The ethics committee of Ain Shams University or college approved the protocol. Methods All the included participants underwent 1) A detailed history taking focusing on the following info: age, sex, disease duration. Fiacitabine manifestations (65%), malar rash (54%), and nephritis (51%), respectively. All individuals experienced positive ANA (100%), while anti-dsDNA rate of recurrence was 83%. The most common anti-ENA antibodies were anti-RNP (41%), anti-Sm (31%), anti-SSA (27%), and anti-SSB (20%), respectively. Anti-RNP experienced a medical association with oral ulcer, Raynaud phenomena, haematological, neuropsychiatric and thromboembolic manifestations. In the mean time, anti-Sm had a significant association with serositis, mucocutaneous, constitutional, and neuropsychiatric manifestations. Anti-SSA was associated with mucocutaneous, musculoskeletal, Raynaud phenomena, renal, haematological and cardiac manifestations, while anti-SSB was significantly associated with malar rash, serositis, thromboembolic, musculoskeletal, and neuropsychiatric manifestations. Concerning SLEADI score, anti-dsDNA antibody was significantly associated with moderate disease activity score (p=0.032) while anti-SSA significantly associated with large disease activity (p=0.045). Both anti-SSB and anti-Sm were significantly associated with both moderate and high disease activities, in the mean time anti-U1-RNP was associated with moderate disease activity (p=0.014). Summary Anti-dsDNA and anti-ENAs antibodies were frequently found in JSLE individuals (83%, 63%), respectively. They were significantly associated with variable clinical manifestations and could be used as predictors for assessment of disease activity. strong class=”kwd-title” Keywords: juvenile systemic lupus erythematosus, medical manifestations, anti-ENA, disease activity score Intro Systemic lupus erythematosus (SLE) is definitely a chronic autoimmune disorder that is most common among the young-aged females. Its precise cause is still unclear; however, it may be a result of the interplay between many genetic, epigenetic, and environmental factors.1 Juvenile SLE (JSLE) is that type of SLE that affects people 18 years old. Worldwide, it is estimated to be 10C20% of all SLE instances.2 Moreover, the introductory disease severity of JSLE is much greater than adult type. Flares and fresh systems/organs involvement may develop after a long-term remission, up to 10 years after the initial diagnosis.3 The hallmark of SLE is the excessive production of pathogenic antibodies recognizing self-antigens and the formation of antigenCantibody complexes that trigger the immune response to cause multiple organ injury.4 This includes antinuclear antibodies (ANAs) which are found in almost all individuals with SLE, double-stranded DNA (dsDNA), and a variety Fiacitabine of anti-extractable nuclear antigen (ENA) antibodies are detected in SLE individuals at analysis and during disease progression.5 These antibodies include four groups of RNA-binding proteins, namely Sm, RNP, SSA and SSB.6 Owing to the pathophysiological significance of autoantibodies in SLE, several studies possess attempted to elucidate the association between anti-ENA antibodies and SLE specific clinical features. Anti-ds DNA antibodies and anti-Sm antibodies are highly specific for SLE, and the presence of anti-dsDNA and/or anti-Sm antibodies is one of the important criteria for the classification of SLE.7,8 Anti-dsDNA antibody titer has been reported to be consistently associated with the Fiacitabine development of lupus nephritis and disease flare in individuals with SLE.9,10 However, the clinical significance of anti-ENA antibodies remains unclear, but it may forecast the involvement of different organs as well as the severity of the disease, helping in making an early analysis and in the specification of involved organs as well as initiation the appropriate treatment as early as possible.11 In this study, we aimed to estimate the frequency of ANA, anti-dsDNA, and anti-ENA antibodies among individuals with JSLE and their association with the different clinical manifestations and the activity of the disease. Patients and Methods Patients We carried out a cross-sectional study with 100 JSLE individuals aged less than 18 years and were classified relating to Systemic Lupus International Collaborating Clinics classification criteria.8 Patients were recruited from Ain Shams University or college Hospital from July 2019 to August 2020. Honest Considerations We carried out this study according to the defined principles of the World Medical Associations Declaration of Helsinki. Prior to the study, we explained the aim of the study and the involved methods to the caregiver of the included individuals; then, we acquired an informed consent from them. The ethics committee of Ain Shams University or college approved the protocol. Methods All the included participants underwent 1) A detailed history taking focusing on the following info: age, sex, disease period. 2) A full clinical exam that includes rheumatological exam (medical symptoms and manifestations were cumulative data). GluN2A 3) Laboratory investigations (obtained at time of inclusion) were complete blood count, erythrocyte sedimentation rate, C-reactive protein, serum blood urea nitrogen, serum creatinine (mg/dL), creatinine clearance (mL/min), total urine analysis with assessment of active urinary sediments (RBCs C WBCs C proteins or solid), protein/creatinine percentage, 24 h urinary protein, serum match (C3, C4), anticardiolipin (ACL) (IgG, IgM), and lupus anticoagulant (LAC). 4) Renal biopsy for individuals with lupus nephritis (LN) (acute increase in serum creatinine, proteinuria 500 mg/24 h or urine protein/creatinine percentage 0.5 g protein/g creatinine, hematuria in presence.