Primer Sjögren Sendromunda Ekstra-glandüler Bulguların Oto-antikorlarla İlişkisi

Abstract

Primer Sjögren sendromunda (pSS) serumdaki oto-antikorların varlığı hem daha şiddetli glandüler tutulum hem de daha fazla extra-glandüler bulgu prevalansı ile ilişkilendirilmektedir. Bu çalışmanın amacı pSS hastalarındaki extra-glandüler bulguların sıklığının saptanması ve oto-antikorların varlığı ve sayısı ile ilişkisinin belirlenmesidir. Bu retrospektif kesitsel çalışmaya 2018-2023 tarihleri arasında Mersin Üniversitesi Tıp Fakültesi Hastanesi Romatoloji polikliniğinde pSS tanısı ile takip edilmiş olan 209 hasta dahil edildi. Hastaların demografik ve klinik verileri, extra-glandüler bulguları ve serum oto-antikor profili analiz edildi. Hastaların 92’sinde (%44) ekstra-glandüler bulgu mevcuttu. En sık olarak artrit görülürken, bunu interstisyel akciğer hastalığı takip etmekteydi. Erkeklerde ekstra-glandüler tutulum sıklığı kadınlardan daha fazla bulundu (p=0.050). Anti-SSB pozitifliği olanlarda interstisyel akciğer hastalığı (İAH) ve polinöropati (PNP) daha sık saptanmıştır (sırasıyla p=0.002, p=0.050). RF pozitifliği olanlarda ise artrit ve lenfomanın daha sık görüldüğü gözlenmiştir (sırasıyla p=0.031, p=0.011). Tüm oto-antikorları negatif hastalarda extra-glandüler tutulum daha az saptandı (p=0.005). Anti-SSA ve SSB’nin birlikte pozitif olduğu hastalarda İAH ve PNP daha sıktı (p=0.004 ve p=0.043, sırasıyla). pSS’unda hastaların yaklaşık yarısında extra-glandüler bulgular eşlik etmekte olup, otoantikor oluşumu ve bunların birlikte pozitifliği daha fazla sistemik tutuluma neden olmaktadır. Anti-SSB antikor pozitifliği PNP ve İAH gelişimi riski ile ilişkili iken RF pozitifliği ise artrit ve lenfoma riski ile ilişkilidir.

Keywords

• Anti-SSA
• Anti-SSB
• Extra-glandüler bulgular
• Otoantikor
• Primer Sjögren sendromu

Relationship between Extra-glandular Manifestations and Auto-antibodies in Primary Sjögren's Syndrome

Abstract

The presence of serum autoantibodies in primary Sjögren's syndrome (pSS) is associated with more severe glandular disease and a higher incidence of extra-glandular manifestations. This study aims to establish the prevalence of extra glandular symptoms in patients with pSS and their relationship to the existence and number of autoantibodies. This retrospective cross-sectional study comprised 209 patients diagnosed with pSS in the Rheumatology outpatient clinic at Mersin University Faculty of Medicine Hospital between 2018 and 2023. The patient's demographic and clinical data, extra glandular manifestations, and serum autoantibody profiles were analyzed. Extra glandular symptoms were identified in 92 patients (44%). The most common manifestation was arthritis, followed by interstitial lung disease (ILD). Extra-glandular involvement was found to be more common in men than in women (p=0.050). Extra glandular involvement was less prevalent in patients who were negative for all autoantibodies (p=0.005). Patients who were positive for Anti-SSB had higher frequencies of interstitial lung disease (ILD) and polyneuropathy (PNP) (p=0.002 and p=0.050, respectively). Additionally, those who were positive for rheumatoid factor (RF) showed a higher prevalence of arthritis and lymphoma (p=0.031 and p=0.011, respectively). Patients who were seropositive for both anti-SSA and anti-SSB also had a greater risk of interstitial lung disease (ILD) and polyneuropathy (PNP) (p=0.004 and p=0.043, respectively). Approximately half of pSS patients have extra glandular symptoms, and the production of autoantibodies and their concomitant positivity result in increasing systemic involvement. Anti-SSB antibody positivity increases the likelihood of developing PNP and ILD, whereas RF positivity increases the risk of developing arthritis and lymphoma.

Keywords

• Anti-SSA
• Anti-SSB
• Extra-glandular manifestations
• Autoantibody
• Primary Sjögren

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