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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1787047PMC
http://dx.doi.org/10.1136/bmj.4.5843.791-cDOI Listing

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Objective: This study investigated the efficacy of comprehensive management and predictable inflammatory markers for idiopathic retroperitoneal fibrosis (iRPF)-related hydronephrosis outcomes.

Methods: Patients with iRPF-related hydronephrosis underwent surgical (ureteral stent and/or nephrostomy tube placement) and medical (corticosteroid-based multiple immunosuppressants) management were classified according to stent-indwelling outcomes. Univariate analysis of clinical profiles was conducted to screen possible predictors of hydronephrosis remission.

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Histiocytosis are caused by pathogenic myeloid cells, and can be classified as Langerhans cell histiocytosis (LCH) and non-LCH. Erdheim-Chester disease (ECD) is a non-LCH, characterized by multi-organ involvement, typical imaging findings, and confirmatory histological studies. A case with multi-organ involvement and histological confirmation is presented.

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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.

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IgG4-related disease for the hematologist.

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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.

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Article Synopsis
  • Retroperitoneal fibrosis (RPF) is a rare condition causing abnormal tissue growth around major blood vessels in the abdomen, often affecting nearby structures like the ureters, with most cases being idiopathic.
  • Recent advancements in understanding RPF focus on its causes, diagnosis through biomarkers and imaging, and various treatment methods, including corticosteroids and surgery.
  • A comprehensive management approach that combines both medical and surgical options is essential for achieving the best outcomes for patients with RPF.
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