Dis Mon
January 2025
Department of Pharmaceutical Sciences, Babasaheb Bhimrao Ambedkar University, Vidya Vihar, Raebareli Road, Lucknow-226025, (U.P.), India. Electronic address:
Erdheim-Chester disease (ECD) is an extremely rare non-Langerhans cell disorder that is believed to include both inflammatory and neoplastic characteristics. It is caused due to genetic mutations in proto-oncogenes like BRAF and MEK, while immunological pathways have an essential role in the onset and progression of the disease. Despite its rarity, ECD poses significant diagnostic and therapeutic challenges due to its heterogeneous clinical presentation and limited understanding of its underlying pathophysiology.
View Article and Find Full Text PDFClin Rheumatol
January 2025
Department of Rheumatology and Immunology, The First Affiliated Hospital of Naval Medical University, Shanghai, 200433, China.
Objective: Retroperitoneal fibrosis (RPF) is a rare condition marked by inflammation and fibrosis affecting the peritoneal and retroperitoneal soft tissues. In recent years, the identification of IgG4-related diseases has brought to light a significant association with fibrous disorders, including RPF, which were once considered independent. In this comprehensive cohort study, we performed a comparative analysis of the demographic, clinical, laboratory, histopathological, and therapeutic characteristics between patients with IgG4-related RPF and those with idiopathic retroperitoneal fibrosis (iRPF).
View Article and Find Full Text PDFCureus
November 2024
Department of Internal Medicine/Rheumatology, Texas Tech University Health Sciences Center Paul L. Foster School of Medicine, El Paso, USA.
Retroperitoneal fibrosis (RPF) is a rare disease with a nonspecific presentation. RPF can be classified into Idiopathic, the most common, or secondary due to malignancy and various medications resulting in chronic inflammation and fibrosis in the retroperitoneum. The complications arise due to the compression of structures in the retroperitoneum.
View Article and Find Full Text PDFFront Med (Lausanne)
December 2024
Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing, China.
Objective: Retroperitoneal fibrosis (RPF) is a rare disease characterized by the presence of fibroinflammatory tissue that surrounds the abdominal aorta and the iliac arteries and often entraps the ureters. Hydronephrosis is a common complication of RPF, however, its clinical features and outcomes have not been well elucidated.
Methods: A total of 115 RPF-related hydronephrosis patients have been recruited from 9 clinical centers in China since March 2010.
Rheumatology (Oxford)
December 2024
Department of Rheumatology and Clinical Immunology, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), The Ministry of Education Key Laboratory, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Objectives: Idiopathic retroperitoneal fibrosis (IRF) is a rare autoimmune-mediated condition characterized by fibro-inflammatory tissue development around the abdominal aorta and iliac arteries. Ureteral entrapment and hydronephrosis are the most common manifestations and acute renal failure or chronic renal insufficiency may occur as the consequence. Glucocorticoids and immunosuppressants may be effective but the therapeutic response and outcome are heterogeneous and hard to predict.
View Article and Find Full Text PDFMod Rheumatol Case Rep
December 2024
The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Japan, Kitakyushu, Japan.
The patient was a 57-year-old man who developed bilateral thigh pain and chest tightness one year ago. Chest CT scan showed reticular shadows, thickened interlobular septa in both lung fields, and pericardial effusion. Three months ago, his symptoms worsened.
View Article and Find Full Text PDFRheumatology (Oxford)
December 2024
Department of Renal and Transplant Medicine, West London Renal and Transplant Centre, Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK.
Objectives: To determine the clinical outcomes of patients with immunoglobulin 4-related disease (IgG4-RD) treated with a defined B cell depletion protocol using rituximab.
Methods: Patients were included if they had (1) an IgG4-RD diagnosis at Imperial College Healthcare NHS Trust between February 2017 and October 2022, and (2) >9 months of follow-up data available following the first rituximab dose. The rituximab protocol targeted B cell depletion to < 10 cells/microliter for a maintenance period of two years.
Hematology Am Soc Hematol Educ Program
December 2024
Division of Hematology, University of British Columbia, Vancouver, British Columbia, Canada; and Division of Hematology, Dalhousie University, Halifax, Nova Scotia, Canada.
Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated disease with many important manifestations in hematopoietic and lymphoid tissue. IgG4 is the least naturally abundant IgG subclass, and the hallmark feature of IgG4-RD is markedly increased IgG4-positive plasma cells (with an IgG4 to IgG ratio >40%) in affected tissue, along with elevated polyclonal serum IgG and IgG4 in most patients. Histological diagnosis is essential, and other key features include storiform fibrosis, lymphoplasmacytic infiltrate, tissue eosinophilia, and obliterative phlebitis.
View Article and Find Full Text PDFMol Cell Endocrinol
December 2024
Laboratorio de Estudio de Enfermedades Metabólicas Relacionadas con la Nutrición, Facultad de Bioquímica y Ciencias Biológicas, Universidad Nacional del Litoral, Santa Fe, Argentina; Consejo Nacional de Investigaciones Científicas y Técnicas (CONICET), Argentina. Electronic address:
Clin Nucl Med
January 2025
From the Department of Nuclear Medicine, University of Health Sciences Turkey, Basaksehir Cam and Sakura City Hospital, Istanbul, Turkey.
IgG4-releated disease is systemic fibroinflammatory disease characterized by infiltration of IgG4-positive plasma cells in the affected tissue. Mediastinal fibrosis, which is also known as fibrosing mediastinitis, is a rare form of IgG4-releated disease. We present a case of mediastinal and retroperitoneal fibrosis in 52-year-old man who was detected with increased radiopharmaceutical uptake in 18F-FDG PET/CT imaging.
View Article and Find Full Text PDFIntern Med
November 2024
Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University, Japan.
Diagnostics (Basel)
November 2024
Department of Medicine, Universitat Autònoma de Barcelona (UAB), 08193 Barcelona, Spain.
Introduction: We conducted a comprehensive comparative analysis of the Okazaki, Umehara, and American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for diagnosing immunoglobulin G4-related disease (IgG4-RD).
Materials And Methods: A retrospective study was conducted in a single tertiary hospital, using expert clinical judgment as the gold standard. We compared the diagnostic accuracy of the Okazaki, Umehara, and ACR/EULAR criteria in a cohort of 41 patients with suspected IgG4-RD.
Urologiia
September 2024
Center of Urology with robot-assisted surgery of City Mariinsky hospital, Saint Petersburg, Russia.
Introduction: After buccal ureteroplasty of long stricture of ureteropelvic junction and proximal ureter, there is a risk of recurrent stricture and urinary stone formation, requiring endoscopic procedure.
Aim: To evaluate the possibility of performing ureteroscopy (URS) in patients after onlay ureteroplasty, as well as to study its results and efficiency.
Materials And Methods: Buccal ureteroplasty was performed in 30 patients who had previously undergone endoscopic and reconstructive procedures on the upper urinary tract.
Pediatr Nephrol
November 2024
Department of Pediatrics, AIIMS Jodhpur, Jodhpur, India.
Background: Shear wave elastography (SWE) is proven for liver fibrosis. However, there are challenges in assessing the kidney owing to its surrounding structures, retroperitoneal location, and visceral fat. Kidney biopsy is the gold standard for estimating fibrosis, but is associated with inherent risks of bleeding and sedation.
View Article and Find Full Text PDFEur Urol Focus
November 2024
Institute of Urology, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA. Electronic address:
Background And Objective: The feasibility and safety of a robotic approach for postchemotherapy retroperitoneal lymph node dissection (PC-RPLND) in testicular cancer have been demonstrated, but data on long-term oncological outcomes of this procedure are limited. Our aim was to evaluate oncological outcomes following robotic PC-RPLND in this setting.
Methods: This retrospective cohort study included consecutive patients with testicular cancer treated with robotic PC-RPLND at 11 academic centers worldwide between 2011 and 2023.
Isr Med Assoc J
November 2024
Department of Internal Medicine E, Soroka University Medical Center, Faculty of Health Sciences, Ben Gurion University of the Negev, Beer Sheva, Israel.
Background: Idiopathic retroperitoneal fibrosis is characterized by the development of inflammatory infiltrates with marked fibrosis along the large retroperitoneal vessels. Rituximab in combination with glucocorticoids constitute an effective therapy, but the responses are not long-lasting. In other similar situations, addition of cyclophosphamide to the combination achieved longer and deeper responses.
View Article and Find Full Text PDFClin Imaging
December 2024
Bezmialem Vakıf University Hospital, Department of Radiology, 34093 Istanbul, Turkey.
Primary retroperitoneal neoplasms (PRNs) are a complex and diverse group of tumors arising in the retroperitoneal space, excluding those from retroperitoneal organs. These masses present significant diagnostic challenges due to their heterogeneous nature. PRNs primarily include sarcomas, neurogenic tumors, extragonadal germ cell tumors, and lymphomas, with the majority being malignant.
View Article and Find Full Text PDFInt J Surg Case Rep
December 2024
Santa Casa de Misericordia de Santos, Divisão de Urologia, Departamento de Cirurgia, Santos, São Paulo, Brazil; Faculdade de Medicina, Universidade Metropolitana de Santos (UNIMES), Departamento de Urologia, Santos, São Paulo, Brazil.
Introduction And Importance: Retroperitoneal fibrosis is a proliferative disease of fibroblasts with a still unclear appearance and low incidence. The clinical manifestations are nonspecific and appear late, pain is the most common symptom present. Elevated serum IgG4 levels are observed in up to 60 % of the patients and the main goal of treating this condition is to preserve kidney function.
View Article and Find Full Text PDFRambam Maimonides Med J
October 2024
The B. Shine Rheumatology Institute, Rambam Health Care Campus, Haifa, Israel.
IgG4-related disease (IgG4-RD) is a rare illness with inflammatory and fibrotic changes in affected organs such as pancreas, thyroid, salivary or lacrimal glands, and retroperitoneal space; rarely other organs may be involved. IgG4-related breast disease (IgG4-BD) is very rare and generally presents as a lump or mastitis. IgG4-BD as a presenting feature of IgG4-RD is extremely rare.
View Article and Find Full Text PDFWorld J Urol
November 2024
Department of Urology, University of Leipzig, Leipzig, Germany.
J Indian Assoc Pediatr Surg
August 2024
Department of Paediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.
Background: The occupational hazards of glutaraldehyde are well known; the possibility of harm to the patients has been highlighted in the form of isolated reports only.
Objective: To synthesize evidence for contact mucosal injury or injury due to intraperitoneal instillation of glutaraldehyde following its use during laparoendoscopy.
Materials And Methods: The current review is Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) compliant.
Rheumatology (Oxford)
October 2024
Department of Rheumatology, Zhongshan Hospital, Fudan University, Shanghai, China.
Asian J Surg
October 2024
Department of Ultrasound, The First Affiliated Hospital of Dalian Medical University, China.
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