The immunological pathogenesis of IgG4-related disease categorized by clinical characteristics.

IgG4-related disease (IgG4-RD) is an immune disorder characterized by organ enlargement and fibrosis leading to functional impairment. Key immune cell subsets contributing to the pathogenesis of IgG4-RD include T follicular helper 2 cells (Tfh2), Tfh1, CX3CR1 + cytotoxic T cells (CX3CR1 + CTLs), Tregs and IgG4 + B cells. Tfh2 and Tregs are commonly involved in inducing IgG4 class-switching in this disease.

New insights into predictors of autoimmune pancreatitis relapse after steroid therapy.

Objectives: While autoimmune pancreatitis (AIP) responds well to steroid therapy, the high relapse rate in type 1 AIP remains a critical problem. The present study examined predictors of relapse of type 1 AIP following steroid therapy.

Materials And Methods: Nine factors potentially predictive of relapse were analyzed in 81 AIP patients receiving steroid therapy with follow-up ≥ 12 months.

IgG4-Related Disease in a 90-Year-Old Man with an 18-Year Disease Course: A Case Report.

IgG4-related disease (IgG4-RD) is a multi-organ inflammatory immune-mediated illness caused by IgG4-secreting plasma cells infiltrating the tissue. This condition usually affects elderly men. A 90-year-old Chinese male was diagnosed with IgG4-RD based on the new 2019 ACR/EULAR classification criteria, as he had multiple organ involvement.

Interesting New Findings of Retroperitoneal Fibrosis: Three Cases.

Objective: Retroperitoneal fibrosis is a rare disease characterized by chronic inflammation and fibrosis in the retroperitoneal space that may wrap around the ureter and cause an obstruction. Here we present the cases of three patients diagnosed and treated between April and August 2022.

Case Presentation: Here we present three cases of retroperitoneal fibrosis.

Subcutaneous ureteral bypass for treatment of proximal ureteral obstruction secondary to retroperitoneal fibrosis after renal transplantation in a cat.

Case Summary: A 5.5-year-old male neutered domestic shorthair cat was presented with a 2-year history of progressive chronic kidney disease. Abdominal ultrasonography revealed bilateral chronic renal degeneration, nephrolithiasis, cortical hyperechogenicity and infarction.

IgG4-Related Retroperitoneal Fibrosis in a Patient With B-Cell Lymphoproliferative Disorder.

We report an interesting case of a 25-year-old male patient who presented with a complaint of pain in the abdomen for six months, which was not associated with any other symptom, the patient was diagnosed with IgG4-related retroperitoneal fibrosis (RF) via endoscopic ultrasound (EUS)-guided biopsy. He was prescribed steroids and proton pump inhibitors. Due to the limited presentation and rarity of RF, diagnosis of this disease requires extensive diligence and care.

A Case of Idiopathic Retroperitoneal Fibrosis Triggering Recurrent Gastrointestinal Bleeding.

Idiopathic retroperitoneal fibrosis (IRF) is a rare condition characterized by fibrous tissue proliferation in the retroperitoneal space, commonly affecting the ureters and other abdominal structures. This case report describes a previously undocumented presentation of IRF in a 52-year-old female, who presented with recurrent gastrointestinal bleeding and severe anemia over six months. Diagnostic workup included endoscopy, colonoscopy, abdominal computed tomography (CT), and biopsy, revealing fibrous encasement of the mesenteric vessels leading to ischemic damage and gastrointestinal bleeding.

A review of the current treatment methods for retroperitoneal fibrosis with obstructive uropathy.

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.

Mini-review: Evaluation and Management of Retroperitoneal Masses in Patients with Testicular Cancer.

Testicular germ cell tumours (GCTs) account for the majority of testicular malignancies. Seminomas and nonseminomas differ in prognosis and management strategies. While cisplatin-based chemotherapy has significantly improved survival rates, identification of residual masses after chemotherapy is crucial for determining further treatment and survival.

Description and Cross-Sectional Analyses of 25,880 Adults and Children in the UK National Registry of Rare Kidney Diseases Cohort.

Introduction: The National Registry of Rare Kidney Diseases (RaDaR) collects data from people living with rare kidney diseases across the UK, and is the world's largest, rare kidney disease registry. We present the clinical demographics and renal function of 25,880 prevalent patients and sought evidence of bias in recruitment to RaDaR.

Methods: RaDaR is linked with the UK Renal Registry (UKRR, with which all UK patients receiving kidney replacement therapy [KRT] are registered).

A Rare Case of Retroperitoneal Tumefactive Fibroinflammatory Lesion Related to IgG4-Sclerosing Disease.

We present a unique case of a retroperitoneal tumefactive fibroinflammatory lesion related to IgG4-sclerosing disease; it is a rare manifestation of the IgG4-related disease, which usually causes diffuse fibrosis when located in the retroperitoneum, rather than mass-like lesions. A 49-year-old man presented to the emergency department complaining of abdominal pain and vomiting. Subsequent testing with abdominal ultrasound, CT, and MRI revealed a large retroperitoneal mass of unknown origin, heterogenous, with a concentric circles pattern best visualized in MRI.