This patient presents with the constellation of symptoms referred to as keratoconjunctivitis sicca: dry mouth and dry eyes. Her right parotid gland also is enlarged. After further evaluation, she was diagnosed with Sjögren's syndrome.
A chronic inflammatory condition, Sjögren's syndrome is characterized by exocrine and nonexocrine involvement. Originally described by Johann Mikulicz, MD, in 1892, it was ultimately named for Henrik Sjögren, MD, a Danish ophthalmologist who introduced the term keratoconjunctivitis sicca in 1933. The condition may be primary or secondary to another autoimmune disorder such as rheumatoid arthritis, systemic lupus erythematosus, or scleroderma.
Patients may present with limited exocrine involvement like the patient above or with diverse multisystem features. The most commonly involved exocrine manifestations involve the lacrimal and salivary glands. Patients often complain of dry, irritated eyes, a feeling of grittiness, or a foreign body sensation in the eyes. Complications to the eyes include corneal ulcers and infections of the eyelids. Salivary gland dysfunction results in dry mouth, difficulty swallowing solids without the aid of liquids, and difficulty speaking for long periods of time. Dental caries, gingival recession, oral candidiasis, and chronic esophagitis may subsequently occur. Enlargement of the salivary glands is not uncommon, and may be episodic or chronic.
With involvement of exocrine glands in the upper airways, patients may present with rhinitis, sinusitis, and chronic cough. Because of the bronchial dryness that may result, patients with Sjögren's syndrome are particularly prone to mucus plugging during upper respiratory infections, and should avoid the use of anticholinergic medications. Women also may complain of vaginal dryness and dyspareunia.
Nonexocrine manifestations may occur in almost any organ system. Skin complaints of dryness or pruritus without any visible rash are common. Skin changes may be seen secondary to vasculitis. Patients develop palpable or nonpalpable purpura of the lower extremities. Other cutaneous changes secondary to vasculitis include erythematous plaques, urticaria, ulcers, and erythema multiforme-like eruptions.
Pulmonary involvement is often asymptomatic, but results in a nonspecific interstitial pneumonitis. Plain films may reveal basilar changes and hyperinflation, but CT scanning is more sensitive for visualizing the interstitial and ground glass opacities that occur. Patients may present with acute symptomatic pericarditis usually with no significant effusion. GI and hepatic involvement may result in chronic atrophic gastritis, primary biliary cirrhosis, and chronic active hepatitis. Renal involvement presents with an interstitial nephritis or glomerulonephritis.
Peripheral and central nervous system involvement is of particular importance because some of the potentially serious complications may be treated with immunosuppressive medications. Peripheral neuropathies usually present with pure sensory deficits. Cranial nerve involvement may present with Bell's palsy, sensorineural hearing loss, or diplopia. Central nervous system involvement may range from focal lesions to diffuse brain lesions. Spinal cord involvement may present with acute transverse myelopathy. Not surprisingly, patients with nervous system involvement may be misdiagnosed as having multiple sclerosis.
Other musculoskeletal manifestations include arthralgias, arthritis, and myositis. Besides these complications, patients with Sjögren's syndrome are at increased risk for malignant lymphoproliferative disorders, in particular monoclonal proliferation, non-Hodgkin's lymphoma, and extranodal marginal zone B cell lymphoma of MALT type.
In the literature, there are a number of classification systems proposed for diagnosis. In general, the diagnosis is based on various clinical and laboratory findings as well as excluding other potential causes. Clinical symptoms should include the classic oral and ocular symptoms described. Objective evidence of dry eyes can be determined by performance of the Schirmer test. This test uses sterile filter paper, and measures tear production over five minutes. Salivary glands can be objectively studied by doing a labial salivary gland biopsy and salivary gland scintigraphy. Other tests include parotid sialography, sialometry, MRI, and ultrasound. Finally, serologic testing for autoantibodies to Sjögren's SS-A (Ro) and/or SS-B (La) may be performed.
Treatment clearly depends on the organ systems involved and the severity of symptoms. Multiple specialists in conjunction with rheumatology may be required. Most patients initially present with sicca symptoms, which can be initially treated by the emergency physician. Stimulation of salivary flow with dried fruit slices or sugarless citrus-flavored candies and replacement of oral secretions with frequent sips of water should be advised. Particular emphasis should be paid to good oral hygiene, and artificial tears can be prescribed for dry eyes. Drugs with anti-cholinergic properties should be avoided. Finally, appropriate follow-up with rheumatology and ophthalmology should be arranged.