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., said at a meeting sponsored by the Sjögren's Syndrome Foundation.
Until more reliable biomarkers for the syndrome are identified, expediting the diagnosis may require physicians to have a higher level of suspicion for some of the less common manifestations of Sjögren's, said Dr. Sherrer, medical director and director of clinical research at the Centre for Rheumatology, Immunology, and Arthritis in Fort Lauderdale.
Although the cause of the 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, Dr. Sherrer said.
Typically, Sjögren's occurs in the context of a previously diagnosed autoimmune disorder, such as lupus, rheumatoid arthritis, or scleroderma.
The atypical patients with primary Sjögren's “are our most challenging patients, because they don't have an accompanying autoimmune disease,” Dr. Sherrer said. These patients generally present with neuropathy, accelerated dental caries, salivary gland swelling, joint pain without overt arthritis or myositis, and corneal melt. The current international criteria for diagnosing Sjögren's require that patients demonstrate some objective evidence of autoimmunity, in addition to other symptoms. (See box.)
However, 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 problems.
▸ 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. In addition, Sjögren's patients are at increased risk for lymphoma.
▸ 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.
▸ Renal. Renal involvement is rare, but when it occurs, it is usually interstitial tube involvement and is more likely to be chronic, compared with highly progressive kidney involvement associated with lupus.
▸ 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.
▸ Other. Fibromyalgia occurs in 20%-30% of Sjögren's patients. Sleep disorders, often due to dryness-related discomfort, are common and may contribute to the fibromyalgia. Depression is a common comorbidity, as it is in patients with other autoimmune disease.
Recommended Diagnostic Criteria
The diagnosis of primary Sjögren's syndrome requires that patients meet at least four of the following six criteria:
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.
The patient must have at least 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.
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.
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.
Positive lip biopsy.
Positive anti-SSA or anti-SSB tests.
Source: “The New Sjögren's Syndrome Handbook” (New York: Oxford University Press, 2005)