Chronic Sclerosing Sialadenitis



Chronic Sclerosing Sialadenitis


Bruce M. Wenig, MD










Submandibular chronic sclerosing sialadenitis is characterized by retention of the lobular architecture, dense inflammatory infiltrate, acinar atrophy, and interlobular fibrosis.






It is now recognized that submandibular chronic sclerosing sialadenitis may be an IgG4-related disease, confirmed by the presence of abundant IgG4 immunoreactive plasma cells image.


TERMINOLOGY


Abbreviations



  • Chronic sclerosing sialadenitis (CSS)


Synonyms



  • Küttner tumor


  • IgG4-associated sialadenitis


  • Punctate parotitis


Definitions



  • Chronic fibroinflammatory salivary gland disease with characteristic morphology that may represent IgG4- associated disease


ETIOLOGY/PATHOGENESIS


Immune-Mediated



  • Increasing evidence that CSS, if IgG4 related, may be component of IgG4-related systemic disease



    • IgG4-related diseases include



      • Autoimmune pancreatitis


      • Involvement of extrapancreatic organs including kidney, lung, retroperitoneum, liver, gallbladder, lymph nodes, breast, salivary glands, lacrimal gland, aorta


    • Morphologic features in CSS suggesting immune-mediated process include



      • Presence of prominent lymphoplasmacytic infiltrate and lymphoid follicles


      • Presence of unusual cytotoxic T-cell populations


Obstructive Sialadenitis



  • Prior to IgG4 association, sialolithiasis felt to be commonly associated with CSS of submandibular gland



    • May be true in percentage of non-IgG4-related cases


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Unknown, but under recognized


  • Age



    • Most often occurs in 4th to 7th decades


  • Gender



    • Affects males slightly more often than females


Site



  • Primarily affects submandibular gland



    • Rarely, multiple salivary glands (major and minor) may be affected in single patient


Presentation



  • Pain and swelling of affected gland common



    • Often associated with ingestion of food


  • Patients may present with asymptomatic swelling of affected gland


  • May be localized to salivary gland involvement


  • May be associated with sclerosing lesions in extrasalivary gland tissues (systemic IgG4-related disease)


Laboratory Tests



  • Serum IgG4, IgG, IgG4/IgG ratio (normally 3-6%) typically elevated


  • Antibodies present in Sjögren syndrome including anti-SS-A, anti-SS-B not found in CSS


  • Absence of antineutrophilic antibodies (cytoplasmic and perinuclear)


  • Eosinophilia, hypergammaglobulinemia, and antinuclear antibodies (ANA) may be present in systemic but not localized disease


Treatment



  • Options, risks, complications



    • IgG4-related sialadenitis steroid sensitive


  • Surgical approaches




    • CSS associated with sialolithiasis



      • Removal of stone by surgery, endoscopy or lithotripsy


      • In approximately 20% of cases, symptoms persist, necessitating surgical resection of involved gland


Prognosis



  • IgG4-related



    • Excellent response to steroid


  • Sialolithiasis-related



    • Removal of stone results in reduction in swelling and pain


  • Rarely, extranodal marginal zone B-cell lymphoma (MALT) of salivary gland and salivary duct carcinoma may arise in background of CSS


IMAGE FINDINGS


Radiographic Findings

Jul 7, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Chronic Sclerosing Sialadenitis
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