Background Primary Sjögren’s syndrome(pSS) is autoimmune disease, characterized with sicca symptoms and systemic involvement. Pulmonary involvement is main extraglandular feature. Pulmonary arterial hypertension(PAH) is frequently seen in many connective tissue diseases, mainly in systemic sclerosis.
Objectives To evaluate the frequency of pulmonary arterial hypertension in patients with primary SS and possible correlation with clinical and laboratory findings.
Methods 47 pSS patients diagnosed according to AECG criteria were included in the study. All of the patients were clinically and laboratory evaluated. Echocardiography was performed to evaluate pulmonary hypertension and cardiac involvement. Pulmonary arterial hypertension was defined as pulmonary arterial pressure(SPAP) >30mm Hg with the patient at rest.
Results 47pSS patients were included in the study. Of all patients, 2 were males and 45 were females. Mean age was 48 years and the mean disease duration was 5.5 years. Pulmonary arterial hypertension was recorded in 11(%23.4) patients. Among 11patients, five patients had SPAP>35mmHg, six patients had SPAP 30-35mmHg. All of 11 patients with PAH were females.
Conclusions Our study showed that pulmonary hypertension are highly frequent among patients with pSS. More investigations are needed to support our findings.
Gardiner P, Ward C, Allison A, Ashcroft J, Simpson W, Walters H et al (1993) Pleuropulmonary abnormalities in primary Sjogren’s syndrome. J Rheumatol 20:831–837.
Sato T, Matsubara O, Tanaka Y, Kasuga T (1993) Association of Sjogren’s syndrome and primary pulmonary hypertension. Report of two cases and review of the literature. Hum Pathol 24:199–205.
Chisholm DM, Mason DK (1968) Labial salivary gland biopsy in Sjogren’s disease. J Clin Pathol 21:656–660.
Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE et al (2002) Classification criteria for Sjogren’s syndrome: a revised version of the European criteria proposed by the American–European Consensus Group. Ann Rheum Dis 61:554–558.
Disclosure of Interest None Declared