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Key Features

Essentials for Diagnosis

  • Dryness of eyes and dry mouth (sicca components); they occur alone or with rheumatoid arthritis or other connective tissue disease

  • Rheumatoid factor and antinuclear autoantibodies common

  • Increased incidence of lymphoma

General Considerations

  • A systemic autoimmune disorder

  • Clinical presentation is usually dominated by dryness of the eyes and mouth due to immune-mediated dysfunction of the lacrimal and salivary glands

  • Can occur in isolation ("primary" Sjögren syndrome) or in association with another rheumatic disease, most frequently rheumatoid arthritis

Associated conditions

  • Rheumatoid arthritis

  • Systemic lupus erythematosus (SLE)

  • Primary biliary cirrhosis

  • Scleroderma

  • Polymyositis

  • Hashimoto thyroiditis

  • Polyarteritis

  • Interstitial pulmonary fibrosis

Demographics

  • Predominantly a disease of women, with a female:male ratio of 9:1

  • Greatest incidence between ages 40 and 60 years

Clinical Findings

Symptoms and Signs

  • Eyes

    • Ocular burning, itching, ropy secretions

    • "Grain of sand in the eye" sensation

  • Parotid glands

    • Enlargement may be chronic or relapsing

    • Develops in one-third of patients

  • Dryness of the mouth (xerostomia) leads to difficulty in swallowing dry foods (like crackers), to constant thirst for fluids, and to severe dental caries

  • There may be loss of taste and smell

  • Systemic manifestations

    • Dysphagia, pancreatitis

    • Pleuritis, obstructive airways disease and interstitial lung disease (in the absence of smoking)

    • Neuropsychiatric dysfunction

    • Small vessel vasculitis

  • Kidney

    • Renal tubular acidosis (type I, distal) occurs in 20% of patients

    • Chronic interstitial nephritis, which may result in impaired kidney function, may be seen

Differential Diagnosis

  • Sicca complex associated with other autoimmune disease, eg, sarcoidosis, rheumatoid arthritis, SLE, scleroderma

  • Other causes of dry mouth or eyes, eg, anticholinergics, mumps, irradiation, seasonal allergies, irritation from smoking

  • IgG4-related systemic disease

Diagnosis

Laboratory Tests

  • Rheumatoid factor is found in 70% of patients

  • Antibodies against SS-A and SS-B (also called Ro and La, respectively) are often present (Table 20–7)

  • When SS-A antibodies are present, extraglandular manifestations are far more common

Diagnostic Procedures

  • Lip biopsy reveals characteristic lymphoid foci in accessory salivary glands

  • Biopsy of the parotid gland should be reserved for patients with atypical presentations such as unilateral gland enlargement

  • The Schirmer test measures the quantity of tears secreted

Treatment

Medications

  • Topical ocular 0.05% cyclosporine improves ocular symptoms and signs of dryness

  • Pilocarpine (5 mg four times daily) and the acetylcholine derivative cevimeline (30 mg three times daily) are helpful for severe xerostomia

  • Prednisone or various immunosuppressive medications are used to treat severe systemic inflammatory manifestations

  • Atropinic drugs and decongestants decrease salivary secretions and should be avoided

Therapeutic Procedures

  • Treatment of sicca symptoms is symptomatic and supportive

  • Artificial ...

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