Sjogren’s Syndrome

 Description

  • Is a chronic inflammatory autoimmune process of unknown cause that affects the lacrimal and salivary glands.
  • It is thought that antibodies directed at exocrine glands are produced, causing lymphocytic infiltration and impairing function of involved tissue.
  • The syndrome may cause variety of complications such as corneal disease, tooth loss, pulmonary fibrosis or hypertension, obstructive airway disease, chronic atrophic gastritis, chronic pancreatitis, abnormal liver or kidney function, and dementia.
Causes
  • The direct cause of SS is unknown. It seems likely that both environmental and genetic factors (see Genetic Considerations) contribute to its development. In a genetically susceptible individual, either bacterial or viral infection or exposure to pollen may be the catalyst for SS.
Assessment
  1. Decreased tear production leading to keratoconjunctivitis, photophobia.
  2. Dry mouth (xerostomia), mucosal ulcers, stomatitis, salivary gland enlargement (unilateral or bilateral), dysphagia.
  3. Nasal dryness, epistaxis, nasal ulcers.
  4. Dryness of bronchial tree, hoarseness, recurrent otitis media, pneumonia, and bronchitis.
  5. Skin dryness (xeroderma), urticaria, purpura.
  6. Pancreatitis, hypochlorhydria or achlorhydria, autoimmune liver disease.
  7. Renal tubular acidosis, nephrogenic diabetes insipidus.
  8. Trigeminal neuropathy (Bell’s Palsy), polymyopathy, sensory and motor neuropathy, seizures, multiple sclerosis like syndrome.
  9. Autoimmune thyroiditis.
  10. Raynaud’s phenomenon, vasculitis infants born to mothers with Sjogen’s syndrome may have congenital heart block.
  11. Vaginal dryness, dyspareunia.
  12. Nonerosive polyarthritis.
Diagnostic Evaluation
  1. Complete blood count shows mild anemia, leukopenia present in 30% of patients.
  2. Erythrocyte sedimentation rate is elevated in 90% of patients.
  3. Rheumatoid factor is positive in 75% to 90% of patients.
  4. Antinuclear antibody is positive in 70% of patients; speckled and nucleolar patterns are most common.
  5. Antobodies to SSA or SSB detect antibodies to specific nuclear proteins.
  6. Salivary scintigraphy evaluates salivary gland function.
  7. X-rays of affected joints rule out erosive arthritis.
  8. Salivary gland biopsy may be done to determine lymphocytic infiltration of tissue.
Primary Nursing Diagnosis
  • Impaired skin integrity related to diminished or absent glandular secretions
Therapeutic Intervention
  • Care of the patient with SS is designed to treat symptoms. Instill artificial tears as often as every 30 minutes to prevent corneal ulcerations or opacifications that may be caused by insufficient lacrimal secretions.
  • Patients who also have RA may benefit from a combined program of medical, rehabilitative, and surgical treatments. The primary goals are suppression of further joint and tissue inflammation, maintenance of joint and tissue function, repair of joint damage, and relief of pain.
Pharmacologic Intervention
  1. Corticosteroids and immunosuppressants such as cyclophosphamide are used in severe cases.
  2. Antifungal agents therapeutically or prophylactically for superimposed fungal infections of mouth or vagina.
  3. Cevimeline 30 mg t.i.d., a cholinergic agonist for treatment of dry mouth.
Nursing Intervention
  1. Inspect oral mucosa for oral Candida infection, ulcers, saliva pools, and dental hygiene.
  2. Instruct or assist patient in proper oral hygiene.
  3. Encourage frequent intake of non-caffeinated, non-sugar liquids. Keep pitcher filled with cool water.
  4. Instruct or assist patient in daily inspection of skin for areas of trauma or potential breakdown.
  5. Apply lubricants to skin daily.
  6. Avoid shearing forces and encourage or perform frequent position changes.
  7. Increase liquid intake with meals.
  8. Assist or instruct patient to avoid choosing spicy or dry foods from menu choices.
  9. Suggest smaller, more frequent meals.
  10. Weigh patients weekly and review diet history for basic nutrient deficiencies.
  11. Advise patient on proper use of water-soluble vaginal lubrication.
  12. Suggest alternate positioning and practices to prevent dyspareunia.
  13. Teach the patient to recognize and report symptoms of vaginitis because infection may result from altered mucosal barrier.
  14. Advise patient of commercially available artificial saliva preparations, artificial tears, moisturizing nasal spray, and artificial vaginal moisturizers.
  15. Encourage frequent dental visits. Dental cavities are more frequent in patients with Sjogren’s syndrome.
  16. Advice patient to check with health care provider before using any medications because many cause mouth dryness (eg, diuretics, tricyclic antidepressants, antihistamines).
  17. Advice patient to wear protective eyewear while outdoors.
Documentation Guidelines
  • Physical findings of dysphagia (difficulty swallowing)
  • Physical findings of presence of red, irritated, or ulcerated mucosal membranes
  • Reaction to remoisturizing the affected areas
Discharge and Home Healthcare Guidelines
  • Instruct the patient to avoid sugar, tobacco, alcohol, and spicy, salty, and highly acidic foods.
  • Recommend high-calorie, protein-rich liquid supplements to patients with painful mouth lesions. Teach the patient how to instill eye drops, ointments, or sustained-release capsules.
  • Advise the patient to avoid over-the-counter medications that include saliva-decreasing compounds, such as antihistamines, antidepressants, anticholinergics, and atropine derivatives.

Sources:
Marilyn Sawyer Sommers, RN, PhD, FAAN , Susan A. Johnson, RN, PhD, Theresa A. Beery, PhD, RN , DISEASES AND DISORDERS A Nursing Therapeutics Manual, 2007 3rd ed
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