title: Criteria sets for primary Sjogren's syndrome are not adequate for those presenting with extraglandular organ involvements as their dominant clinical features.
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.
authors.affiliation: Department of Internal Medicine-Rheumatology, Faculty of Medicine, Ege University, 35040, Izmir, Turkey. yasemin.kabasakal@gmail.com.
authors.forename: Yasemin
authors.initials: Y
authors.lastname: Kabasakal
authors.affiliation: Biostatistics and Medical Informatics, Ege Üniversitesi Tıp Fakültesi, İzmir, Turkey.
authors.forename: Gul
authors.initials: G
authors.lastname: Kitapçıoğlu
authors.affiliation: Department of Internal Medicine-Rheumatology, Faculty of Medicine, Ege University, 35040, Izmir, Turkey.
authors.forename: Gonca
authors.initials: G
authors.lastname: Karabulut
authors.affiliation: Rheumatology, Gazi Universitesi Tıp Fakültesi, Ankara, Turkey.
authors.forename: Mehmet
authors.initials: M
authors.lastname: Tezcan
authors.affiliation: Rheumatology, Pamukkale Universitesi Tıp Fakültesi, Denizli, Turkey.
authors.forename: Ayse
authors.initials: A
authors.lastname: Balkarlı
authors.affiliation: Rheumatology, Dokuz Eylül Üniversitesi Tıp Fakültesi, İzmir, Turkey.
authors.forename: Adem
authors.initials: A
authors.lastname: Aksoy
authors.affiliation: Rheumatology, Marmara Üniversitesi Tıp Fakültesi, İstanbul, Turkey.
authors.forename: Şule
authors.initials: Ş
authors.lastname: Yavuz
authors.affiliation: Rheumatology, Selçuk Üniversitesi Tıp Fakültesi, Konya, Turkey.
authors.forename: Sema
authors.initials: S
authors.lastname: Yılmaz
authors.affiliation: Rheumatology, Eskişehir Osmangazi Üniversitesi Tıp Fakültesi, Eskişehir, Turkey.
authors.forename: Timuçin
authors.initials: T
authors.lastname: Kaşifoğlu
authors.affiliation: Rheumatology, Hacettepe Üniversitesi Tıp Fakültesi, Ankara, Turkey.
authors.forename: Umut
authors.initials: U
authors.lastname: Kalyoncu
authors.affiliation: Rheumatology, Uludağ Üniversitesi Tıp Fakültesi, Bursa, Turkey.
authors.forename: Ediz
authors.initials: E
authors.lastname: Dalkılıç
authors.affiliation: Rheumatology, Gazi Universitesi Tıp Fakültesi, Ankara, Turkey.
authors.forename: Abdurrahman
authors.initials: A
authors.lastname: Tufan
authors.affiliation: Rheumatology, Gazi Universitesi Tıp Fakültesi, Ankara, Turkey.
authors.forename: Rıdvan
authors.initials: R
authors.lastname: Mercan
authors.affiliation: Rheumatology, Çukurova Üniversitesi Tıp Fakültesi, Adana, Turkey.
authors.forename: Fatih
authors.initials: F
authors.lastname: Yıldız
authors.affiliation: Rheumatology, Adnan Menderes Üniversitesi Tıp Fakültesi, Aydın, Turkey.
authors.forename: Taşkın
authors.initials: T
authors.lastname: Şentürk
authors.affiliation: Rheumatology, Dokuz Eylül Üniversitesi Tıp Fakültesi, İzmir, Turkey.
authors.forename: Fatoş
authors.initials: F
authors.lastname: Önen
authors.affiliation: Rheumatology, Abant İzzet Baysal Üniversitesi Tıp Fakültesi, Bolu, Turkey.
authors.forename: Cemal
authors.initials: C
authors.lastname: Bes
authors.affiliation: Rheumatology, Çukurova Üniversitesi Tıp Fakültesi, Adana, Turkey.
authors.forename: Eren
authors.initials: E
authors.lastname: Erken
authors.affiliation: Rheumatology, Süleyman Demirel Üniversitesi Tıp Fakültesi, Isparta, Turkey.
authors.forename: Ercan
authors.initials: E
authors.lastname: Tunç
authors.affiliation: Rheumatology, İstanbul Üniversitesi İstanbul Tıp Fakültesi, İstanbul, Turkey.
authors.forename: Sevil
authors.initials: S
authors.lastname: Kamalı
authors.affiliation: Rheumatology, İzmir Katip Çelebi Üniversitesi Tıp Fakültesi, İzmir, Turkey.
authors.forename: Emine
authors.initials: E
authors.lastname: Tarhan
authors.affiliation: Rheumatology, Kocaeli Üniversitesi Tıp Fakültesi, İzmit, Turkey.
authors.forename: Ayten
authors.initials: A
authors.lastname: Yazıcı
authors.affiliation: Rheumatology, Ankara Üniversitesi Tıp Fakültesi, Ankara, Turkey.
authors.forename: Nurşen
authors.initials: N
authors.lastname: Düzgün
authors.affiliation: Rheumatology, Yeditepe Üniversitesi Tıp Fakültesi, İstanbul, Turkey.
authors.forename: Müge
authors.initials: M
authors.lastname: Bıçakçıgil
authors.affiliation: Rheumatology, Gülhane Askeri Tıp Akademisi, Ankara, Turkey.
authors.forename: Sedat
authors.initials: S
authors.lastname: Yılmaz
authors.affiliation: Rheumatology, İzmir Atatürk Eğitim Araştırma Hastanesi, İzmir, Turkey.
authors.forename: Mustafa
authors.initials: M
authors.lastname: Özmen
authors.affiliation: Rheumatology, İstanbul Üniversitesi İstanbul Tıp Fakültesi, İstanbul, Turkey.
authors.forename: Lale
authors.initials: L
authors.lastname: Öcal
authors.affiliation: Rheumatology, Marmara Üniversitesi Tıp Fakültesi, İstanbul, Turkey.
authors.forename: Fatma
authors.initials: F
authors.lastname: Alibaz-Öner
authors.affiliation: Rheumatology, Dokuz Eylül Üniversitesi Tıp Fakültesi, İzmir, Turkey.
authors.forename: Dilek
authors.initials: D
authors.lastname: Solmaz
authors.affiliation: Rheumatology, Pamukkale Universitesi Tıp Fakültesi, Denizli, Turkey.
authors.forename: Veli
authors.initials: V
authors.lastname: Çobankara
authors.affiliation: Rheumatology, GATA Haydarpaşa Eğitim Hastanesi, İstanbul, Turkey.
authors.forename: Selim
authors.initials: S
authors.lastname: Nalbant
authors.affiliation: Rheumatology, Maltepe Üniversitesi Tıp Fakültesi, İstanbul, Turkey.
authors.forename: Esen
authors.initials: E
authors.lastname: Kasapoğlu Günal
authors.affiliation: Rheumatology, Başkent Üniversitesi Tıp Fakültesi, Ankara, Turkey.
authors.forename: Derya
authors.initials: D
authors.lastname: Kaşkari
authors.affiliation: Rheumatology, Gazi Universitesi Tıp Fakültesi, Ankara, Turkey.
authors.forename: Berna
authors.initials: B
authors.lastname: Göker
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012219:Rheumatology; D012859:Sjogren's Syndrome / Q000175:diagnosis*; D063189:Symptom Assessment; D055815:Young Adult
keywords: Classification criteria; Epidemiology; Extraglandular involvement; Sjogren’s syndrome