Sclerosing Fibroinflammatory Lesions



Sclerosing Fibroinflammatory Lesions


Elizabeth A. Montgomery, MD





Key Facts


Terminology



  • Group of poorly understood lesions unified by sclerosis and inflammation, with variable tumor formation and variable association with elevated serum IgG4 and intralesional IgG4-producing plasma cells


  • IgG4-related chronic sclerosing disorders



    • Lymphoplasmacytic sclerosing pancreatitis (autoimmune pancreatitis)


    • Sclerosing lymphoplasmacytic tubulointerstitial nephritis


    • Reidel struma (thyroiditis)


    • Orbital pseudotumor (chronic sclerosing dacryoadenitis)


    • Chronic sclerosing sialadenitis (Küttner tumor)


    • Retroperitoneal fibrosis (Ormond disease, sometimes called retractile mesenteritis)


  • Some patients manifest several of above lesions simultaneously


  • Other lesions less associated with increased IgG4



    • Sclerosing mesenteritis


    • Plasma cell granuloma (inflammatory myofibroblastic tumor of lung)


Clinical Issues



  • Steroids administered for lesions associated with elevated serum IgG4


Microscopic Pathology



  • All lesions feature fibrosis and lymphoplasmacytic inflammation with scattered eosinophils


  • IgG4-labeled plasma cells on immunohistochemistry






Hematoxylin & eosin shows a needle biopsy specimen from an example of retroperitoneal fibrosis that encased the ureters. The process has surrounded a nerve image and infiltrates adipose tissue.






Lymphoplasmacytic sclerosing (autoimmune) pancreatitis is the prototype of the IgG4-related sclerosing disorders. In this field, there is dense lymphoplasmacytic inflammation around a duct.


TERMINOLOGY


Synonyms



  • IgG4-related chronic sclerosing disorders


Definitions



  • Poorly understood lesions unified by sclerosis, inflammation, variable tumor formation and variable association with elevated serum IgG4/intralesional IgG4-producing plasma cells



    • Lymphoplasmacytic sclerosing pancreatitis (autoimmune pancreatitis)


    • Sclerosing lymphoplasmacytic tubulointerstitial nephritis


    • Reidel struma (thyroiditis)


    • Orbital pseudotumor (chronic sclerosing dacryoadenitis)


    • Chronic sclerosing sialadenitis (Küttner tumor)


    • Retroperitoneal fibrosis (Ormond disease, sometimes called retractile mesenteritis)



      • Associated with various medications, including methysergide, β-blockers, chemotherapeutic agents


    • Aortic inflammatory lesions


    • Some patients manifest several of these lesions simultaneously


  • Other lesions less associated with increased IgG4



    • Sclerosing mesenteritis



      • Overlaps with retroperitoneal fibrosis and is thus also sometimes called retractile mesenteritis


    • Plasma cell granuloma (inflammatory myofibroblastic tumor of lung)



      • Subset of such lung lesions is associated with increased serum IgG4 and similar lesions in other sites


ETIOLOGY/PATHOGENESIS


Unknown



  • Association with elevated IgG4 in some cases raises possibility of autoimmune or infectious disorder



    • Specific trigger unknown


  • No characteristic genetic alteration established


CLINICAL ISSUES


Presentation



  • Most present as hard masses in various sites


  • Patients with retroperitoneal fibrosis can present with urinary obstructive symptoms


  • Most pancreatic examples initially believed to be pancreatic cancer clinically


Treatment



  • Surgery for sclerosing mesenteritis


  • Steroids for lesions associated with elevated serum IgG4


  • For retroperitoneal fibrosis, surgery to reduce ureteral obstruction



    • Added medical therapy includes steroids, methotrexate, cyclophosphamide, and mycophenolate mofetil


Prognosis

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Jul 9, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Sclerosing Fibroinflammatory Lesions

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