Objective. To assess long‐term outcome in patients with isolated keratoconjunctivitis sicca (KCS), primary Sjögren's syndrome (SS), and secondary SS. Methods. In 112 patients referred because of dry eyes, an ophthalmologic diagnosis of KCS was made based on results of the Schirmer I test, the tear fluid lysozyme concentration, and rose bengal staining. Subsequent assessments, including sublabial salivary gland biopsy, were performed. Followup assessments were performed 10–12 years after initial diagnosis. Results. Six patients were excluded because no biopsy specimen was available. Seventy‐three percent of the remaining 106 patients were female, with a mean age of 53.5 years and a mean symptom duration of 3.9 years. Application of the 1987 classification criteria of Daniels and Talal revealed a diagnosis of isolated KCS in 56 patients, primary SS in 31, and secondary SS in 19. At baseline, 2 of 56 patients with isolated KCS and 8 of 31 with primary SS exhibited mild features of organ‐specific autoimmune disease. At followup, 2 of 38 patients with isolated KCS and 4 of 21 with primary SS had developed new features related to autoimmune disease, not necessitating treatment with corticosteroids; none of the patients developed major glandular complications. Three of 30 patients with primary SS died of malignant lymphoma. In 1 of these patients, the possibility could not be excluded that sicca symptoms and infiltrates seen on sublabial salivary gland biopsy had occurred concomitantly with early stages of lymphoma. Malignant lymphoma did not develop in any of the patients with isolated KCS or secondary SS. Conclusion. Primary Sjögren's syndrome is characterized by a stable and rather mild course of glandular and extraglandular manifestations, in marked contrast to the increased risk for development of malignant lymphoma in these patients. Since patients with isolated KCS do not have an increased risk for development of malignant lymphoma, a presumptive diagnosis of primary SS should be confirmed in patients with sicca syndrome.

Aims: To determine the prevalence of plasma cell monotypia in labial salivary gland tissue of patients with and without

Objective. The aim of the present study was to evaluate the diagnostic value of quantitative immunohistologic criteria for Sjögren's syndrome (SS) in labial salivary gland biopsies. Methods. Quantitative immunohistologic examination was performed on labial salivary gland biopsy samples from 80 healthy controls, 32 patients with primary SS, 14 patients with secondary SS, 5 with “probable” SS, 36 with keratoconjunctivitis sicca (KCS) with a lymphocytic focus score <1 on the lip biopsy, and 18 with rheumatoid arthritis (RA) without clinical evidence of SS. Results. This is the first study to show that immunohistologic criteria for SS, based on the percentages of IgA‐containing and IgG‐containing plasma cells, are able to 1) confirm the diagnosis of SS in labial salivary glands of KCS patients in the absence of grade IV lymphocytic adenitis; and 2) distinguish between a grade IV focal lymphocytic adenitis in the labial salivary glands of SS patients and of RA patients without SS. Conclusion. Quantitative immunohistologic criteria were shown to be much more sensitive and disease specific than the widely accepted grade IV lymphocytic adenitis criterion, which corresponds to a lymphocytic focus score >1, and these criteria should be included in the international diagnostic criteria for Sjögren's syndrome.

Objective. Salivary glands of patients with Sjögren's syndrome (SS) have been shown to be a site of anti‐SS‐B/La antibody production. The present study investigated differences in the localization of the SS‐B/La antigen in labial salivary gland (LSG) tissue between SS and non‐SS patients, which may explain the local antigen‐driven anti–SS‐B/La response. Methods. Distribution of SS‐B/La was studied immunohistologically in the LSG biopsy samples of 9 SS patients, 10 non‐SS patients, and in normal tissues obtained at autopsy within 2 hours after death, using a mouse monoclonal antibody directed to SS‐B/La. In 3 SS and 3 non‐SS patients, LSGs were also studied with affinity‐purified biotinylated human antibodies directed against SS‐B/La. Results. In the non‐SS patients, SS‐B/La was primarily observed in the nucleoli of acinic cells of the LSGs. Patients with either primary SS or secondary SS showed an accumulation of SS‐B/La in the nucleoplasm of acinic cells. In the SS patients, SS‐B/La was also detected in the cytoplasm as a diffuse or perinuclear staining. Sometimes, SS‐B/La was found along the membrane of acinic cells as well. This aberrant nuclear and cytoplasmic distribution of SS‐B/La in SS patients correlated well with abnormalities in the composition of the plasma cell population in the LSGs, but not with a lymphocytic focus score > 1. Conclusion. The accumulation and redistribution of SS‐B/La in the LSGs may play an important role in the local antigen‐driven anti–SS‐B/La response in SS, and can also be used to improve the diagnostic possibilities of the LSG biopsy.

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