Retroperitoneal fibrosis - PubMed

3 hours ago

Retroperitoneal fibrosis is a rare immune-mediated disease characterized by fibro-inflammatory tissue around the aorta and iliac arteries that often encases nearby structures like ureters.
It can be idiopathic (isolated or part of IgG4-related disease) or secondary due to causes such as histiocytosis, malignancies, or infections, with multifactorial origins including genetic, environmental, and lifestyle factors.
Immunopathogenesis involves B-cell and T-cell interactions, macrophage and fibrocyte chemotaxis, and recruitment of eosinophils and mast cells, leading to complications like kidney injury, caval occlusion, and deep vein thrombosis.
Treatment relies on medical therapy, with glucocorticoids as effective options and increasing use of B-cell-targeting therapies, though relapses are common after discontinuation; interventional or surgical procedures may be needed for complications.

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