Sjogrens syndrome is an autoimmune exocrinopathy that manifests as a decrease in lacrimal and salivary gland function. This disorder is most common in middle-aged women. Sjogrens syndrome is usually a slowly progressing disease with 8 to 10 years elapsing from the onset of symptoms to full-blown disease. It is the second most common autoimmune disorder behind rheumatoid arthritis. It may occur as a primary disease or be a secondary phenomenon to some other disorder.
Keratoconjunctivitis sicca (KCS) refers to the destruction of the corneal and bulbar conjunctival epithelium which results in the clinical symptoms of burning, decreased tearing, redness, pruritus, fatigue, and ocular photosensitivity. The Schirmer test is used to assess tear flow while either the tear break-up time or tear lysozyme content are used to assess composition. The Schirmer test is performed by absorbing tears on filter paper, and absorption of less than 5 mm in 5 minutes is considered consistent with Sjogrens syndrome. A more specific study is slit-lamp examination after Rose Bengal or lissamine green staining. Punctate corneal ulcerations and attached filaments of corneal epithelium are consistent with the diagnosis of Sjogrens syndrome.
Xerostomia results from salivary gland involvement and manifests as dry mouth, burning oral sensation, dental caries, thickened saliva, and impaired oropharyngeal manipulation of dry foods. Physical exam often reveals firm, nontender enlarged submandibular and parotid salivary glands. Tests used to assess salivary gland involvement include salivary flow measurements, parotid sialography and salivary scintigraphy. Biopsy of salivary glands often reveals sialadenitis.
Systemic manifestations occur less frequently and include involvement of the lungs, kidneys, blood vessels, muscles, and reticuloendothelial system. Raynauds phenomenon is noted in up to 35% of cases of Sjogrens syndrome.
Laboratory abnormalities are nonspecific and include an elevated ESR and polyclonal hypergammaglobulinemia on serum protein electrophoresis (SPEP). Antinuclear antibodies(ANA) are found in approximately 90% of cases, with rheumatoid factor (RF) being present less frequently in only about 60% of cases. Antibodies to anti-Ro (SSA) and anti-La (SSB) are found in 55% and 40% of patients, respectively.
Therapy is usually supportive. Avoiding tobacco smoke and abstaining from using tobacco is helpful. Room humidifiers may prove helpful. Eye patching, artificial tears, nocturnal ocular ointments, Lacriserts, and punctal occlusion are available therapies for KCS. Lacriserts are a pellet form of a lubricant that moisturizes the eye when wettened with artifical terars. Topical steroids or cyclosporin A drops can be added when the eye is inflamed. Xerostomia may be relieved with either pilocarpine or cevimeline taken three to four times daily. Regular dental visits, meticulous dental care, flouride containing toothpastes, and low sugar diets are advised for proper oral care. Apply emollients to skin when moist frequently throughout the day for skin care. Vaginal dryness may be alleviated with propionic acid gels. Hydroxychloroquine is another potential treatment modality for Sjogrens syndrome. When systemic complications (renal, pulmonary, vasculitic, etc.) predominate, immunosuppressive agents and steroids should be employed. Associated disorders include bronchitis, tracheobronchitis, lymphocytic interstitial pneumonitis, pleurisy, type 1 RTA, peripheral neuropathy, cranial neuropathy, hepatitis, cirrhosis, primary biliary cirrhosis, vasculitis, Raynaud's phenomenon, lymphoma, and thyroid disorders (both Grave's disease and Hashimoto's thyroiditis. Rheumatology consultation may helpful in caring for this disease.