SjÖgrens Syndrome
The next most common autoimmune disorder after rheumatoid arthritis, Sjögren’s syndrome results from a chronic exocrine gland dysfunction. The syndrome is marked by diminished lacrimal and salivary gland secretion (sicca complex). The mean age of occurrence is 50 and about 90% of those affected are females.
Sjögren’s syndrome may be a primary disorder or may be associated with inflammatory connective tissue disorders, such as rheumatoid arthritis (about 50% of patients), scleroderma, systemic lupus erythematosus, primary biliary cirrhosis,
Hashimoto’s thyroiditis, polyarteritis, and interstitial pulmonary fibrosis.
Hashimoto’s thyroiditis, polyarteritis, and interstitial pulmonary fibrosis.
Nephritis (seldom leading to chronic renal failure) may affect up to 40% of patients with primary Sjögren’s syndrome and may result in renal tubular acidosis in about 25% of patients.
Patients with Sjögren’s syndrome may also have Raynaud’s phenomenon (about 20%) and vasculitis (usually limited to the skin; they may be systemic or localized in the legs). Sensory polyneuropathy and biochemical hypothyroidism (resembling Hashimoto’s thyroiditis) occur in up to 50% of patients. Rarely, systemic necrotizing vasculitis develops and involves the skin, peripheral nerves, and GI tract.
Overall, the prognosis for a patient with Sjögren’s syndrome is good.
Causes
No one knows what causes Sjögren’s syndrome. However, researchers think that genetic and environmental factors may contribute to its development. Viral or bacterial infection—or possibly exposure to pollen—may trigger this disease in a genetically susceptible person. Tissue damage results from infiltration by lymphocytes or deposition of immune complexes. Lymphocytic infiltration may be classified as benign lymphoma, malignant lymphoma, or pseudolymphoma (nonmalignant but tumorlike aggregates of lymphoid cells).
Complications
The disease seldom produces significant complications.
Ocular complications include corneal ulcerations or perforations, ocular vascularization, infection, or symblepharon. Oral complications include infection, ulceration, and dental complications. Pulmonary and respiratory complications include dry nasal mucosa with epistaxis, hoarseness, dysphagia, deafness, otitis media, and lower respiratory tract complications. GI complications include splenomegaly, decreased gastric acid, constipation, and pancreatic and hepatic insufficiency. Renal complications include renal tubular necrosis, aminoaciduria, glycosuria, and hypergammaglobulinemia purpura. Other complications include leukopenia, vasculitis with leg ulcers, chronic thyroiditis, and lymphoproliferative disease.
Assessment
The patient typically reports slowly developing dryness affecting the eyes (xerophthalmia), the mouth (xerostomia), and other organs. Initially, she may describe a foreign body sensation in the eye (gritty, sandy eye) along with redness, burning, photosensitivity, eye fatigue, itching, and mucoid discharge. She may also complain of a film across her eyes.
With oral dryness, the patient may report difficulty swallowing and talking, an abnormal taste or smell sensation (or both), thirst, severe dental caries, and ulcers of the tongue, mouth, and lips (especially at the corners of the mouth).
With other dryness—of the respiratory tract, for example—the patient may report epistaxis, hoarseness, chronic nonproductive cough, recurrent otitis media, and frequent respiratory tract infections. With vaginal dryness, the patient may report dyspareunia and pruritus.
Additional complaints include generalized itching, fatigue, recurrent low-grade fever, and arthralgia or myalgia.
Inspection may disclose mouth ulcers, dental caries and, possibly, enlarged salivary glands.
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