Lynn M. Petruzzi and Frederick B. Vivino
AORN Journal, Denver, Colorado (March, 2003)

The signs and symptoms of Sjögren's Syndrome (SS), are divided into the hallmark signs and symptoms and the extraglandular signs and symptoms. The hallmark signs and symptoms also are called the Sicca Symptoms.

Xerostomia (i.e., dry mouth) and xerophthalmia (i.e., dry eyes) are the hallmark symptoms of SS. Xerostomia is the most prevalent and impairing symptom, although it often is intermittent early in the disease course.

Xerophthalmia may develop before, after, or simultaneously with xerostomia. Although xerostomia and xerophthalmia seem insignificant, both can have a profound affect on a patient's health and quality of life.

In Xerostomia, three pairs of major salivary glands (i.e., parotid, submandibular, sublingual) produce 95% of saliva. The remainder is produced by the minor salivary glands located inside the mouth. These glands are particularly numerous under the surface of the lips and palate.

In patients with SS, both the major and minor salivary glands are affected, resulting in a reduction of salivary flow. Some people with SS complain of a feeling of dryness. Others describe a cotton mouth sensation, difficulty in swallowing food, an inability to eat dry foods, changes in taste, discomfort or burning sensation in the mouth, or problems wearing dentures.

One of the earliest clinical signs of SS is a decreased sublingual salivary pool. The tongue and mucous membranes lose their glistening appearance and the buccal mucosa becomes sticky. Major salivary gland swelling, particularly in the parotid glands, occurs in episodes or as a chronic problem and can be quite painful.

As the disease progresses, xerostomia leads to multiple oral problems. The tongue becomes erythematous, fissured, or ulcerated. Dental caries develop and may progress rapidly.

Patients may exhibit an increased incidence of oral candidiasis and difficulty with phonation. Dysphagia (i.e., difficulty swallowing), an altered sense of taste, mouth burning, and an intolerance of acidic or spicy foods may develop and lead to weight loss or malnutrition. Lack of nocturnal saliva may cause sleep deprivation and trigger the onset of fibromyalgia.

Xerophthalmia, also known as keratoconjunctivitis sicca, results from a water deficiency of the tear film. The tear film is a three-layer structure consisting of the mucin, aqueous, and lipid layers. The aqueous layer produces 90% of tear volume. Ocular symptoms occur as tear volume diminishes.

Patients typically complain of a scratchy or gritty sensation in the eye. Eye redness, itching, and burning frequently are reported. Xerophthalmia symptoms vary considerably, but most patients notice an increase in dryness as the day progresses.

The eye also is affected by environmental conditions, such as low humidity, dust, and smoke. Diminished lacrimal production eventually can damage the comeal surface and lead to blurred vision, photosensitivity, and glare. Serious complications, such as infection, corneal ulceration, perforation, and vision loss, can result from undiagnosed or untreated xerophthalmia.

Extraglandular signs and symptoms. Extraglandular manifestations occur in up to one-third of patients with primary SS. Occasionally, the extraglandular symptoms may overshadow the sicca symptoms, and they also are the presenting manifestations of SS. Systemic involvement can lead to significant morbidity and mortality. In addition, other autoimmune or lymphoproliferative disorders may develop at any time in a patient with SS.

Fatigue is a common complaint of patients with SS and may have many causes. Active systemic inflammation is one cause and may be detected in the patient's blood test results.

Fatigue also may be related to poor sleep. Inadequate sleep may be caused by joint or muscle pain, nocturia due to large liquid consumption to relieve dryness, insomnia due to steroid use, or physiological sleep disorders.