Long‐term followup of patients with Sjögren's syndrome

Abstract

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.

Efficacy of Low-dose Prednisolone Maintenance for Saliva Production and Serological Abnormalities in Patients with Primary Sjoegren's syndrome.

“…Kruize et al (13) assessed the follow-up of 31 patients with primary Sjogren's syndrome for 10-12 years after initial diagnosis without systemic treatment. They reported that 3 patients developed malignant lymphomas and 4 patients developed new autoimmunediseases or extraglandular manifestations.…”

A Case of Lung Involvement Showing Multiple Lung Cysts in Primary Sjögren's Syndrome

“…Theander et al 14 did not find an increase of mortality in patients with lung involvement of Sjögren's syndrome compared with the general population. Kruize et al 15 reported that a long-term follow-up (10 to 12 years) of 29 patients with primary Sjögren's syndrome showed a mild and stable course of glandular and extraglandular manifestations and that none of them developed clinically significant pulmonary diseases. Although Sjögren's syndrome is a benign and non-life threatening disorder, patients should be managed with appropriate treatment in order to improve quality of life and to avoid complications.…”

Prevalence of Extraglandular Manifestations in Patients With Primary Sjögren’s Syndrome in Southern Norway: A Comparison to the Literature

“…Extraglandular organs may be complicated by the development of extraglandular involvement and organ-specific autoimmune disease. 3 As patients with pSS may have involvement of many organs beyond the exocrine glands, the clinical manifestation of pSS is divided as exocrine gland features and extraglandular manifestations (EGM), 4 the latter being experienced by a relatively high number of patients. [5][6][7] The development of other types of autoimmune diseases such as rheumatoid arthritis and systemic lupus erythematosus among patients with SS is also frequently observed.…”