Sjögren’s Syndrome

Meridith Balbach

Background

  • Systemic autoimmune disorder primarily targeting exocrine glands causing sicca symptoms; may also involve musculoskeletal, pulmonary, renal, neurologic, and hematologic features.
  • May be primary or secondary (occurring alongside another autoimmune disease such as RA or SLE).
  • Incidence: presents most often around age 40-60y; female-to-male ratio ~9:1.
  • Etiology: genetic predisposition (e.g. increased risk with specific HLA-DQA_DQB_ haplotypes) + environmental factors triggers aberrant autoantibody production and lymphocytic infiltration of glands (predominantly CD4+ T cells; also B cells), resulting in glandular dysfunction.

Presentation

  • Classic sicca: dry eyes (gritty, burning, or foreign body sensation) and dry mouth (difficulty chewing/swallowing dry foods, frequent water intake, dental caries).
  • Extra-glandular features:
    • MSK: arthralgias, arthritis, myalgias.
    • Dermatologic: eyelid dermatitis, Raynaud’s, cutaneous vasculitis.
    • Hematologic: cytopenias, hyper- and hypogammaglobulinemia, monoclonal gammopathy, cryoglobulinemia; non-Hodgkin lymphoma, particularly MALT lymphoma (most feared complication; higher risk if persistent parotid enlargement or hypocomplementemia).
    • Pulmonary: ILD, xerotrachea, bronchiectasis.
    • Gastrointestinal: dysphagia, chronic diarrhea, abdominal pain.
    • o Nephrology: tubulointerstitial nephritis +/- renal tubular acidosis.
    • May also include neurologic, renal, cardiovascular (but typically <5%).

Evaluation

  • Serologic workup: ANA with reflex (typically + Anti-Ro/SSA or anti-La/SSB), RF (often positive, even in the absence of RA), immunoglobulins (IgG high), ESR (often elevated) and CRP (usually normal or mildly elevated).
    • Anti-Ro/SSA is most sensitive and specific but is not diagnostic.
  • Additional workup: Schirmer test, slit-lamp exam, salivary flow rate.
  • Gold standard: labial salivary gland biopsy.
  • Clinical diagnosis but 2016 ACR/EULAR classification criteria can help guide in unclear cases (score ≥4 confirms classification):
    • + anti-Ro/SSA (3).
    • Focal lymphocytic sialadenitis (3).
    • Ocular staining score ≥5 (1).
    • Schirmer’s test ≤5 mm/5 min (1).
    • Unstimulated salivary flow ≤0.1 mL/min (1).

Management

  • Treatment is currently targeted to organ involvement (though several biologics are being studied). Immunosuppression is not used for dryness symptoms.
    • Xerophthalmia: preservative-free artificial tears, cyclosporine eye drops; punctal plugs if refractory
    • Xerostomia: frequent sips, sugar-free lozenges or gum, saliva substitutes + good dental hygiene; muscarinic agonists (e.g. pilocarpine) if persistent
    • MSK: hydroxychloroquine
    • Organ-threatening disease: steroids +/- steroid-sparing agent (MMF, azathioprine, rituximab)
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