Sjögren Syndrome

Gordon H. Sun and Allen M. Seiden

History

A 54-year-old white woman was referred to the otolaryngology clinic for evaluation of persistent dry mouth starting 4 months ago. She stated that she has difficulty swallowing solid foods without drinking large amounts of water. The patient had also had problems with dry eyes and has been using over-the-counter moisturizing eyedrops frequently. The patient denied pain with swallowing, reflux, or aspiration. Her voice had not undergone any recent change, although on rare occasion if her mouth was particularly dry, her voice might sound mildly hoarse. She also complained of chronically cracked, chapped lips. She denied joint and muscle pain, skin rashes, or hearing loss.

Her medical history was significant only for hypertension, which was well-controlled with hydrochlorothiazide and lisinopril. Family history was significant for multiple family members with arthritis and hypertension and a maternal aunt who was hospitalized for treatment of gastric ulcers at age 44. The patient was a nonsmoker and drank alcohol only rarely. Other than antihypertensives and eye lubricant, she was not using any other medications. She reported having seasonal allergies only.

On physical examination, the patient was afebrile, alert, and fully oriented. Her conjunctivae were mildly injected, although her vision was normal and extraocular movements appeared fully intact. The nasal passages were clear, although the mucosal lining appeared dry. Oral examination demonstrated very dry oropharyngeal mucosa and patchy red and white, mildly painful lesions overlying the palate. Two dental caries were found. The parotid glands were mildly enlarged but symmetric and nontender to palpation. No cervical lymphadenopathy or thyromegaly was appreciated. Flexible nasopharyngoscopy and laryngoscopy were unremarkable. Schirmer test and a lower-lip biopsy were performed, and blood work was obtained.

Differential Diagnosis—Key Points

1. In this patient, dysphagia is most likely related primarily to the patient’s xerostomia (dryness of the mouth). It is important to ascertain whether other medical problems might be contributing to dysphagia, such as an underlying neurologic disorder, gastroesophageal reflux disease, or lesions of the pharynx or esophagus.

3. The presence of xerophthalmos (dry eyes) should raise the index of suspicion for Sjögren disease. Tear-deficient dry eye is classified by ophthalmologists into Sjögren-related and non-Sjögren tear deficiency, the latter of which is generally associated with systemic medications, graft-versus-host disease, other autoimmune processes, or HIV infection. The Schirmer test measures aqueous tear flow and can be done rapidly in the office. Less than 5 mm of wetting of the test strip after 5 minutes is associated with a clinically significant decrease in tear production. Rose Bengal dye staining of the ocular surface may be able to detect ocular abnormalities such as corneal ulcerations.

4. Sometimes, but not always, patients with Sjögren disease present with associated complaints of myalgia or arthralgia. Only about 6% of Sjögren’s patients with myalgias demonstrate muscle enzyme elevation consistent with acute myositis. If present, the arthralgias are typically symmetric, although radiographically the only sign that is visible might be minimal joint-space narrowing, without apparent bony erosion.