FORT LAUDERDALE, FLA. — Sjögren's syndrome is the second most common autoimmune disorder that affects the musculoskeletal system, and yet the average time to diagnosis is 6 years, said Yvonne Sherrer, M.D., at a meeting sponsored by the Sjögren's Syndrome Foundation.
Although the cause of Sjögren's is still unknown, researchers suspect that a combination of genetic, environmental, and hormonal factors contribute to predisposition for the disease. Indeed, for every male with the syndrome, an estimated nine women are affected, underscoring the relevance of hormonal influences.
Inflammation of the exocrine glands, the common denominator of Sjögren's syndrome, most obviously affects the eyes, mouth, and vagina, said Dr. Sherrer, medical director and director of clinical research at the Centre for Rheumatology, Immunology, and Arthritis in Fort Lauderdale.
Typically, Sjögren's occurs in the context of a previously diagnosed autoimmune disorder, such as lupus, rheumatoid arthritis, or scleroderma.
The following less typical symptoms may also warrant suspecting Sjögren's syndrome:
▸Ocular. In addition to extreme dry eyes, patients may suffer from conjunctivitis, keratitis, blepharitis, ulcerations, and perforations.
▸Ears, Nose, and Throat. Tracheal dryness causes a chronic dry cough in some patients. Nosebleeds, otitis, and sinusitis can be recurring.
▸Oral. Severe dry mouth can cause swallowing problems, which may lead to malnourishment and excessive weight loss. Patients may also have accelerated caries, loss of dentition, and malfunctioning dentures.
▸Dermatologic/Vascular. Skin rashes are common, and skin eruptions and purpura may occur. Raynaud's phenomenon is a typical vascular manifestation. Vasculitis is always a concern in Sjögren's patients, but symptoms vary depending on the location of the inflammation in the body.
▸Gastrointestinal. Patients may suffer from esophageal dysmotility. In severe cases, they are at increased risk for pancreatitis, hepatitis, or atrophic gastritis.
▸Hematologic. Anemia, blood dyscrasias, and cryoglobulinemias are rare but may occur.
▸Pulmonary. Lung involvement and coronary involvement are rare but can develop due to dryness of bronchial tubes. Other potential manifestations include bronchitis, bronchitis obliterans-organized pneumonia, and interstitial fibrosis.
▸Neurologic. Neuropathies tend to be less symmetrical in Sjögren's patients, compared with other conditions. Central nervous system disorders might manifest as changes in cognitive function or as seizures.
▸Musculoskeletal. More often than not, patients with Sjögren's have arthralgia, rather than arthritis, but secondary Sjögren's patients may have concurrent arthritis or myositis.
Criteria for Primary Sjögren's Syndrome
The diagnosis of primary Sjögren's syndrome requires that patients meet at least four of the following six criteria:
1. The patient must have at least one of three ocular symptoms:
▸Dry eyes for less than 3 months.
▸Need to use artificial tears more than three times daily.
▸Sensation of a foreign body in the eye.
2. The patient must have at least one of three oral symptoms:
▸Persistent dry mouth for more than 3 months.
▸Swollen salivary glands.
▸Need to add extra liquid to the mouth in order to swallow.
3. The patient must have at least one of two ocular signs:
▸Unanesthetized Schirmer's test result of 5 mm/5 minutes or less in both eyes.
▸Positive vital dye staining.
4. The patient must have at least one of three signs of poor salivary gland function:
▸Abnormal salivary scintigraphy.
▸Abnormal parotid sialography.
▸Unstimulated salivary flow rate of 0.1 mL/minute or less.
5. Positive lip biopsy.
6. Positive anti-SSA or anti-SSB tests.
Source: “The New Sjögren's Syndrome Handbook” (New York: Oxford University Press, 2005)