Sjögren's syndrome (SS) is a chronic autoimmune exocrinopathy characterised by dryness of eyes (keratoconjunctivitis sicca) and mouth (xerostomia) as well as recurrent parotid or submandibular gland swellings. The clinical course of SS has been regarded as chronic, mild and stable. However, apart from the eVects on the lacrimal and salivary glands, various extraglandular manifestations may develop. Also, an increased risk of lymphoproliferative diseases, especially of non-Hodgkin's lymphoma, has been related to SS. 1 Scant attention has been paid to the prognosis and progression of mild, smouldering diseases and sicca symptoms. Forstot et al made a study of 45 patients with keratoconjunctivitis sicca with respect to oral and serological findings and observed SS in eight (17%).2 In a long term follow up study of 106 SS patients a subgroup of patients with isolated keratoconjunctivitis sicca was also described.
3None of the patients with isolated keratoconjunctivitis sicca developed lymphoproliferative disease.Our study was specifically designed to evaluate the prognosis of SS in the mildest forms of its clinical spectrum and to obtain further information on the natural history of primary SS (pSS). The main purpose was to identify the most useful clinical and immunological indicators of the subsequent development of SS in patients with early onset sicca symptoms.
Methods
SELECTION OF SUBJECTS WITH SICCA SYMPTOMS FOR RE-EVALUATIONThe charts of 259 subjects with sicca symptoms initially examined in the Department of Internal Medicine, Section of Rheumatology, in Tampere University Hospital during the years 1977 to 1986 were reviewed by one of the authors (MP). Californian criteria 4 with the modifications that salivary flow rate measurements were not performed and the histological findings were graded according to the Chisholm-Mason scale 5 grades 3 and 4 regarded as diagnostic, were applied in the diagnosis of SS. One hundred and nine patients with sicca symptoms fulfilling two or less of the criteria were included in the study. Of these, 14 subjects had died; the causes of death being cardiovascular in seven, malignancy in five, hepatic cirrhosis in one, and accident in one patient. The remaining 95 subjects were invited for re-evaluation. A total of 87 subjects (92% of those invited) attended the study. They comprised 72 women and 15 men with a median age of 60 years (range, 28-80 ) and a median follow up time of 11 years (range, 8-17).