The majority of individuals ultimately diagnosed with SS initially present with dry eyes and/or dry mouth. A few will present with the result of a secretory deficiency such as increased dental decay at incisal edges or exposed root surfaces of the teeth, oral mucosal burning secondary to a fungal infection, and corneal ulceration or corneal scarring. Dryness complaints may be accompanied by involvement of a diverse array of other organ systems: Hashimoto's thyroiditis/Graves' disease, severe fatigue, increased sedimentation rate, fever of unknown origin, myositis, peripheral neuropathy, leukocytoclastic vasculitis-purpura, renal tubular acidosis, atrophic gastritis, arthritis, and positive test for antinuclear antibodies or RF in asymptomatic patients (2,5,6).
Ocular manifestations are associated with lacrimal gland dysfunction. Many patients with SS cannot cry. The symptoms of ocular dryness may manifest as itching, grittiness, a sandy sensation, photophobia, eye fatigue, decreased visual acuity, discharge, a sensation of film across the eye, photosensitivity, and conjunctival injection. Ocular symptoms may be exacerbated by air-conditioning, computer use, cigarette smoke, low humidity, and medication with anticholinergic effects. It should be noted that tear flow rates are often not correlated with ocular discomfort. That is, individuals with objective signs of severe dry eyes may have minimal complaints. Because tears contain antimicrobial components, the loss of tears predisposes to ocular infections, including blepharitis, bacterial keratitis, and conjunctivitis, which can further exacerbate the sensation of dryness. In addition to the decreased volume of tears, there is a change in the mucous component. Filamentary keratitis can occur with mucous filaments adhering to damaged sections of the ocular surface. Ocular complications include corneal ulceration/abrasion, corneal melt, vascularization, opacifi-cation, and, rarely, perforation with loss of vision. Enlargement of the lacrimal glands rarely occurs.
Oral manifestations are associated with salivary gland dysfunction (7). The patient may complain of difficulty in eating dry foods (e.g., crackers), oral soreness, hoarseness, lipstick adhering to the front teeth, increased dental decay, swollen salivary glands, and dry mouth. There will often be a history of carrying a water bottle and of needing fluid at mealtimes to aid swallowing. It is estimated that salivary gland swelling will occur in only 25% of those diagnosed with SS. Interestingly, the clinical oral examination can be within normal limits, as some individuals eventually diagnosed with SS will have normal or near-normal salivary flow rates. The oral findings will be distinctive in those with the most severe salivary gland dysfunction, including depapillation, fissuring, and erythema of the dorsal tongue, and/or generalized mucosal erythema, with or without burning symptoms (fungal infection); cloudy and/or viscous saliva expressible from the parotid or submandibular/sublingual ducts; multiple carious lesions located at the incisal or root surfaces of teeth; angular cheilitis; and a lack of salivary pooling in the floor of the mouth. There may be a "clicking" sound heard during speech, due to the tongue sticking to teeth.
While dryness is known to occur in the eyes and mouth, it can also occur in other organ systems including skin, vagina, ear, nose (blockage, dryness, and epistaxis are common; crusting and hyposmia are rare), and throat (hoarseness; chronic, nonproductive cough). A recent paper on otolaryngological examinations of 111 patients with primary SS and 26 patients with secondary SS reported that symptoms were common, but objective observations were rare and included dry mucosa, postnasal drip, and middle ear effusion (8). Sensorineural hearing loss of high-frequency hearing may be present and associated with autoimmunity, but is considered to be of limited clinical impact in SS (8-10).
Extraglandular manifestations of SS involve numerous organ systems listed in Table 5. These may be treated separately without recognition of the underlying process. The diversity of the manifestations, perhaps coupled with an insidious onset of the sicca complaints, may help explain delays in SS diagnosis.
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