ORIGINAL ARTICLE Survival analysis of patients with Sjögren’s syndrome in Turkey: a tertiary hospital-based study Veli Yazisiz 1 & Mesut Göçer 2 & Funda Erbasan 1 & İsmail Uçar 1 & Bengisu Aslan 1 & Şuayp Oygen 1 & Edip Gökalp Gök 1 & Mustafa Ender Terzioğlu 1 Received: 19 June 2019 /Revised: 5 August 2019 /Accepted: 6 August 2019 # International League of Associations for Rheumatology (ILAR) 2019 Abstract Objectives This study was conducted to determine long-term survival rates and the factors associated with mortality in Turkish primary Sjögren syndrome (pSS) patients. Methods All patients diagnosed with pSS between 2004 and 2014 were included in this study. By January 2019, all subjects still living by the end of the study, as well as any death, were identified. Survival rates and standard mortality rates (SMRs) using general population mortality data were calculated. Mortality-related factors were determined by univariate and multivariate analysis. Results During follow-up, 33 cases of 372 pSS patients resulted in death (8.9%). Of those patients, they were typically older at disease onset, at recruitment, and had shorter follow-up times (p < 0.001 for all). The overall SMR of all pSS patients compared with the general population was 2.11 (95% confidence interval (CI) 1.39–2.83). Male pSS patients had a higher SMR than that of general male patients. Overall survival rates were 97.8% at five years, 90.2% at 10 years, and 87.1% at 15 years in patients with pSS. The survival rate of pSS patients was significantly lower than the general Turkish population (p = 0.011). Multivariate Cox regression analysis showed that older age at disease onset and the presence of interstitial lung disease (ILD) were independent risk factors for mortality. Conclusions Based on these data, mortality rates of Turkish pSS patients are higher compared with the general population. Survival significantly decreased in the pSS patients with ILD, especially in older male patients at disease onset. Male gender and malignancy may also be associated with a worse prognosis in pSS patients. Key Point • Mortality in Sjögren’ s syndrome. Keywords Mortality . Prognosis . Sjögren’ s syndrome . Standard mortality rates . Survival Introduction Primary Sjögren’ s syndrome (pSS) is a systemic autoimmune disease displaying symptoms of ocular and oral dryness, sal- ivary gland enlargement due to lymphocytic infiltration, and extra-glandular involvement, such as joints, skin, and neurological symptoms [1]. Although the symptoms are non- specific at the onset of disease, serious dryness symptoms, organ dysfunction, and laboratory abnormalities are seen in the advanced disease [2]. pSS is a chronic disease that requires a long follow-up period, treatment, and supportive care. There are several studies that reported mortality rates of pSS patients [3–8]. It is also suggested that the mortality in patients with pSS may be related to systemic involvement and lympho- ma [8–11]. The mortality rates decreased from 48% in 1970 to 5–15% in 2000 [3, 5, 7, 12–14]. In one study [15], an adjusted standardized mortality ratio was calculated as 4.66 (95% CI 3.85–5.60), and survival rates at 5, 10, 20, and 30 years were calculated as 96%, 90%, 81%, and 60%, respectively. It was suggested that male gender, cryoglobulinemia, low C4, consti- tutional symptoms, pulmonary involvement, and biologic do- mains of EULAR SS Disease Activity Index (ESSDAI) were * Veli Yazisiz drvyazisiz@yahoo.com.tr 1 Department of Internal Medicine, Division of Rheumatology, Akdeniz Üniversitesi Hastanesi, Konyaaltı, Antalya, Turkey 2 Department of Internal Medicine, Division of Hematology, Antalya Education and Research Hospital, Antalya, Turkey Clinical Rheumatology https://doi.org/10.1007/s10067-019-04744-6