Cause:Autoimmune?; a retrovirus?; impaired response to cholinergic stimluli (Ann Rheum Dis 2000;59:48); primary or secondary to scleroderma, rheumatoid arthritis, SLE, primary biliary cirrhosis, vasculitis, thyroiditis, hepatitis C (J Hepatol 1999;31:210), etc
Pathophys:Lymphocyte-mediated destruction of exocrine glands leads to mucosal dryness; an immune complex small vessel vasculitis
Females >> males; 2nd most common collagen vascular disease after RA. Associated with HLA B8 (54%) and HLA DRW3 (75%)
Sx:Primary type: Raynaud’s (20%); sicca syndrome with dry eyes and mouth and secondary caries, thirst; dry cough with frequent upper and lower respiratory infections; dysphagia; dyspareunia
Secondary type: other collagen vascular disease present
Si:Primary type: parotid enlargement (80%); pseudolymphoma; caries; positive Schirmer test (<5 mm/5 min filter paper ascent from eyelid) but many false positives and negatives (Br J Rheum 1993;32:231)
Primary type: obstructive and restrictive lung disease; gastric atrophy; pancreatitis; interstitial renal disease and Fanconi’s syndrome; B-cell lymphomas (Nejm 1987;316:1118) and perhaps other cancers; staph conjunctivitis; blindness and other MS-like syndromes in 20% (Ann IM 1986;104:322)
r/o other causes of sicca syndrome (Cornea 1999;18:625): type IV and V hyperlipidemia, sarcoid, hemachromatosis, amyloid, local irradiation, HIV infection, which can be associated with as well as cause secondary Sjögren’s (Bull Rheum Dis 1992;40[6]:6)
Lab:
Hem:ESR elevated (Semin Arth Rheum 1992;22:114)
Path:Lip salivary gland bx shows lymphocytic infiltration (94%) (Nejm 1987;316:1118)
Serol:Positive RA titer (90%), ANA (50-80%), SSA, SSB (Ankle and Foot Injuries)
Rx:Jama 2010;304:452
Methylcellulose eye gtts and mouth wash, or “Lacrisert” methylcellulose lid insert q 12 h; perhaps cyclosporine 0.05% opth gtts bid (Med Let 2003;45:42) for Sjögren’s and idiopathic, marginal help for $6/d
Steroids if marked organ- or life-threatening complications; or other immunosuppressive agents and Rituxan
of aphthous ulcers: tetracycline oral rinse
of dry mouth (Med Let 2000;42:70):