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Criteria sets for primary Sjogren’s syndrome are not adequate for those presenting with extraglandular organ involvements as their dominant clinical features

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Abstract

Patients with primary Sjogren’s syndrome (pSS) may go undiagnosed or be misclassified due to the insidious nature and wide spectrum of the disease. The available several classification criteria emphasize glandular findings. We aimed to analyze the efficiency of various classification criteria sets in patients diagnosed on the clinical basis by expert opinion and to compare those pSS patients who fulfilled these criteria with those who did not. This is a multicenter study in which 834 patients from 22 university-based rheumatology clinics are included. Diagnosis of pSS was made on the clinical basis by the expert opinion. In this study, we only interviewed patients once and collected available data from the medical records. The European criteria, American-European Consensus Group (AECG) and American College of Rheumatology (ACR) Sjogren’s criteria were applied. Majority of the patients were women (F/M was 20/1). The median duration from the first pSS-related symptom to diagnosis was significantly shorter in men (2.5 ± 2.3 vs 4.3 ± 5.9 years) (p = 0 < 0.016). When the European, AECG and ACR Sjogren’s criteria were applied, 666 patients (79.9%) satisfied at least one of them. In total, 539 patients (64.4%) satisfied the European, 439 (52.6%) satisfied the AECG, and 359 (43%) satisfied the ACR criteria. Among the entire group, 250 patients (29.9%) satisfied all and 168 (20.1%) met none of the criteria. The rates of extraglandular organ involvements were not different between patients who met at least one of the criteria sets and those who met none. There is an urgent need for the modification of the pSS criteria sets to prevent exclusion of patients with extraglandular involvements as the dominant clinical features.

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Authors and Affiliations

  1. Department of Internal Medicine-Rheumatology, Faculty of Medicine, Ege University, 35040, Izmir, Turkey

    Yasemin Kabasakal & Gonca Karabulut

  2. Biostatistics and Medical Informatics, Ege Üniversitesi Tıp Fakültesi, İzmir, Turkey

    Gul Kitapçıoğlu

  3. Rheumatology, Gazi Universitesi Tıp Fakültesi, Ankara, Turkey

    Mehmet Tezcan, Abdurrahman Tufan, Rıdvan Mercan & Berna Göker

  4. Rheumatology, Pamukkale Universitesi Tıp Fakültesi, Denizli, Turkey

    Ayse Balkarlı & Veli Çobankara

  5. Rheumatology, Dokuz Eylül Üniversitesi Tıp Fakültesi, İzmir, Turkey

    Adem Aksoy, Fatoş Önen & Dilek Solmaz

  6. Rheumatology, Marmara Üniversitesi Tıp Fakültesi, İstanbul, Turkey

    Şule Yavuz & Fatma Alibaz-Öner

  7. Rheumatology, Selçuk Üniversitesi Tıp Fakültesi, Konya, Turkey

    Sema Yılmaz

  8. Rheumatology, Eskişehir Osmangazi Üniversitesi Tıp Fakültesi, Eskişehir, Turkey

    Timuçin Kaşifoğlu

  9. Rheumatology, Hacettepe Üniversitesi Tıp Fakültesi, Ankara, Turkey

    Umut Kalyoncu

  10. Rheumatology, Uludağ Üniversitesi Tıp Fakültesi, Bursa, Turkey

    Ediz Dalkılıç

  11. Rheumatology, Çukurova Üniversitesi Tıp Fakültesi, Adana, Turkey

    Fatih Yıldız & Eren Erken

  12. Rheumatology, Adnan Menderes Üniversitesi Tıp Fakültesi, Aydın, Turkey

    Taşkın Şentürk

  13. Rheumatology, Abant İzzet Baysal Üniversitesi Tıp Fakültesi, Bolu, Turkey

    Cemal Bes

  14. Rheumatology, Süleyman Demirel Üniversitesi Tıp Fakültesi, Isparta, Turkey

    Ercan Tunç

  15. Rheumatology, İstanbul Üniversitesi İstanbul Tıp Fakültesi, İstanbul, Turkey

    Sevil Kamalı & Lale Öcal

  16. Rheumatology, İzmir Katip Çelebi Üniversitesi Tıp Fakültesi, İzmir, Turkey

    Emine Tarhan

  17. Rheumatology, Kocaeli Üniversitesi Tıp Fakültesi, İzmit, Turkey

    Ayten Yazıcı

  18. Rheumatology, Ankara Üniversitesi Tıp Fakültesi, Ankara, Turkey

    Nurşen Düzgün

  19. Rheumatology, Yeditepe Üniversitesi Tıp Fakültesi, İstanbul, Turkey

    Müge Bıçakçıgil

  20. Rheumatology, Gülhane Askeri Tıp Akademisi, Ankara, Turkey

    Sedat Yılmaz

  21. Rheumatology, İzmir Atatürk Eğitim Araştırma Hastanesi, İzmir, Turkey

    Mustafa Özmen

  22. Rheumatology, GATA Haydarpaşa Eğitim Hastanesi, İstanbul, Turkey

    Selim Nalbant

  23. Rheumatology, Maltepe Üniversitesi Tıp Fakültesi, İstanbul, Turkey

    Esen Kasapoğlu Günal

  24. Rheumatology, Başkent Üniversitesi Tıp Fakültesi, Ankara, Turkey

    Derya Kaşkari

Authors
  1. Yasemin Kabasakal
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  2. Gul Kitapçıoğlu
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  3. Gonca Karabulut
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  4. Mehmet Tezcan
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  5. Ayse Balkarlı
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  6. Adem Aksoy
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  7. Şule Yavuz
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  8. Sema Yılmaz
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  9. Timuçin Kaşifoğlu
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  12. Abdurrahman Tufan
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  13. Rıdvan Mercan
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  14. Fatih Yıldız
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  15. Taşkın Şentürk
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  16. Fatoş Önen
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  17. Cemal Bes
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  18. Eren Erken
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  19. Ercan Tunç
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  20. Sevil Kamalı
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  21. Emine Tarhan
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  22. Ayten Yazıcı
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  23. Nurşen Düzgün
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  24. Müge Bıçakçıgil
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  25. Sedat Yılmaz
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  26. Mustafa Özmen
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  27. Lale Öcal
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  28. Fatma Alibaz-Öner
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  29. Dilek Solmaz
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  30. Veli Çobankara
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  31. Selim Nalbant
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  32. Esen Kasapoğlu Günal
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  33. Derya Kaşkari
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  34. Berna Göker
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Corresponding author

Correspondence to Yasemin Kabasakal.

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Conflict of interest

Kabasakal, Yasemin, M.D., Kitapcıoğlu, Gül, Karabulut, Gonca, M.D., Tezcan, Mehmet E., M.D., Balkarlı, Ayşe, M.D., Aksoy, Adem, M.D., Yavuz, Şule, M.D., Yılmaz, Sema, M.D., Kaşifoğlu, Timuçin, M.D., Kalyoncu, Umut, M.D., Dalkılıç, Ediz, M.D., Tufan, Abdurrahman, M.D., Mercan, Rıdvan, M.D., Yıldız, Fatih, M.D., Şentürk, Taşkın, M.D., Önen, Fatoş, M.D., Bes, Cemal, M.D., Erken, Eren, M.D., Tunç, Ercan, M.D., Kamalı, Sevil, M.D., Tarhan, Emine, M.D., Yazıcı, Ayten, M.D., Düzgün, Nurşen, M.D., Bıçakçıgil, Müge, M.D., Yılmaz, Sedat, M.D., Özmen, Mustafa, M.D., Öcal, Lale, M.D., Alibaz-Öner, Fatma, M.D., Solmaz, Dilek, M.D., Çobankara, Veli, M.D., Nalbant, Selim, M.D., Kasapoğlu Günal, Esen, M.D., Kaskari, Derya, M.D., Göker, Berna, M.D., declare that they have no conflict of interest related to this manuscript to disclose.

Financial support

This study was funded by the Rheumatology Society of Turkey (TRD) (award recipient is Yasemin Kabasakal, M.D.).

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Kabasakal, Y., Kitapçıoğlu, G., Karabulut, G. et al. 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, 675–684 (2017). https://doi.org/10.1007/s00296-017-3691-8

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