performed. However, postoperatively, he developed ascites accompanied by scrotal swelling due to scrotal edema, and despite thorough evaluation, no identifiable cause was found. The patient was managed conservatively and fully recovered without any subsequent complications. Results: The occurrence of ascites stemming from an idiopathic allergic or inflammatory peritoneal reaction during a laparoscopic procedure is exceptionally rare. During the patient's evaluation, the paramount concern was to rule out potentially significant complications such as bowel or urinary tract injuries, which can commonly result in postoperative peritoneal fluid accumulation. After the procedure, the boy underwent a thorough evaluation, but no definitive cause for the ascites was identified, further highlighting the enigmatic nature of this condition. Conclusion: Postoperative ascites without a discernible cause following laparoscopic surgery is an unexpected complication. In such cases, the leading hypothesis points to a peritoneal inflammatory reaction induced by agents utilized during laparoscopy. [ABSTRACT FROM AUTHOR]

Idiopathic retroperitoneal fibrosis, also known as Ormond's disease, can present as an IgG4-related disease involving the deposition of fibrous tissue in the retroperitoneal space. It is caused by chronic inflammation developing around the abdominal aorta and common iliac arteries. The case report describes a patient hospitalised for complaints of low back pain with positive Goldflam's sign and hydronephrosis, whose abdominal CT scan showed a tumour in the retroperitoneal space pressing on the ureter. Based on histopathological examination and laboratory tests, the patient was diagnosed with idiopathic retroperitoneal fibrosis, most likely related to IgG4. After diagnosis, the patient was initially treated in the urology department for urinary retention and then received treatment with glucocorticosteroids, according to the treatment guidelines for IgG4-dependent disease. [ABSTRACT FROM AUTHOR]

Background: Postoperative lymphatic leakage is a complication of ineffective conservative treatment for retroperitoneal mass. Herein, we report a case of lymphatic leakage that arose after retroperitoneal tumor resection and that was treated with retrograde transvenous thoracic duct embolization. Case presentation: A 28-year-old man with persistent abdominal pain was diagnosed with a large retroperitoneal metastatic tumor measuring 10 cm and a subdiaphragmatic lymph node originating from a testicular tumor. After high orchidectomy and neoadjuvant chemotherapy, the subdiaphragmatic lymph node and retroperitoneal tumor were resected together with the abdominal aorta; the latter was reconstructed using a prosthetic graft. Postoperatively, the patient developed chylothorax. No improvement was observed after conservative treatment that included fasting and somatostatin therapy. The leakage site could not be identified using antegrade lymphangiography of the bilateral inguinal lymph nodes, but was detected using retrograde transvenous lymphangiography. The leakage site was successfully embolized. Conclusion: This case report describes successful treatment with retrograde transvenous thoracic duct embolization for chylothorax following resection of a retroperitoneal tumor and lymph node. This approach is a less invasive and more effective mode of treatment for chylothorax and should be considered before surgical thoracic duct ligation when the leakage point cannot be identified using the antegrade approach. [ABSTRACT FROM AUTHOR]

Background: Peritoneal fibrosis is the prevailing complication induced by prolonged exposure to high glucose in patients undergoing peritoneal dialysis. Methods: To elucidate the molecular mechanisms underlying this process, we conducted an integrated analysis of the transcriptome and chromatin accessibility profiles of human peritoneal mesothelial cells (HMrSV5) during high-glucose treatment. Results: Our study identified 2775 differentially expressed genes (DEGs) related to high glucose-triggered pathological changes, including 1164 upregulated and 1611 downregulated genes. Genome-wide DEGs and network analysis revealed enrichment in the epithelial–mesenchymal transition (EMT), inflammatory response, hypoxia, and TGF-beta pathways. The enriched genes included VEGFA, HIF-1α, TGF-β1, EGF, TWIST2, and SNAI2. Using ATAC-seq, we identified 942 hyper (higher ATAC-seq signal in high glucose-treated HMrSV5 cells than in control cells) and 714 hypo (lower ATAC-seq signal in high glucose-treated HMrSV5 cells versus control cells) peaks with differential accessibility in high glucose-treated HMrSV5 cells versus controls. These differentially accessible regions were positively correlated (R = 0.934) with the nearest DEGs. These genes were associated with 566 up- and 398 downregulated genes, including SNAI2, TGF-β1, HIF-1α, FGF2, VEGFA, and VEGFC, which are involved in critical pathways identified by transcriptome analysis. Integrated ATAC-seq and RNA-seq analysis also revealed key transcription factors (TFs), such as HIF-1α, ARNTL, ELF1, SMAD3 and XBP1. Importantly, we demonstrated that HIF-1α is involved in the regulation of several key genes associated with EMT and the TGF-beta pathway. Notably, we predicted and experimentally validated that HIF-1α can exacerbate the expression of TGF-β1 in a high glucose-dependent manner, revealing a novel role of HIF-1α in high glucose-induced pathological changes in human peritoneal mesothelial cells (HPMCs). Conclusions: In summary, our study provides a comprehensive view of the role of transcriptome deregulation and chromosome accessibility alterations in high glucose-induced pathological fibrotic changes in HPMCs. This analysis identified hub genes, signaling pathways, and key transcription factors involved in peritoneal fibrosis and highlighted the novel glucose-dependent regulation of TGF-β1 by HIF-1α. This integrated approach has offered a deeper understanding of the pathogenesis of peritoneal fibrosis and has indicated potential therapeutic targets for intervention. [ABSTRACT FROM AUTHOR]

Reported here are two Asian patients with Takayasu arteritis (TA) with contrasting presentations and responses to treatment. The first patient was a 53-year-old man who presented with disabling abdominal pain. Imaging revealed thickening of the coeliac axis extending to common hepatic and splenic arteries causing external compression with luminal narrowing. There was uniform thickening of the aortic arch and the wall of the descending aorta along with retroperitoneal fibrosis. Early treatment resulted in almost complete remission in eight weeks. The second patient was a 48-year-old woman with pain down the left upper limb with eventual ischaemia of the fourth finger. Imaging revealed circumferential wall thickening at the origin of the left subclavian artery. Treatment started after 3 weeks of the initial presentation, and took up to 14 months for clinical improvement. The relative rarity of this disease and the heterogeneous nature of its clinical manifestations predispose to late diagnosis and delayed treatment. Clinical suspicion and relevant imaging are crucial for the early and accurate diagnosis and management of patients with TA.

Abstract Background Retroperitoneal 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 presentation A 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. Conclusions With 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.

We present the case of periaortitis which presented initially with left flank pain. A diagnosis of IgG4-related disease (IgG4-RD) was subsequently made and managed as such. IgG4-RD is rare, can be difficult to diagnose, and requires clinical, serological, radiological and pathological correlation, particularly given that serum IgG4 levels may be normal. Immunosuppression is the mainstay treatment for this chronic condition alongside regular rheumatology input.

Abstract IgG4‐related diseases are adverse events that occur after receiving treatment with immune checkpoint inhibitors (ICI). This study reports the first case of IgG4‐related retroperitoneal fibrosis after the administration of chemotherapy with nivolumab and ipilimumab (NI therapy). An 80‐year‐old man developed lower abdominal pain eight months after NI therapy was initiated. Although the primary lesion maintained its reduced size on computed tomography, there was an increase in the soft tissue shadows intensity around the abdominal aorta, bladder, and seminal vesicles, suggesting retroperitoneal fibrosis. Blood tests showed elevated IgG4 levels. Computed tomography‐guided biopsy of the retroperitoneum showed B cell‐dominant lymphocyte infiltration consistent with IgG4‐related retroperitoneal fibrosis and characteristic CD8‐positive lymphocyte infiltration, suggestive of the involvement of cytotoxic T cells. Based on the clinical, imaging, and pathological findings, the patient was diagnosed with IgG4‐related retroperitoneal fibrosis due to ICI. Immunotherapy discontinuation alone did not result in improvement; therefore, steroid therapy was initiated. In clinical practice, IgG4‐related retroperitoneal fibrosis can occur as an immune‐related adverse event when administering anti‐PD‐1 and anti‐CTLA‐4 antibodies for cancer immunotherapy. Early steroid therapy could be effective in controlling this immune‐related adverse event.

IgG4-related disease is a rare cause of retroperitoneal fibrosis with mass formation. A 46-year-old female presented with lower back pain. She had an on-and-off mild fever and constitutional symptoms with evidence of tenderness over the lower back. Investigations revealed high inflammatory markers. Magnetic resonance imaging revealed an ill-defined signal area in the left paraspinal area. Histology revealed fibromuscular tissue with scattered plasma cells showing positivity for immunoglobulin-G-4 (IgG4). The immunomorphological features are suggestive of IgG4-related-retroperitoneal fibrosis. She was commenced on glucocorticoids. It is imperative to consider unusual diagnoses such as IgG4-related disease in a patient with retroperitoneal disease with evidence of mass formation.

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.

Long-term peritoneal dialysis (PD) causes structural and functional alterations of the peritoneal membrane. Peritoneal deterioration and fibrosis are multicellular and multimolecular processes. Under stimulation by deleterious factors such as non-biocompatibility of PD solution, various cells in the abdominal cavity show differing characteristics, such as the secretion of different cytokines, varying protein expression levels, and transdifferentiation into other cells. In this review, we discuss the role of various cells in the abdominal cavity and their interactions in the pathogenesis of PD. An in-depth understanding of intercellular communication and inter-organ communication in PD will lead to a better understanding of the pathogenesis of this disease, enabling the development of novel therapeutic targets. [ABSTRACT FROM AUTHOR]

IgG4‐related disease is an inflammatory, multisystemic disease that affects the immune system. The disease progresses to fibrosis due to inflammation. Retroperitoneal fibrosis is a serious complication. Pancreas, biliary tract, glands, thyroid, lymph nodes, etc. may be involved. Prognosis is usually subacute, and seen in middle age and advanced men. It is characterized histopathologically by IgG4 positive plasma cells, lymphoplasmocytic cell infiltration, and storiform fibrosis. In our case, we evaluated a patient who referred to our clinic from an external center with the complaints of generalized pain, itching, tearing and redness in eyes, involvement of bilateral large joints, and impaired renal function. Diagnosis, treatment and management of the disease are important. Response to glucocorticoid therapy is good. [ABSTRACT FROM AUTHOR]

IgG4‐related diseases are adverse events that occur after receiving treatment with immune checkpoint inhibitors (ICI). This study reports the first case of IgG4‐related retroperitoneal fibrosis after the administration of chemotherapy with nivolumab and ipilimumab (NI therapy). An 80‐year‐old man developed lower abdominal pain eight months after NI therapy was initiated. Although the primary lesion maintained its reduced size on computed tomography, there was an increase in the soft tissue shadows intensity around the abdominal aorta, bladder, and seminal vesicles, suggesting retroperitoneal fibrosis. Blood tests showed elevated IgG4 levels. Computed tomography‐guided biopsy of the retroperitoneum showed B cell‐dominant lymphocyte infiltration consistent with IgG4‐related retroperitoneal fibrosis and characteristic CD8‐positive lymphocyte infiltration, suggestive of the involvement of cytotoxic T cells. Based on the clinical, imaging, and pathological findings, the patient was diagnosed with IgG4‐related retroperitoneal fibrosis due to ICI. Immunotherapy discontinuation alone did not result in improvement; therefore, steroid therapy was initiated. In clinical practice, IgG4‐related retroperitoneal fibrosis can occur as an immune‐related adverse event when administering anti‐PD‐1 and anti‐CTLA‐4 antibodies for cancer immunotherapy. Early steroid therapy could be effective in controlling this immune‐related adverse event. [ABSTRACT FROM AUTHOR]

Immunoglobulin G4 (IgG4)-related disease has the potential to impact any part of the body, including the walls of large- and medium-sized blood vessels and the ureters. While histopathologic examination is currently the standard method for identifying organ involvement and diagnosing IgG4-related disease (IgG4-RD), obtaining biopsy or surgical samples from vessel or ureteral walls is challenging. Given that patients may display only mild symptoms, non-invasive imaging plays a vital role in both diagnosing and managing IgG4-related diseases. Multidetector CT scans are valuable in establishing the primary diagnosis, identifying anatomical landmarks and assessing their relationships. Involvement of the genitourinary organs, such as the ureter, bladder, urethra, and male and female reproductive organs in IgG4-RD, is infrequent when compared to kidney involvement. The imaging findings may include the presence of a localised mass within or surrounding the affected organ or a generalised enlargement of the organ. This report includes cross-sectional images of five cases of IgG4-RD involving large- and medium-sized blood vessels (the aorta and superior mesenteric artery) and the ureters. Contribution: This case series provides insight into the various imaging appearances of IgG4-related retroperitoneal organ involvement and helps differentiate it radiologically from retroperitoneal fibrosis.

Essential thrombocythemia (ET) is associated with an increased risk of thrombosis and autoimmune renal involvement. We report an extremely rare case of an acute kidney injury (AKI) in the presence of bilateral renal pelvises fibrosis in a patient with a proven diagnosis of ET. A 48-year-old male patient with a past medical history of mild chronic kidney disease and ET was admitted to our hospital with AKI. The patient discontinued his hydroxyurea treatment for the past 2 months and laboratory data showed increasing serum creatinine levels and platelet counts with increased renal sizes, severe hydrocalyx, and bilateral renal sinuses’ fibrosis in imaging. The patient started again on hydroxyurea therapy and showed improvement in all laboratory scales. ET and increased levels of platelet-derived growth factors could cause renal sinuses fibrosis and glomerulopathy. In ET patients with renal sinuses’ fibrosis and glomerulopathy, initiating cytoreductive therapy could improve the outcome.

We report the case of a 24-year-old male presenting with obstructive renal failure, characterised by imaging evidence of a cystic lesion contingent upon the seminal vesicle and concurrent renal agenesis. Initial management involved urinary diversion, followed by outpatient monitoring and subsequent recurrence. Subsequent diagnostic assessments led to the identification of Zinner's syndrome, accompanied by retroperitoneal fibrosis. We present the clinical course, diagnostic methodology and the efficacious implementation of medical-surgical therapeutic interventions, yielding favourable outcomes.

Immunoglobulin G4 (IgG4)‐related disease (IgG4‐RD) is a multi‐organ immune‐mediated fibroinflammatory disorder that may imitate malignancy, infectious or any other inflammatory disorder. IgG4‐related retroperitoneal fibrosis (IgG4‐RPF) is a rare form of IgG4‐RD, diagnosis of which is often relied on radiological technology. Herein, we describe a case of 60 year old male, presenting with low back pain and weight loss for a period of 2 months and 15 days. Imaging studies showed a retroperitoneal tumorous mass along with bilateral hydroureteronephrosis, which was later confirmed to be IgG4‐related retroperitoneal fibrosis on the basis of extensive histopathological analysis. Immunosuppressive therapy resulted in a decrease in fibrosis and restoration of renal function. [ABSTRACT FROM AUTHOR]

Erdheim-Chester disease is an uncommon non-Langerhans cell histiocytosis affecting multiple systems. There is limited knowledge on the imaging capabilities of this disease. We present an extremely rare case of Erdheim-Chester illness in a 67-year-old man with multisystem involvement, including the cardiovascular system, skeleton, retroperitoneum (renal and adrenal infiltration) and the neurologic system. The involvement of the various organs was thoroughly assessed using multimodal imaging modalities such as computed tomography, magnetic resonance imaging, positron emission tomography and bone scintigraphy. Erdheim-Chester illness was revealed by a bone biopsy. Especially when there is cardiac and cerebral involvement, Erdheim-Chester illness is a rare condition with a poor prognosis. Knowing the imaging characteristics of Erdheim-Chester disease may be helpful in understanding the radiological results of many organs affected by the disease as described and discussed in the current case report.

Abstract Immunoglobulin G4 (IgG4)‐related disease (IgG4‐RD) is a multi‐organ immune‐mediated fibroinflammatory disorder that may imitate malignancy, infectious or any other inflammatory disorder. IgG4‐related retroperitoneal fibrosis (IgG4‐RPF) is a rare form of IgG4‐RD, diagnosis of which is often relied on radiological technology. Herein, we describe a case of 60 year old male, presenting with low back pain and weight loss for a period of 2 months and 15 days. Imaging studies showed a retroperitoneal tumorous mass along with bilateral hydroureteronephrosis, which was later confirmed to be IgG4‐related retroperitoneal fibrosis on the basis of extensive histopathological analysis. Immunosuppressive therapy resulted in a decrease in fibrosis and restoration of renal function.

A potential complication of pharmacotherapy for a given patient is the possibility of various side effects of drugs, which are manifested in many ways and constitute iatrogenic causes of diseases. Among the systemic side effects of drugs, there are also those involving the urinary tract, although these are less reported in the literature. The use of numerous drugs—especially of anticholinergics or drugs with anticholinergic potential, opioid analgesics, non-steroidal anti-inflammatory drugs, antidepressants, first-generation antipsychotics (classic neuroleptics) and selected cardiovascular drugs (beta-blockers, thiazides potassium-sparing diuretics, statins), as well as others—may increase the risk of developing urological disorders, such as urinary retention or incontinence, urinary tract infections, urolithiasis, erectile dysfunction in men and retroperitoneal fibrosis. The purpose of this paper is to characterise the abovementioned drug-induced disorders of the lower urinary tract on the basis of a non-systematic literature review. [ABSTRACT FROM AUTHOR]

Immunoglobulin G4-related disease (IgG4-RD) is an autoimmune fibrosclerotic inflammatory condition with distinctive histopathological findings affecting various organ systems. The disease can be tumefactive or “mass-like” in morphology, or less commonly, infiltrative. Many historic and well-known disease entities are manifestations of IgG4-RD in different organs. Radiologists need to be aware of this multifaceted disorder and its systemic nature. We searched Pubmed and Embase using the keywords “head and neck,” “IgG4-related disease,” and “imaging.” We included the relevant review articles published in the English language from 1997 to 2021, whose major area of discussion was IgG4-RD manifestations in the head-and-neck. Filters applied for inclusion were: “full text,” “humans,” “English,” “cancer,” article type: “review,” “meta-analysis,” “systematic review,” and “guideline.” In this review article, we have discussed the wide spectrum of manifestations of this disease at different head-and-neck subsites to familiarize radiologists with this interesting disease entity.

Introduction Retroperitoneal fibrosis (RPF) is a rare disease associated with the formation of hard inflammatory and fibrous tissue in the retroperitoneum. Taking into consideration the fact that RPF is a rare disease with different subtypes, we compared the basal clinical and biochemical characteristics of the vascular and urorenal subtypes.Patients and methods From January 2005 until December 2021, 27 patients were identified as vascular subtype (18 males) and 11 as urorenal subtype (9 males).Results Patients with a primary urorenal origin had significantly worse kidney function as reflected by serum creatinine and eGFR (both p

Abstract Background Retroperitoneal fibrosis is a rare disease characterized by chronic nonspecific inflammation, which leads to clinical compression manifestations of retroperitoneal organs especially ureter. Approximately 70 percent of retroperitoneal fibrosis cases are idiopathic which has no clear etiology. This study reported a rare case of a 48-year-old woman presented with idiopathic retroperitoneal fibrosis and endometrial cancer. Case presentation A 48-year-old woman presented with irregular vaginal bleeding without abdominal pain, bloating or discomfort. The patient was diagnosed iRPF after splenectomy 13 years ago. Then she took prednisone for 2 years and took tamoxifen for about 11 years. She stopped taking the medication from October 2019 to May 2020 and then started taking tamoxifen again until November 2020. Two weeks after she stopped taking tamoxifen, she presented with irregular vaginal bleeding. Gynecological ultrasound revealed a thick endometrium with uneven echo enhancement and blood flow signals. Then diagnostic curettage was performed with pathological examination showed endometroid carcinoma. Later, the patient was admitted to Peking University Third Hospital for surgery. Preoperative imaging examinations, including CT, MRI, and PET/CT, all showed pelvic enlarged lymph nodes and they were highly suspected to have lymph node metastasis. The patient underwent laparoscopic surgical staging and enlarged lymph nodes in the pelvic and aortic regions were removed. Finally, the pathology confirmed that endometrioid adenocarcinoma and fibrosis, but there was no tumor infiltration in these enlarged lymph nodes. The patient is now in good condition. Conclusion This case report stressed the difficulty to distinguish between lymph node metastasis and inflammatory hyperplasia by common imaging methods. Due to increased surgical difficulty among retroperitoneal patients, lymphadenectomy should be carefully evaluated to avoid additional surgical complications and over-treatment.

Inflammatory abdominal aortic aneurysms (iAAA) are a form of noninfectious aortitis in patients with abdominal aortic aneurysms (AAA). Ultrasound could help to detect iAAA early. This retrospective observational study assessed the potential of using ultrasound to detect iAAA in a case series of iAAA patients, and the diagnostic value of ultrasound to detect iAAA in consecutive patients in a follow-up for AAA, referred to as a feasibility study. In both studies, diagnosis of iAAA was based on a cuff surrounding the aneurysm using CT (golden standard). The case series included 13 patients (age 64 (61; 72) years; 100% male). The feasibility study included 157 patients (age 75 (67; 80) years; 84% male). In the case series, all iAAA patients showed a cuff surrounding the aortic wall on ultrasound. In the feasibility study with AAA patients, ultrasound yielded no cuff in 147 (93.6%; CT negative in all cases), a typic cuff in 8 (5.1%; CT positive in all cases), and an inconclusive cuff in 2 (1.3%; CT negative in both cases) patients. Sensitivity and specificity were 100% and 98.7%, respectively. This study indicates that iAAA can be identified with ultrasound, and safely ruled out. In positive ultrasound cases, additional CT imaging might still be warranted. [ABSTRACT FROM AUTHOR]

Spontaneous perforation of a gastric ulcer is a rare entity and can often be overlooked considering the frequently silent clinical picture. The posterior erosion of the ulcer through the omental bursa in the retroperitoneal space determines local inflammation which, together with the fibrosis of the retroperitoneal tissue facilitates the enclosure of the gastric content. We present the case of a 49-year old patient investigated for pain in the upper abdomen. The endoscopy performed one month before the admission described a retractile area with a central ulcer on the posterior surface of the stomach, adjacent to the lesser curvature. Given the fact that the abdominal x-ray was normal, a CT scan was performed and a voluminous retroperitoneal cystic lesion was discovered. Combining all the preoperative information the diagnosis was of retroperitoneal abscess by posterior perforation of a gastric ulcer. Surgical intervention was performed, the abscess was evacuated and its wall was completely resected; because of local conditions a distal hemigastrectomy with Roux en Y gastro-enteroanastomosis was chosen over gastrorhaphy and omentoplasty. [ABSTRACT FROM AUTHOR]

Background: Retroperitoneal fibrosis (RPF) can occur due to many etiologies and is categorized into idiopathic and secondary. Etiologies of secondary RPF include medications, autoimmune disease, malignancy, and IgG4-related disease (IgG4-RD). Although IgG4-RD usually involves multiple systems synchronically including the pancreas, aorta, and kidneys, it can present with isolated RPF without involvement of other organ systems. Caution must be exercised in these instances as the diagnosis should be confirmed based on specific clinical, radiographic, and histopathologic criteria. Such confirmation can affect the work-up and therapeutic approach as treatment with corticosteroids can lead to remission, both clinically and radiographically. [ABSTRACT FROM AUTHOR]

Epithelioid hemangioendotheliomas (EHEs), low-grade malignant tumors of vascular endothelial cell origin, are characterized by vascular endothelial proliferation. In 2002, the World Health Organization classified EHEs as locally aggressive tumors with the potential to metastasize. Currently, the diagnosis of EHE is based on pathology, histological and immunohistochemical examinations. There are no standard treatment guidelines. We here report a 69-year-old man who presented with left-sided chest and abdominal pain for more than 2 months. Enhanced computed tomography of the thorax and abdomen in another hospital suggested a mass in the left adrenal region that was considered malignant. Positron emission tomography-computed tomography in our hospital suggested a large multi-loculated, hypermetabolic, cystic mass in the left adrenal region that was considered malignant. Accordingly, a puncture biopsy of the mass was performed and the diagnosis of EHE confirmed by pathological examination, including immunohistochemical staining. This patient was treated with the programmed death 1 (PD-1) immune checkpoint inhibitor toripalimab with long-term success. The best response was stable disease (SD) with a progression-free survival (PFS) of more than 13 months. The patient is still alive now. Because the sample size of previous studies was small, further studies are needed to determine the safety and efficacy of toripalimab in the treatment of EHE. [ABSTRACT FROM AUTHOR]

Chyloperitoneum (chylous ascites) is a rare complication of peritoneal dialysis (PD). Its causes may be traumatic and nontraumatic, associated with neoplastic disease, autoimmune disease, retroperitoneal fibrosis, or rarely calcium antagonist use. We describe six cases of chyloperitoneum occurring in patients on PD as a sequel to calcium channel blocker use. The dialysis modality was automated PD (two patients) and continuous ambulatory PD (the rest of the patients). The duration of PD ranged from a few days to 8 years. All patients had a cloudy peritoneal dialysate, characterized by a negative leukocyte count and sterile culture tests for common germs and fungi. Except for in one case, the cloudy peritoneal dialysate appeared shortly after the initiation of calcium channel blockers (manidipine, n = 2; lercanidipine, n = 4), and cleared up within 24–72 h after withdrawal of the drug. In one case in which treatment with manidipine was resumed, peritoneal dialysate clouding reappeared. Though turbidity of PD effluent is due in most cases to infectious peritonitis, there are other differential causes including chyloperitoneum. Although uncommon, chyloperitoneum in these patients may be secondary to the use of calcium channel blockers. Being aware of this association can lead to prompt resolution by suspension of the potentially offending drug, avoiding stressful situations for the patient such as hospitalization and invasive diagnostic procedures. [ABSTRACT FROM AUTHOR]

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Retroperitoneal fibrosis (RPF) is an uncommon fibrotic disorder that can cause pain, ureteral obstruction, deep venous thrombosis, hydrocele, and, rarely, aortic occlusion. Herein is described a 65-year-old man with aortic occlusion from idiopathic RPF who was treated with axillobifemoral bypass grafting, which failed in the intermediate term. On representation with critical claudication, he underwent thoracobifemoral bypass grafting via a lateral retroperitoneal tunnel created through a midline, infraumbilical counterincision. He was discharged home on postoperative day 5. This illustrates the successful use of thoracic aortic inflow to treat the aortoiliac occlusive complication of RPF.

Immunoglobulin (Ig)G4-related disease (IgG4-RD) with retroperitoneal fibrosis (RPF) is a rare, fibroinflammatory disease involving the soft tissues of the retroperitoneum. A 73-year-old man with IgG4-related RPF affecting the abdominal aorta and iliac arteries was treated with steroids and mycophenolate mofetil. The prevalence of the disease remains unknown because it is often misdiagnosed and can mimic many malignant, infectious, and inflammatory conditions. Autoimmune pancreatitis is a common presenting condition of IgG4-RD. Because As IgG4-RD is responsive to steroids, diagnosing IgG4-related RPF early can prevent the exposure of patients with RPF to unnecessary diagnostic and therapeutic interventions.

The dried flower bud of Syzygium aromaticum L. (S. aromaticum) (Myrtaceae), cloves, have been used for their analgesic and anti‐inflammatory activities. Peritoneal adhesion (PA) is the most common complication of abdominal and pelvic surgeries, which causes significant adverse effects and severe economic burden. The present study aimed to evaluate the preventive effect of S. extract (SAE) on PA formation in a rat model. Male Wistar 8‐week‐old rats were randomly divided into sham, control (received vehicle), and treatment (0.25%, 0.5%, and 1% w/v of SAE) groups. The adhesion and related factors were examined using the Nair scoring system and immunological and biochemical kits for the levels of inflammatory cytokines [interleukin (IL)‐6 and tumor necrosis factor (TNF)‐α], growth factors [transforming growth factor (TGF)‐β1 and vascular endothelial growth factor (VEGF)], oxidative [nitric oxide (NO) and malondialdehyde (MDA)], and anti‐oxidative [glutathione (GSH)] factors. Our results figured out that the adhesion score and IL‐6, TNF‐α, TGF‐β1, VEGF, NO, and MDA levels were significantly increased, but the GSH level was decreased in the control group compared to the sham group (p < 0.001–0.05). On the other hand, the 0.25% SAE group had a lower adhesion score, and IL‐6, TNF‐α, TGF‐β1, VEGF, NO, and MDA levels were significantly decreased compared with the vehicle group, and the level of GSH was increased (p < 0.001–0.05). SAE could efficiently reduce adhesion score and regulate inflammatory cytokines, oxidative and anti‐oxidative factors, and biomarkers of fibrosis and angiogenesis. Therefore, clove extract can be considered a potential candidate for PA management. [ABSTRACT FROM AUTHOR]

New Findings: What is the central question of this study?Peritoneal injury can result in a persistent fibroproliferative process in the abdominal cavity, causing pain and loss of function of internal organs. This study aimed to demonstrate the use of sodium butyrate (NaBu) as a potential agent to attenuate peritoneal fibrosis induced by a synthetic matrix.What is the main finding and its importance?Our findings provide the first evidence that NaBu attenuates the inflammatory, angiogenesis and fibrogenesis axes involved in the formation of peritoneal fibrovascular tissue, indicating the potential of this compound to ameliorate peritoneal fibrosis. The aim of this study was to identify the bio‐efficacy of sodium butyrate (NaBu) on preventing the development of peritoneal fibrovascular tissue induced by implantation of a synthetic matrix in the abdominal cavity. Polyether–polyurethane sponge discs were implanted in the peritoneal cavity of mice, which were treated daily with oral administration of NaBu (100 mg/kg). Control animals received water (100 μl). After 7 days, the implants were removed for assessment of inflammatory, angiogenic and fibrogenic markers. Compared with control values, NaBu treatment decreased mast cell recruitment/activation, inflammatory enzyme activities, levels of pro‐inflammatory cytokines, and the proteins p65 and p50 of the nuclear factor‐κB pathway. Angiogenesis, as determined by haemoglobin content, vascular endothelial growth factor levels and the number of blood vessels in the implant, was reduced by the treatment. In NaBu‐treated animals, the predominant collagen present in the abdominal fibrovascular tissue was thin collagen, whereas in control implants it was thick collagen. Transforming growth factor‐β1 levels were also lower in implants of treated animals. Sodium butyrate downregulated the inflammatory, angiogenesis and fibrogenesis axes of the fibroproliferative tissue induced by the intraperitoneal synthetic matrix. This compound has potential to control/regulate chronic inflammation and adverse healing processes in the abdominal cavity. [ABSTRACT FROM AUTHOR]