Sjogren Syndrome



Sjogren Syndrome


Kevin R. Torske, DDS, MS

Lester D. R. Thompson, MD










The parotid gland is diffusely infiltrated by chronic inflammatory cells. The normal architectural morphology is intact, with fibrous septae separating the glandular lobules. Glandular effacement is not present.






Labial minor salivary gland biopsy shows a lymphoid aggregate image within the salivary acini. One or more foci within 4 mm2 of salivary gland tissue is supportive of a diagnosis of Sjögren syndrome.


TERMINOLOGY


Abbreviations



  • Sjögren syndrome (SS)


Synonyms



  • Mikulicz disease, Sicca syndrome


Definitions



  • Primary SS: Chronic, systemic autoimmune disease primarily affecting parotid and lacrimal excretory glands, leading to xerostomia and xerophthalmia, respectively


  • Secondary SS: Above in association with another autoimmune, connective tissue disorder



    • Typically rheumatoid arthritis or systemic lupus erythematosus


ETIOLOGY/PATHOGENESIS


Primary SjöGren Syndrome



  • Etiology unknown, yet likely multifactorial and complex



    • Endocrine: Systemic and local androgen deficiencies


    • Genetics



      • 1° SS associated with HLA-B8 and HLA-Dw3


      • 2° SS associated with HLA-DRw4


    • Viral infection: EBV, Coxsackievirus, and HTLV-1


    • Autonomic nervous system



      • Blockage of receptor sites by antimuscarinic antibodies (anti-M3R)


    • Upregulation of INFα and β-cell activating factor


CLINICAL ISSUES


Epidemiology



  • Incidence



    • 0.5-3% of population



      • May be undiagnosed in nearly half of affected patients


  • Age



    • Mean: 5th-7th decades



      • Peaks at menarche and menopause


  • Gender



    • Female > > > Male (9:1)



      • Males may be more common in juvenile presentation


  • Ethnicity



    • No racial predilection


Site



  • Lacrimal and parotid glands most severely affected



    • Commonly bilateral


  • Submandibular, sublingual, and minor salivary glands affected to lesser degree


  • Upper respiratory tract



    • Sinonasal mucosa, eustachian tube, and larynx


  • Multiple other organ systems possibly affected


Presentation



  • Xerostomia (dry mouth)



    • Typically noted after saliva production falls below 50% of normal


    • Taste alterations


    • Difficulties in speech and mastication


    • Possible burning sensation


    • Less frequently associated with



      • Dental caries


      • Dry, cracked lips, angular cheilitis


      • Fissured or depapillated tongue


      • Chronic candidiasis


      • Oral ulcerations


  • Xerophthalmia (dry eyes, keratoconjunctivitis sicca)



    • Pain and foreign body sensation


    • Photosensitivity, ocular fatigue


    • Redness, loss of visual acuity, filamentary keratitis


  • Parotid gland




    • Recurrent, firm, diffuse parotid enlargement lasting weeks to months, with occasional remission



      • Enlargement correlated with increased disease severity


      • Persistent swelling may indicate transformation into low-grade lymphoma


    • Possible discomfort/pain, which may increase with eating


    • Potential retrograde infections with suppuration from Stensen duct


  • Lacrimal gland



    • Typically painless, bilateral swellings of lateral orbital margin


    • Associated with chronic dacryoadenitis


    • Associated with infection (especially viral), sarcoidosis, Graves disease, and orbital inflammatory syndrome


  • Other presentations based upon affected system


  • Many patients have other connective tissue, autoimmune, or rheumatologic disorders



    • Diabetes, sarcoidosis, malnutrition, infection, alcoholism, bulimia


    • Medications can be related to disorder development


Laboratory Tests



  • Diagnosis based upon results of multiple tests



    • Ocular



      • Schirmer test: Decreased tear secretion


      • Rose bengal and lissamine green: Dyes used to examine eye surface for dry spots


    • Salivary flow tests (sialometry)


    • Serology



      • Positive anti-SS-A (RO) &/or anti-SS-B (LA)


      • Also autoantibodies for muscarinic receptor M3, IFI16, KLHL12, or KLHL7


      • Rheumatoid factor positive in up to 95%


      • Anti-nuclear antibody (ANA) positive in up to 80%


      • Erythrocyte sedimentation rate (ESR) usually elevated


      • Immunoglobulins: Usually elevated in SS patients


Treatment



  • Treatment mostly supportive


  • Xerostomia: Adequate hydration and stimulation of salivary flow



    • Topical stimulation: Sugar-free gum, candies, or lozenges


    • Systemic stimulation: Pilocarpine, cevimeline, bromhexine, tibolone


  • Xerophthalmia



    • Topical: Artificial tears, including eye drops or ointments


    • Systemic: Androgens (methyl-testosterone) or cyclosporine


  • Oral hygiene instruction, topical fluoride, and chlorhexidine rinses


  • Antifungal therapy for chronic candidiasis


Prognosis

Jul 7, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Sjogren Syndrome
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