Turk J Immunol. 2021; 9(3): 128-133 | DOI: 10.4274/tji.galenos.2021.85047

Is There a Need for Such a Naming: Seronegative Primary Sjogren’s Syndrome?

Samet Karahan¹, Aycan Karabulut^2
1Kayseri City Training and Research Hospital, Clinic of Internal Medicine, Division of Rheumatology, Kayseri, Turkey
²Kayseri City Training and Research Hospital, Clinic of Internal Medicine, Kayseri, Turkey

Objective: Autoantibodies such as rheumatoid factor, anti-nuclear antibody, anti-Ro/SSA, and anti-La/SSB have an important role in the diagnosis of primary Sjogren’s syndrome (PSS), but are not essential in seronegative PSS when there is at least one focus in minor salivary gland biopsy. The aim of this study was to compare the clinical and serological differences between patients with seropositive and seronegative PSS.
Materials and Methods: The study included 273 patients with PSS who were followed up at Kayseri City Training and Research Hospital, a tertiary hospital, between June 2018 and July 2021. The diagnosis of PSS was based on the 2016 American College of Rheumatology/European League Against Rheumatism PSS classification criteria. Autoantibodies were not detected in 45 (16.5%) patients, and they were evaluated as having seronegative PSS. Clinical and laboratory parameter data of all the patients were collected retrospectively from the hospital database, and compared between the patients with seronegative PSS and patients with seropositive PSS.
Results: Females constituted 93.9% (n=214) of the seropositive group and 91.1% (n=41) of the seronegative group. The mean age at diagnosis was 47.87±9.07 years in the seropositive group and 44.34±11.48 years in the seronegative group (p=0.026). No statistically significant differences were determined between the groups in terms of sex distribution, clinical features, and the rate of patients with leukopenia, lymphopenia, thrombocytopenia, hypergammaglobulinemia, hypocomplementemia, and increased acute phase reactant. The median C4 value was lower, and the median IgG value was higher in the seropositive PSS group.
Conclusion: Clinical findings in patients with seronegative PSS overlapped with those of patients with seropositive PSS, but diagnosis was made later in patients with seronegative PSS.

Keywords: Sjogren’s syndrome, rheumatoid factor, anti-CCP, ANA, anti-Ro, anti-SSA, anti-La

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Seronegatif Primer Sjögren Sendromu: Böyle Bir Ýsimlendirme Gerekli mi?

Samet Karahan¹, Aycan Karabulut^2
1Kayseri Þehir Eðitim ve Araþtýrma Hastanesi, Ýç Hastalýklarý - Romatoloji Kliniði, Kayseri, Türkiye
²Kayseri Þehir Eðitim ve Araþtýrma Hastanesi, Ýç Hastalýklarý Kliniði, Kayseri, Türkiye

Amaç: Romatoid faktör, anti-nükleer antikor, anti-Ro/SSA ve anti-La/SSB gibi otoantikorlar, primer Sjögren sendromunun (PSS) tanýsýnda önemli bir role sahiptir, ancak minör tükürük bezi biyopsisinde en az bir fokus mevcut olduðunda seronegatif PSS tanýsý için gerekli deðildir. Bu çalýþmada, seropozitif ve seronegatif PSS’li hastalar arasýndaki klinik ve serolojik farklýlýklar araþtýrýlmýþtýr.
Gereç ve Yöntem: Haziran 2018-Temmuz 2021 tarihleri arasýnda üçüncü basamak bir hastane olan Kayseri Þehir Eðitim ve Araþtýrma Hastanesi’nde takip edilen 273 PSS’li hasta çalýþmaya dahil edildi. PSS tanýsý, 2016 Amerikan Romatoloji Koleji/Romatizmaya Karþý Avrupa Ligi PSS sýnýflandýrma kriterleri belirlenmiþtir. Kýrk beþ (%16.5) hastada otoantikor saptanmadý ve bu hastalar seronegatif PSS olarak deðerlendirildi. Hastalarýn klinik ve laboratuvar parametreleri hastane veri tabanýndan geriye dönük olarak toplandý. Seronegatif ve seropozitif PSS’li hastalarýn klinik ve laboratuvar verileri karþýlaþtýrýldý.
Bulgular: Seropozitif grupta 214 (%93.9), seronegatif grupta 41 (%91.1) kadýn hasta vardý. Ortalama taný yaþý seropozitifler için 47.87±9.07 yýl ve seronegatifler için 44.34±11.48 yýl olarak saptandý (p=0.026). Seronegatifler ve seropozitifler arasýnda cinsiyet daðýlýmý, klinik özellikler ve lökopeni, lenfopeni, trombositopeni, hipergamaglobulinemi, hipokomplementemi ve akut faz reaktaný artýþý olan hasta sayýsý açýsýndan istatistiksel olarak anlamlý fark saptanmadý. Seropozitif PSS grubunda C4 ortanca deðeri daha düþük, IgG ortanca deðeri daha yüksek saptandý.
Sonuç: Seronegatif PSS’li hastalardaki klinik bulgular seropozitif PSS’li hastalardaki bulgular ile örtüþmektedir, ancak seronegatif PSS’li hastalarda daha geç taný koyulmuþtur.

Anahtar Kelimeler: Sjögren sendromu, romatoid faktör, anti-CCP, ANA, anti-Ro, anti-SSA, anti-La

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