IS SERONEGATIVE SJÖGREN'S SYNDROME: AN OVERLOOKED ENTITY?

Year 2024, Volume: 5 Issue: 1, 34 - 36, 28.03.2024

Erdal Bodakçi , Esra Erpek

Abstract

Primary Sjögren syndrome (pSS) is a systemic autoimmune disorder characterized by the chronic inflammation and dysfunction of exocrine glands, mainly salivary glands, causing dryness of the eyes and the mouth. Patients present with a spectrum of clinical manifestations from sicca (dryness) symptoms to potentially severe extra glandular and/or systemic features, such as inflammatory arthritis, parotitis, interstitial lung disease, neurological dysfunction, cryoglobulinemia, and lymphomas. SS have autoantibodies such as anti-Ro/SSA, anti-La/SSB, antinuclear antibodies (ANA), and rheumatoid factors(RF). These autoantibodies are present in 45% to 75% of patients. Patients fulfilling SS criteria who do not express classic serum antibodies are called patients with seronegative SS. Seronegative SS varies in the literature, ranging from 8% to 37% of SS cohorts. Diagnosing seronegative pSS can be difficult. Without readily detectable serum biomarkers, patients with seronegative SS may be overlooked in the clinic. Here we present 3 cases of seronegative SS with different organ involvement.

Keywords

SJÖGREN, SERONEGATIVE

SERONEGATIVE SJÖGREN SENDROMU: GÖZDEN KAÇAN BIR ANTITE MI?

Abstract

Primary Sjögren syndrome (pSS) is a systemic autoimmune disorder characterized by the chronic inflammation and dysfunction of exocrine glands, mainly salivary glands, causing dryness of the eyes and the mouth. Patients present with a spectrum of clinical manifestations from sicca (dryness) symptoms to potentially severe extra glandular and/or systemic features, such as inflammatory arthritis, parotitis, interstitial lung disease, neurological dysfunction, cryoglobulinemia, and lymphomas. SS have autoantibodies such as anti-Ro/SSA, anti-La/SSB, antinuclear antibodies (ANA), and rheumatoid factors(RF). These autoantibodies are present in 45% to 75% of patients. Patients fulfilling SS criteria who do not express classic serum antibodies are called patients with seronegative SS. Seronegative SS varies in the literature, ranging from 8% to 37% of SS cohorts. Diagnosing seronegative pSS can be difficult. Without readily detectable serum biomarkers, patients with seronegative SS may be overlooked in the clinic. Here we present 3 cases of seronegative SS with different organ involvement.

Keywords

SJÖGREN SENDROMU, SERONEGATIVE