Prevalence and Clinical Implications of Scleroderma-Specific Autoantibodies in Seronegative Patients with Sicca Complaints

Abstract

Introduction: Previous studies have revealed the presence of anticentromere antibodies in patients with Sjögren’s syndrome (SS), predominantly in those serologically negative for antibodies against Ro/SSA and La/SSB antigens (seronegative). The prevalence and clinical significance of specific autoantibodies for Systemic Sclerosis (SSc) in seronegative patients with sicca complaints (dry eyes, dry mouth) have not yet been studied. Aim of the study: Investigate the prevalence and clinical significance of SSc-specific autoantibodies in seronegative patients with sicca complaints. Methods: Among 212 patients with sicca symptoms that were investigated at the Laboratory of Physiology, Medical School of Athens, National and Kapodistrian University of Athens between the years 2017–2020 for the presence of antibodies against Ro/SSA and La/SSB antigens, we found 106 patients that did not meet the criteria for SS following a thorough investigation. We have already performed serological tests of 13 specific autoantibodies for Scleroderma by using the test EUROLINE Systemic Sclerosis (Nucleoli) profile (IgG) (EUROIMMUNE Medizinische Labordiagnostika AG) in 51 of these patients. Additionally, their demographic, clinical, and laboratory data have been recorded. Results: The presence of the specific SSc-specific autoantibodies was noticed in 29/51 patients (57%). More precisely, in 7/29 patients (24%) we recorded NOR90 as well as Th/To, in 5/29 (17%) Ku & Ro52, in 4/29 (14%) CENP-B, in 3/29 (10%) CENP-A & RP155, in 2/29 (7%) RP11 & PM/Scl 75 and finally in 1/29 (3.5%) Scl70, PM/Scl 100 & Fibrillarin respectively. The subsequent follow-up of these patients revealed signs of pulmonary hypertension and interstitial lung disease in some cases. Conclusions: The high prevalence of Scleroderma-specific autoantibodies in seronegative sicca patients discloses those needing tighter follow-up and systematic treatment.