Treatment:
Sjögren’s Syndrome
Treatment of Sjögren’s syndrome is aimed at symptomatic relief and limiting the damaging local effects of chronic xerostomia and keratoconjunctivitis sicca by substituting or simulating the missing secretions (Fig. 8-1 ).
To replace deficient tears, there are several readily available ophthalmic preparations (Tearisol; Liquifilm; 0.5% methylcellulose; Hypo Tears). If corneal ulcerations are present, eye patching and boric acid ointments are recommended. Certain drugs that may decrease lacrimal and salivary secretion such as diuretics, antihypertensive drugs, anticholinergics, and antidepressants should be avoided.
For xerostomia the best replacement is water. Propionic acid gels may be used to treat vaginal dryness.To stimulate secretions, pilocarpine (5 mg thrice daily) or cevimeline (30 mg thrice daily) administered orally appears to improve sicca manifestations, and both are well tolerated. Hydroxychloroquine (200 mg) is helpful forarthralgias.
TABLE 8-5
REVISED INTERNATIONAL CLASSIFICATION CRITERIA FOR SJÖGREN’S SYNDROME a‘b‘c
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I. |
Ocular symptoms: a positive response to at least one of three validated questions. |
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1. Have you had daily, persistent, troublesome dry eyes for more than 3 months? |
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2. Do you have a recurrent sensation of sand or gravel in the eyes? |
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3. Do you use tear substitutes more than three times a day? |
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II. |
Oral symptoms: a positive response to at least one of three validated questions. |
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1. Have you had a daily feeling of dry mouth for more than 3 months? |
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2. Have you had recurrent or persistently swollen salivary glands as an adult? |
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3. Do you frequently drink liquids to aid in swallowing dry foods? |
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III. |
Ocular signs: objective evidence of ocular involvement defined as a positive result to at least one of the following two tests: |
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1. Shirmer’s I test, performed without anesthesia (<5 mm in 5 min) |
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2. Rose Bengal score or other ocular dye score (>4 according to van Bijsterveld’s scoring system) |
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IV. |
Histopathology: in minor salivary glands focal lymphocytic sialoadenitis, with a focus score >1. |
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V. |
Salivary gland involvement: objective evidence of salivary gland involvement defined by a positive result to at least one of |
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the following diagnostic tests: |
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1. Unstimulated whole salivary flow (<1.5 mL in 15 min) |
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2. Parotid sialography |
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3. Salivary scintigraphy |
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VI. |
Antibodies in the serum to Ro/SS-A or La/SS-B antigens, or both |
aExclusion criteria: past head and neck radiation treatment, hepatitis C infection, AIDS, preexisting lymphoma, sarcoidosis, graft versus host disease, use of anticholinergic drugs.
hPrimary Sjögren’s syndrome: any four of the six items, as long as item IV (histopathology) or VI (serology) is positive, or any three of the four objective criteria items (items III, IV, V, VI).
cIn patients with a potentially associated disease (e.g., another well-defined connective tissue disease), the presence of item I or item II plus any two from among items III, IV, and V may be considered as indicative of secondary Sjögren’s syndrome.
FIGURE 8-1
Treatment algorithm for Sjögren’s syndrome.
Patients with renal tubular acidosis should receive sodium bicarbonate orally (0.5-2.0 mmol/kg in four divided doses).Glucocorticoids (1 mg/kg per day) and/or immunosuppressive agents (e.g.,cyclophosphamide) are indicated only for the treatment of systemic vasculitis. Anti-tumor necrosis factor agents appear ineffective, while anti-CD20 monoclonal antibody therapy in combination with a classic cyclophosphamide,doxorubicin, vincristine, prednisone (CHOP) regimen leads to increased survival in patients with lymphoma.
