Nutritional deficiency

1. Starvation.

2. Vitamin B6 deficiency.

3. Vitamin C deficiency.

4. Vitamin A deficiency.

F. Drugs associated with the development of dry mouth

1.

Sedatives.

2.

Hypnotics.

3.

Narcotics.

4.

Phenothiazines.

5.

Atropine.

6.

Propantheline.

7.

Antiparkinsonian drugs

8.

Antihistamines.

9.

Ephedrine.

10.

Epinephrine.

11.

Amphetamines.

VII. Treatment. No controlled trials have been undertaken in the therapy of the systemic manifestations of SS. In general, the approach to treatment has been empiric. Vasculitis, renal disease (immune complex-mediated or otherwise), myositis, alveolitis, cryoglobulinemia, and other manifestations have mostly been treated in the past with corticosteroids, immunosuppressive agents, or both, depending on the severity of disease. Because the cytotoxic drugs are immunosuppressive and renal transplant patients and others treated with the agents have an increased incidence of lymphoma (as do untreated SS patients), it is advisable to avoid their use in SS.

A. Ocular abnormalities. Artificial tears are the mainstay of treatment. Solutions consisting of methylcellulose and polyvinyl alcohol are useful. The dosage varies from two drops four times daily to every 15 minutes depending on the clinical state. Inserts placed into the conjunctival sac that release small amounts of methylcellulose during many hours are presently available and may be beneficial in some patients. Lacrimal duct occlusion, temporary or permanent, may enhance local moisture. Bromhexine, a secretagog, is presently being studied in clinical trials for use in the United States.

B. Xerostomia. Salagen (pilocarpine hydrochloride) tablets are indicated for the treatment of symptoms of xerostomia from salivary gland hypofunction. As a cholinergic parasympathetic agent, pilocarpine can increase secretion of the salivary glands. The usual initial dose is 5 mg tid. It is contraindicated in patients with asthma, iritis, and narrow angle glaucoma. Side effects include abdominal cramps and sweating. Lubrication of the mouth and mild secretagogs, such as lemon-flavored juice or lubricating agents proper (water, methylcellulose), are useful. More potent secretagogs may exacerbate the signs and symptoms of parotitis. Prevention of states of dehydration in SS is very important, as dehydration may enhance the formation of parotid ductal calculi. Avoidance of drugs that may aggravate oral dryness (e.g., narcotics, antihistamines, anticholinergics) is also important.

C. Parotid enlargement. In the past, numerous modes of therapy have been employed to treat parotid enlargement.

1. Parotid irradiation has been associated with an increased incidence of lymphoma in SS patients. Because of its oncogenic potential, this form of therapy should not be used in SS.

2. Surgical removal is often technically difficult, and resultant nonhealing fistulae and facial nerve damage preclude this mode of therapy.

3. Drug therapy for symptomatic inflammatory parotid enlargement a. Nonsteroidal antiinflammatory drugs. Useful regimens include 25 to 50 mg of indomethacin four times daily or 400 mg of ibuprofen four times daily.

b. Corticosteroid therapy should be limited to those with severe, recalcitrant disease because of the risk for corticosteroid toxicity. The regimen is 20 to 40 mg/d with dosage tapering as soon as a clinical response is obtained.

c. Oral pilocarpine therapy has been recently approved by the Food and Drug Administration for use in SS. It may be helpful in patients with sicca symptomatology in dosages of 5 mg three or four times daily (see sectjoniV||:iB:above).

d. Hydroxychloroquine has also been studied in SS, and its effects have been very modest in treating sicca symptoms or fatigue symptoms.

4. Cytotoxic therapy (specifically azathioprine, cyclophosphamide, chlorambucil, methotrexate) does not seem to offer any significant benefit unless one is treating a true malignancy (lymphoma) or an invasive form of pseudolymphoma in SS.

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