Sjögren Syndrome

• • Chronic, systemic inflammatory disorder of unknown etiology

  • Dry mouth, eyes, and other mucous membranes

  • Associated with rheumatic disorders such as rheumatoid arthritis, scleroderma, and systemic lupus erythematosus (SLE) (all have lymphocytic infiltration into affected tissues)

  • Association with HLA-DR3

  • Primary syndrome involves the eyes, mouth, and mucous membranes (sicca syndrome or complex); secondary syndrome has an associated generalized collagen-vascular disease

Epidemiology

• • More common than SLE, but less common than rheumatoid arthritis

  • Arthritis occurs in about 33% of patients

Symptoms and Signs

• • Dessicated cornea and conjunctiva

  • 33% of patients have enlarged parotid glands—usually firm, smooth, mildly tender, and fluctuate in size

  • Taste and smell sensations diminished

  • May have dry skin

  • Dry mucous membranes throughout the body

  • Alopecia

  • Joint inflammation

Laboratory Findings

• • Schirmer test: Measures quantity of tears secreted in 5 minutes in response to irritation stimuli; decreased in Sjögren syndrome

  • Evaluate salivary glands by salivary flow, sialography, salivary scintiscan, or biopsy

  • Elevated levels of serum antibodies to gamma globulin, nuclear protein, and many tissue constituents

  • Elevated rheumatoid factor (70% of cases)

  • Negative VDRL

  • Elevated ESR (70% of cases)

  • Proteinuria (in presence of interstitial nephritis)

• • Dryness of the respiratory tract may lead to lung infections

  • Associated with chronic hepatobiliary disease and pancreatitis

  • May develop fibrinus pericarditis, sensory neuropathy, and renal insufficiency (due to interstitial nephritis)

  • Patients at risk for Waldenstrom macroglobulinemia

  • Slit lamp exam is useful to evaluate for Sjögren syndrome

Rule Out

• • Lymphoma (44-fold increased risk for lymphoma in patients with Sjögren syndrome)

• • History and physical exam suggests diagnosis

Surgery

Indications

• • Tarsorrhaphy if artificial tears fail

Medications

• • Artificial tears

  • Saliva substitute, chewing sugar-free gum, and sipping fluids throughout the day

  • Oral corticosteroids or immunosuppressants are rarely indicated as connective tissue involvement is usually mild and chronic

Prognosis

• • Related to the underlying connective tissue disorder

  • Death rarely occurs from pulmonary failure, renal failure, or lymphoma

• • Belafsky PC, Postma GN. The laryngeal and esophageal manifestations of Sjögren's syndrome. Curr Rheumatol Rep. 2003;5:297.

  • Borchers AT et al. Immunopathogenesis of Sjögren's syndrome. Clin Rev Allergy Immunol. 2003;25:89.