fever. Thus, it is more critical than ever that rheumatologists educate their patients and other specialists about the therapy of SS and its wide spectrum of extraglandular manifestations, diagnostic procedures, and therapeutic approaches. Further, rheumatologists need to further educate themselves about the procedures used by other specialists.

Although we are advocates of evidence-based medicine, it should be recognized that most of the studies in therapy of extraglandular manifestations of SS are largely based on small series of patients from single institutions or small multicenter open-label trials. Thus, this chapter represents the current approach we use for treatment

of patients at our SjšgrenÕs clinic. Fortunately, an international consortium of rheumatologists and other SS specialists are developing a standardized disease activity/damage index that will greatly facilitate the uniform standards of diagnosis, therapy, and prognosis.

18.2Treatment and Management of Cutaneous Manifestations

18.2.1 Treatment of Dry Skin

Treatment of dry skin in SjšgrenÕs syndrome is similar to managing xerosis in other conditions.

1.The patient should moisturize with a fragrance-free cream moisturizer once or twice a day. Moisturizing is performed immediately after bathing or showering, while the skin is still damp, to prevent further evaporation from the skin.

Sometimes in cases of extreme dryness, an ointment is suggested for its barrier and protective properties (such as petrolatum jelly or Aquaphor).

If ointment is used, then application should be to damp skin because the ointment itself does not contain water.

Excess greasiness can be blotted with a towel.

Sometimes a moisturizing cream with β-hydroxy acid or α-hydroxy acid or urea can add extra moisture, but in cases of cracks in the skin, these will sting and irritate.

2.Excessive, long, hot showers or baths should be avoided in addition to heavily fragranced cleansers.

3.Cleansing of the skinÑThe usual recom-

mendation is to cleanse with a moisturizing soap such as Dove R fragrance-free bar or a soap-free cleanser such as Cetaphil R gentle cleanser or Aquanil R cleanser.

If the xerosis leads to pruritis, then safe anti-pruritus topical treatments are recommended. The therapeutic approach is similar to contact dermatitis or eczema. Topical steroids and immodulary creams or sprays are initially preferred. Mirtazapine also has some anti-pruritic effect to its strong antagonism of the H1 receptor.

4.Over-the-counter lotions containing menthol, camphor (Sarna Anti-Itch Lotion R ),

2% lidocaine (Neutrogena Norwegian

Formula Soothing Relief Anti-Itch Moisturizer R ), and pramoxine (Aveeno Anti-Itch Concentrated Lotion R ) are readily

available.

5.Oral anti-histamines should be used with caution because of their anti-cholinergic effects. Fexofenadine (Allegra) does not cross the bloodÐbrain barrier and may have slightly less dryness as a side effect. Over-the-counter sleeping medications that

contain hydroxyzine (Atarax) or diphenhydramine (Benadryl) are very drying and may contribute to sleep disturbance.

6.Topical corticosteroidsÑWe generally do not like to use topical corticosteroids (especially on the face) for more than a couple of weeks at a time, especially the ultrapotent ones, but even the mid-potent ones. In the case of inßammatory skin Þndings, local treatment with potent topical steroids can augment systemic treatments.

Sometimes topical corticosteroids are used for pruritis, but their use should be limited due to long-term side effects such as skin atrophy, tachyphylaxis, and absorption.

7.We always suggest constant daily sun protection for patients with autoimmune conditions. Because the wavelength of light causing sun sensitivity in autoimmune conditions may not be in the UVB spectrum (290Ð320 nm), patients should use a broadspectrum sunscreen.

¥SPF factors refer to UVB protection only, so patients cannot count on simply the SPF factor.

¥Most sunscreens available now have added UVA protection (290Ð320 nm), commonly from chemical UVA-absorbing compounds such as Parsol 1789 (avobenzone).

8.We prefer physical sun blocks because wavelengths outside of both UVB and UVA may affect the patient with autoimmune disease.

Physical sun blocks contain titanium dioxide or zinc oxide, which reßects rays.

One commonly available sun block is

Neutrogena Sensitive Skin Sun Block SPF30 R , which uses purely titanium dioxide as

its active ingredient.

9.The most effective protection is sun protective clothing because it will not wear off as sunscreens do.

Obviously, avoiding excess sun contact altogether is prudent, such as trying to stay indoors during the intense sunlight hours of 10:00 a.m.Ð4 p.m.

10.Routine skin checks for skin cancersÑIn addition to the hunt for actinic keratosis