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fibrotic disorders

Idiopathic fibrosclerotic disorders, idiopathic fibrosis

Fibroinflammatory disorders constitute heterogeneous clinical conditions whose cause and pathogenesis are largely unknown. Although the pathogenesis of the fibrosclerotic disorders has not been elucidated, autoimmunity in the context of an established collagen vascular disease or the setting of inflammatory periaortitis in retroperitoneal fibrosis has been one suggested mechanism.

There are several other fibroinflammatory processes, such as focal myositis, inflammatory fibroid polyp of the gastrointestinal tract, calcifying fibrous pseudotumor, and sclerosing peritonitis, which are probably unrelated to inflammatory myofibroblastic tumor or the primary fibrosclerotic disorders.

Inflammatory pseudotumor has been applied in a generic sense to several of these disorders, which present as a mass displacing surrounding anatomic structures or leading to organ dysfunction secondary to compressive growth around the ureter(s), common bile duct, or great vessels in the mediastinum.

Types

- retroperitoneal fibrosis
- sclerosing mediastinitis
- sclerosing cholangitis
- orbital pseudotumor
- Riedel thyroiditis
- sclerosing mesenteritis

References

- Dehner LP, Coffin CM. Idiopathic fibrosclerotic disorders and other inflammatory pseudotumors. Semin Diagn Pathol. 1998 May;15(2):161-73. PMID: 9606807