Sjogren's Syndrome: I. SICCA COMPONENTS

A detailed and comprehensive study of the sicca components of171 patients with Sjøgren's syndrome is described. Seventy-onepatients had Sjøgren's syndrome unassociated with rheumatoidarthritis or other connective tissue or rheumatic diseases,and 94 patients had Sjøgren's syndrome and rheumatoidarthritis, four patients had Sjøgren's syndrome and systemiclupus erythematosus, one patient had Sjøgren's syndromeassociated with progressive systemic sclerosis, and one patienthad Sjøgren's syndrome associated with psoriatic arthritis.One hundred and fifty-three patients were female, and the meanage was 57.2 years. Symptoms of keratoconjunctivitis sicca were in the majorityof patients relatively mild, and in none of the 94 patientswith rheumatoid arthritis was the diagnosis made by the referringphysician. Response to artificial tear drops and/or electrocoagulationof the naso-lacrimal duct was satisfactory in controlling ocularsymptoms in 90 per cent of patients. Ocular complications, includingcorneal pannus formation, perforating corneal ulcers, and externalsymblephora, were encountered among the remaining 10 per centof patients not adequately relieved by artificial tear dropsand/or electro-coagulation of the naso-lacrimal junction. Lacrimalgland enlargement was present in seven patients, one of whomsubsequently developed a lymphoma out with the lacrimal andsalivary glands. Xerostomia was clinically evident in only half the patients,and less than half the patients had an increase in fluid intakeand oral soreness and ulceration. Parotid gland enlargementoccurred in approximately one-quarter of the patients studied,and was more commonly unilateral and episodic. Secondary bacterialinfection occurred in one patient. Treatment of severe xerostomiaproved extremely difficult. The most sensitive index of salivary gland disease was salivaryflow determinations, followed by sialography, labial salivarygland biopsy, and radioactive technetium scanning of the salivaryglands promises to be a valuable diagnostic adjunct. Involvement of the nasal passages, pharynx, and trachea werecommon and oesophageal webs were found in four patients. Drynessof the skin was uncommon, and with one exception mild. Biopsyof the skin of the patient with severe skin dryness revealedplasma and lymphocyte infiltration around the sweat glands.Vaginal dryness was present in less than 5 per cent of patientsand in no patient gave rise to troublesome symptoms.