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Erschienen in: Clinical Rheumatology 1/2020

25.09.2019 | Original Article

Survival analysis of patients with Sjögren’s syndrome in Turkey: a tertiary hospital-based study

verfasst von: Veli Yazisiz, Mesut Göçer, Funda Erbasan, İsmail Uçar, Bengisu Aslan, Şuayp Oygen, Edip Gökalp Gök, Mustafa Ender Terzioğlu

Erschienen in: Clinical Rheumatology | Ausgabe 1/2020

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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.
Literatur
1.
2.
Zurück zum Zitat Kassan SS, Moutsopoulos HM (2004) Clinical manifestations and early diagnosis of Sjögren syndrome. Arch Intern Med 164:1275–1284CrossRef Kassan SS, Moutsopoulos HM (2004) Clinical manifestations and early diagnosis of Sjögren syndrome. Arch Intern Med 164:1275–1284CrossRef
3.
Zurück zum Zitat Martens PB, Pillemer SR, Jacobsson LT, O’Fallon WM, Matteson EL (1999) Survivorship in a population based cohort of patients with Sjögren’s syndrome, 1976–1992. J Rheumatol 26:1296–1300PubMed Martens PB, Pillemer SR, Jacobsson LT, O’Fallon WM, Matteson EL (1999) Survivorship in a population based cohort of patients with Sjögren’s syndrome, 1976–1992. J Rheumatol 26:1296–1300PubMed
4.
Zurück zum Zitat Theander E, Manthorpe R, Jacobsson LT (2004) Mortality and causes of death in primary Sjögren’s syndrome: a prospective cohort study. Arthritis Rheum 50(4):1262–1269CrossRef Theander E, Manthorpe R, Jacobsson LT (2004) Mortality and causes of death in primary Sjögren’s syndrome: a prospective cohort study. Arthritis Rheum 50(4):1262–1269CrossRef
5.
Zurück zum Zitat Kruize AA, Hené RJ, van der Heide A, Bodeutsch C, de Wilde PC, van Bijsterveld OP, de Jong J, Feltkamp TE, Kater L, Bijlsma JW (1996) Long-term followup of patients. Arthritis Rheum 39:297–303CrossRef Kruize AA, Hené RJ, van der Heide A, Bodeutsch C, de Wilde PC, van Bijsterveld OP, de Jong J, Feltkamp TE, Kater L, Bijlsma JW (1996) Long-term followup of patients. Arthritis Rheum 39:297–303CrossRef
6.
Zurück zum Zitat Skopouli FN, Dafni U, Ioannidis JP, Moutsopoulos HM (2000) Clinical evolution, and morbidity and mortality of primary Sjögren’s syndrome. Semin Arthritis Rheum. 29(5):296–304CrossRef Skopouli FN, Dafni U, Ioannidis JP, Moutsopoulos HM (2000) Clinical evolution, and morbidity and mortality of primary Sjögren’s syndrome. Semin Arthritis Rheum. 29(5):296–304CrossRef
7.
Zurück zum Zitat Ioannidis JP, Vassiliou VA, Moutsopoulos HM (2002) Long-term risk of mortality and lymphoproliferative disease and predictive classification of primary Sjögren’s syndrome. Arthritis Rheum 46:741–747CrossRef Ioannidis JP, Vassiliou VA, Moutsopoulos HM (2002) Long-term risk of mortality and lymphoproliferative disease and predictive classification of primary Sjögren’s syndrome. Arthritis Rheum 46:741–747CrossRef
10.
11.
Zurück zum Zitat Voulgarelis M, Tzioufas AG, Moutsopoulos HM (2008) Mortality in Sjögren’s syndrome. Clin Exp Rheumatol 26(5 Suppl 51):S66–S71PubMed Voulgarelis M, Tzioufas AG, Moutsopoulos HM (2008) Mortality in Sjögren’s syndrome. Clin Exp Rheumatol 26(5 Suppl 51):S66–S71PubMed
12.
Zurück zum Zitat Davidson BK, Kelly CA, Griffiths ID (1999) Primary Sjögren’s syndrome in the North East of England: a long-term follow-up study. Rheumatology (Oxford) 38:245–243CrossRef Davidson BK, Kelly CA, Griffiths ID (1999) Primary Sjögren’s syndrome in the North East of England: a long-term follow-up study. Rheumatology (Oxford) 38:245–243CrossRef
13.
Zurück zum Zitat Pertovaara M, Pukkala E, Laippala P, Miettinen A, Pasternack A (2001) A longitudinal cohort study of Finnish patients with primary Sjögren’s syndrome: clinical, immunological, and epidemiological aspects. Ann Rheum Dis 60:467–472CrossRef Pertovaara M, Pukkala E, Laippala P, Miettinen A, Pasternack A (2001) A longitudinal cohort study of Finnish patients with primary Sjögren’s syndrome: clinical, immunological, and epidemiological aspects. Ann Rheum Dis 60:467–472CrossRef
14.
Zurück zum Zitat Alamanos Y, Tsifetaki N, Voulgari PV, Venetsanopoulou AI, Siozos C, Drosos AA (2006) Epidemiology of primary Sjögren’s syndrome in north-west Greece, 1982–2003. Rheumatology (Oxford) 45:187–191CrossRef Alamanos Y, Tsifetaki N, Voulgari PV, Venetsanopoulou AI, Siozos C, Drosos AA (2006) Epidemiology of primary Sjögren’s syndrome in north-west Greece, 1982–2003. Rheumatology (Oxford) 45:187–191CrossRef
15.
Zurück zum Zitat Brito-Zerón P, Kostov B, Solans R, Fraile G, Suárez-Cuervo C, Casanovas A, Rascón FJ, Qanneta R, Pérez-Alvarez R, Ripoll M, Akasbi M, Pinilla B, Bosch JA, Nava-Mateos J, Díaz-López B, Morera-Morales ML, Gheitasi H, Retamozo S, Ramos-Casals M, SS Study Group, Autoimmune Diseases Study Group (GEAS), Spanish Society of Internal Medicine (SEMI) (2016) Systemic activity and mortality in primary Sjögren syndrome: predicting survival using the EULAR-SS Disease Activity Index (ESSDAI) in 1045 patients. Ann Rheum Dis 75(2):348–355. https://doi.org/10.1136/annrheumdis-2014-206418 CrossRefPubMed Brito-Zerón P, Kostov B, Solans R, Fraile G, Suárez-Cuervo C, Casanovas A, Rascón FJ, Qanneta R, Pérez-Alvarez R, Ripoll M, Akasbi M, Pinilla B, Bosch JA, Nava-Mateos J, Díaz-López B, Morera-Morales ML, Gheitasi H, Retamozo S, Ramos-Casals M, SS Study Group, Autoimmune Diseases Study Group (GEAS), Spanish Society of Internal Medicine (SEMI) (2016) Systemic activity and mortality in primary Sjögren syndrome: predicting survival using the EULAR-SS Disease Activity Index (ESSDAI) in 1045 patients. Ann Rheum Dis 75(2):348–355. https://​doi.​org/​10.​1136/​annrheumdis-2014-206418 CrossRefPubMed
16.
Zurück zum Zitat Zhang W, Feng S, Yan S, Zhao Y, Li M, Sun J, Zhang FC, Cui Q, Dong Y (2010) Incidence of malignancy in primary Sjogren’s syndrome in a Chinese cohort. Rheumatology (Oxford) 49:571–577CrossRef Zhang W, Feng S, Yan S, Zhao Y, Li M, Sun J, Zhang FC, Cui Q, Dong Y (2010) Incidence of malignancy in primary Sjogren’s syndrome in a Chinese cohort. Rheumatology (Oxford) 49:571–577CrossRef
17.
Zurück zum Zitat Panchovska M, Sheitanov Y, Uzunov N (2004) Mortality of Bulgarian patients with primary and secondary Sjogren’s syndrome. Bratisl Lek Listy 105(12):434PubMed Panchovska M, Sheitanov Y, Uzunov N (2004) Mortality of Bulgarian patients with primary and secondary Sjogren’s syndrome. Bratisl Lek Listy 105(12):434PubMed
19.
Zurück zum Zitat Kabasakal Y, Kitapçıoğlu G, Karabulut G, Tezcan M, Balkarlı A, Aksoy A, Yavuz Ş, Yılmaz S, Kaşifoğlu T, Kalyoncu U, Dalkılıç E, Tufan A, Mercan R, Yıldız F, Şentürk T, Önen F, Bes C, Erken E, Tunç E, Kamalı S, Tarhan E, Yazıcı A, Düzgün N, Bıçakçıgil M, Yılmaz S, Özmen M, Öcal L, Alibaz-Öner F, Solmaz D, Çobankara V, Nalbant S, Kasapoğlu Günal E, Kaşkari D, Göker B (2017) Criteria sets for primary Sjogren’s syndrome are not adequate for those presenting with extraglandular organ involvements as their dominant clinical features. Rheumatol Int 37(5):675–684. https://doi.org/10.1007/s00296-017-3691-8 CrossRefPubMed Kabasakal Y, Kitapçıoğlu G, Karabulut G, Tezcan M, Balkarlı A, Aksoy A, Yavuz Ş, Yılmaz S, Kaşifoğlu T, Kalyoncu U, Dalkılıç E, Tufan A, Mercan R, Yıldız F, Şentürk T, Önen F, Bes C, Erken E, Tunç E, Kamalı S, Tarhan E, Yazıcı A, Düzgün N, Bıçakçıgil M, Yılmaz S, Özmen M, Öcal L, Alibaz-Öner F, Solmaz D, Çobankara V, Nalbant S, Kasapoğlu Günal E, Kaşkari D, Göker B (2017) Criteria sets for primary Sjogren’s syndrome are not adequate for those presenting with extraglandular organ involvements as their dominant clinical features. Rheumatol Int 37(5):675–684. https://​doi.​org/​10.​1007/​s00296-017-3691-8 CrossRefPubMed
20.
Zurück zum Zitat Shiboski SC, Shiboski CH, Criswell LA, Baer AN, Challacombe S, Lanfranchi H, Schiødt M, Umehara H, Vivino F, Zhao Y, Dong Y, Greenspan D, Heidenreich AM, Helin P, Kirkham B, Kitagawa K, Larkin G, Li M, Lietman T, Lindegaard J, McNamara N, Sack K, Shirlaw P, Sugai S, Vollenweider C, Whitcher J, Wu A, Zhang S, Zhang W, Greenspan JS, Daniels TE, Sjögren’s International Collaborative Clinical Alliance (SICCA) Research Groups (2012) American College of Rheumatology classification criteria for Sjögren’s syndrome: a data-driven, expert consensus approach in the Sjögren’s International Collaborative Clinical Alliance Cohort. Arthritis Care Res 64(4):475–487CrossRef Shiboski SC, Shiboski CH, Criswell LA, Baer AN, Challacombe S, Lanfranchi H, Schiødt M, Umehara H, Vivino F, Zhao Y, Dong Y, Greenspan D, Heidenreich AM, Helin P, Kirkham B, Kitagawa K, Larkin G, Li M, Lietman T, Lindegaard J, McNamara N, Sack K, Shirlaw P, Sugai S, Vollenweider C, Whitcher J, Wu A, Zhang S, Zhang W, Greenspan JS, Daniels TE, Sjögren’s International Collaborative Clinical Alliance (SICCA) Research Groups (2012) American College of Rheumatology classification criteria for Sjögren’s syndrome: a data-driven, expert consensus approach in the Sjögren’s International Collaborative Clinical Alliance Cohort. Arthritis Care Res 64(4):475–487CrossRef
21.
Zurück zum Zitat Daniels TE, Cox D, Shiboski CH, Schiødt M, Wu A, Lanfranchi H, Umehara H, Zhao Y, Challacombe S, Lam MY, de Souza Y, Schiødt J, Holm H, Bisio PAM, Gandolfo MS, Sawaki T, Li M, Zhang W, Varghese-Jacob B, Ibsen P, Keszler A, Kurose N, Nojima T, Odell E, Criswell LA, Jordan R, Greenspan JS, Sjögren’s International Collaborative Clinical Alliance Research Groups (2011) Associations between salivary gland histopathologic diagnoses and phenotypic features of Sjögren’s syndrome among 1,726 registry participants. Arthritis Rheum 63:2021–2030CrossRef Daniels TE, Cox D, Shiboski CH, Schiødt M, Wu A, Lanfranchi H, Umehara H, Zhao Y, Challacombe S, Lam MY, de Souza Y, Schiødt J, Holm H, Bisio PAM, Gandolfo MS, Sawaki T, Li M, Zhang W, Varghese-Jacob B, Ibsen P, Keszler A, Kurose N, Nojima T, Odell E, Criswell LA, Jordan R, Greenspan JS, Sjögren’s International Collaborative Clinical Alliance Research Groups (2011) Associations between salivary gland histopathologic diagnoses and phenotypic features of Sjögren’s syndrome among 1,726 registry participants. Arthritis Rheum 63:2021–2030CrossRef
22.
Zurück zum Zitat American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias (2002) This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med 165:277–304CrossRef American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias (2002) This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med 165:277–304CrossRef
23.
Zurück zum Zitat Andersen PK, Borgan O, Gill RD et al (1993) Statistical models based on counting processes, vol 18. Springer-Verlag, New York, p 322CrossRef Andersen PK, Borgan O, Gill RD et al (1993) Statistical models based on counting processes, vol 18. Springer-Verlag, New York, p 322CrossRef
24.
Zurück zum Zitat Valesini G, Priori R, Bavoillot D, Osborn J, Danieli MG, Del Papa N et al (1997) Differential risk of non-Hodgkin’s lymphoma in Italian patients with primary Sjögren’s syndrome. J Rheumatol 24:2376–2380PubMed Valesini G, Priori R, Bavoillot D, Osborn J, Danieli MG, Del Papa N et al (1997) Differential risk of non-Hodgkin’s lymphoma in Italian patients with primary Sjögren’s syndrome. J Rheumatol 24:2376–2380PubMed
29.
Zurück zum Zitat Ramos-Casals M, Solans R, Rosas J, Camps MT, Gil A, Del Pino-Montes J, Alvo-Alen J, Jiménez-Alonso J, Micó ML, Beltrán J, Belenguer R, Pallarés L, GEMESS Study Group (2008) Primary Sjögren syndrome in Spain: clinical and immunologic expression in 1010 patients. Medicine (Baltimore) 87(4):210–219. https://doi.org/10.1097/MD.0b013e318181e6af CrossRef Ramos-Casals M, Solans R, Rosas J, Camps MT, Gil A, Del Pino-Montes J, Alvo-Alen J, Jiménez-Alonso J, Micó ML, Beltrán J, Belenguer R, Pallarés L, GEMESS Study Group (2008) Primary Sjögren syndrome in Spain: clinical and immunologic expression in 1010 patients. Medicine (Baltimore) 87(4):210–219. https://​doi.​org/​10.​1097/​MD.​0b013e318181e6af​ CrossRef
32.
Zurück zum Zitat Ito I, Nagai S, Kitaichi M, Nicholson AG, Johkoh T, Noma S, Kim DS, Handa T, Izumi T, Mishima M (2005) Pulmonary manifestations of primary Sjogren’s syndrome: a clinical, radiologic, and pathologic study. Am J Respir Crit Care Med 171:632–638CrossRef Ito I, Nagai S, Kitaichi M, Nicholson AG, Johkoh T, Noma S, Kim DS, Handa T, Izumi T, Mishima M (2005) Pulmonary manifestations of primary Sjogren’s syndrome: a clinical, radiologic, and pathologic study. Am J Respir Crit Care Med 171:632–638CrossRef
34.
39.
Zurück zum Zitat Lazarus MN, Robinson D, Mak V, Moller H, Isenberg DA (2006) Incidence of cancer in a cohort of patients with primary Sjogren’s syndrome. Rheumatology (Oxford) 45:1012–1015CrossRef Lazarus MN, Robinson D, Mak V, Moller H, Isenberg DA (2006) Incidence of cancer in a cohort of patients with primary Sjogren’s syndrome. Rheumatology (Oxford) 45:1012–1015CrossRef
40.
Zurück zum Zitat Theander E, Henriksson G, Ljungberg O, Mandl T, Manthorpe R, Jacobsson LTH (2006) Lymphoma and other malignancies in primary Sjogren’s syndrome: a cohort study on cancer incidence and lymphoma predictors. Ann Rheum Dis 65:796–803CrossRef Theander E, Henriksson G, Ljungberg O, Mandl T, Manthorpe R, Jacobsson LTH (2006) Lymphoma and other malignancies in primary Sjogren’s syndrome: a cohort study on cancer incidence and lymphoma predictors. Ann Rheum Dis 65:796–803CrossRef
41.
Zurück zum Zitat Kassan SS, Thomas TL, Moutsopoulos HM, Hoover R, Kimberly RP, Budman DR, Costa J, Decker JL, Chused TM (1978) Increased risk of lymphoma in sicca syndrome. Ann Intern Med 89(6):888–892CrossRef Kassan SS, Thomas TL, Moutsopoulos HM, Hoover R, Kimberly RP, Budman DR, Costa J, Decker JL, Chused TM (1978) Increased risk of lymphoma in sicca syndrome. Ann Intern Med 89(6):888–892CrossRef
43.
Zurück zum Zitat Flores-Chávez A, Kostov B, Solans R, Fraile G, Maure B, Feijoo-Massó C, Rascón FJ, Pérez-Alvarez R, Zamora-Pasadas M, García-Pérez A, Lopez-Dupla M, Duarte-Millán MÁ, Ripoll M, Fonseca-Aizpuru E, Guisado-Vasco P, Pinilla B, dela Red G, Chamorro AJ, Morcillo C, Fanlo P, Soto-Cárdenas MJ, Retamozo S, Ramos-Casals M, Brito-Zerón P, GEAS-SS SEMI Registry (2018) Severe, life-threatening phenotype of primary Sjögren’s syndrome: clinical characterisation and outcomes in 1580 patients (GEAS-SS Registry). Clin Exp Rheumatol 36 Suppl 112(3):121–129PubMed Flores-Chávez A, Kostov B, Solans R, Fraile G, Maure B, Feijoo-Massó C, Rascón FJ, Pérez-Alvarez R, Zamora-Pasadas M, García-Pérez A, Lopez-Dupla M, Duarte-Millán MÁ, Ripoll M, Fonseca-Aizpuru E, Guisado-Vasco P, Pinilla B, dela Red G, Chamorro AJ, Morcillo C, Fanlo P, Soto-Cárdenas MJ, Retamozo S, Ramos-Casals M, Brito-Zerón P, GEAS-SS SEMI Registry (2018) Severe, life-threatening phenotype of primary Sjögren’s syndrome: clinical characterisation and outcomes in 1580 patients (GEAS-SS Registry). Clin Exp Rheumatol 36 Suppl 112(3):121–129PubMed
Metadaten
Titel
Survival analysis of patients with Sjögren’s syndrome in Turkey: a tertiary hospital-based study
verfasst von
Veli Yazisiz
Mesut Göçer
Funda Erbasan
İsmail Uçar
Bengisu Aslan
Şuayp Oygen
Edip Gökalp Gök
Mustafa Ender Terzioğlu
Publikationsdatum
25.09.2019
Verlag
Springer London
Erschienen in
Clinical Rheumatology / Ausgabe 1/2020
Print ISSN: 0770-3198
Elektronische ISSN: 1434-9949
DOI
https://doi.org/10.1007/s10067-019-04744-6

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