Sjögren Syndrome

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

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

Laboratory Tests

• Rheumatoid factor is found in 70% of patients

• Antibodies against SS-A and SS-B 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

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

• Extraglandular disease, including arthritis, vasculitis, or pulmonary manifestations, are treated with similar immunosuppressive medications as SLE or rheumatoid arthritis

Therapeutic Procedures

• Treatment of sicca symptoms is symptomatic and supportive

• Artificial tears applied frequently will relieve ocular symptoms and avert further desiccation

• Sipping water frequently or using sugar-free gums and hard candies usually relieves dry mouth symptoms

• A program of oral hygiene is essential to preserve dentition

Complications

• A spectrum of lymphoproliferation ranging from benign to malignant may be found

• Malignant lymphomas and Waldenström macroglobulinemia occur nearly 50 times more frequently than can be explained by chance alone in primary Sjögren syndrome

Prognosis

• Usually benign and consistent with a normal life span

• Prognosis is mainly influenced by the nature of the associated disease

• The patients (3–10% of the total Sjögren population) at greatest risk for developing lymphoma have

  • Severe dryness

  • Marked parotid gland enlargement

  • Splenomegaly

  • Vasculitis

  • Peripheral neuropathy

  • Anemia

  • Mixed monoclonal cryoglobulinemia

When to Refer

• Presence of systemic symptoms or signs

• Ocular dryness not responsive to artificial tears

When to Admit

• Presence of severe systemic signs such as vasculitis unresponsive to outpatient management