Elevated tissue transglutaminase antibodies in juvenile idiopathic arthritis children: Relation to neutrophil-to-lymphocyte ratio and disease activity

Background: Subclinical gut inflammation is described in juvenile idiopathic arthritis (JIA), so has joint involvement been related to celiac disease (CD). The well-known involvement of tissue transglutaminase (tTG) in the pathogenesis of CD stimulated progress in the field of autoimmune diseases. Aim of the work: To screen JIA children for tTG antibodies and to detect its relation to the neutrophil-lymphocyte ratio (NLR) and disease activity. Patients and methods: The study included 44 JIA children with 44 matched controls. All subjects had no GIT symptoms suggestive of CD. Disease activity was assessed using the juvenile arthritis disease activity score in 27 joints (JADAS-27). The tTG antibodies (IgA and IgG) were assessed. Results: The patients mean age was 12.5 ± 2.8 years and disease duration 5.01 ± 2.9 years; Female:Male 3.4:1. The mean JADAS-27 score was 12.6 ± 2.04. tTG antibodies were positive in 43.2% of the patients compared to 18.2% control (p = 0.01). Antibodies positivity was comparable according to gender and subtypes. The NLR in JIA children (1.62 ± 0.58) was significantly higher than in control (1.3 ± 0.5) (p = 0.006). Those with positive tTG antibodies had a significantly reduced body mass index (p = 0.02) and increased NLR (p = 0.02) compared to those with negative tTG. Only NLR and JADAS-27 would significantly predict antibodies positivity (p = 0.037 and p = 0.04, respectively). Conclusion: Increased tTG antibodies are frequent in JIA children raising the possibility of an associated subclinical CD. Markedly reduced BMI and increased NLR could forecast the presence of these antibodies. In addition to the JADAS-27, the NLR is a simple test that could predict this association and could be a useful biomarker

Assessment of vascular endothelial growth factor in systemic lupus erythematosus patients with anti-phospholipid syndrome

Aim of the work: The aim of the present study was to assess the serum vascular endothelial growth factor (VEGF) in systemic lupus erythematosus (SLE) patients with and without antiphospholipid syndrome (APS). Relation of the VEGF to the clinical characteristics and laboratory investigations were well thought out. Patients and methods: The study included 84 female SLE patients; 37 with APS and 47 without as well as 33 matched control. Disease activity was estimated using the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and damage index evaluated. Serum VEGF level was quantified using ELISA. Results: The mean age of the SLE patients was 29.03 ± 5.4 years with disease duration of 5.2 ± 3.1 years. The VEGF was signficantly higher in the SLE patients (417.1 ± 410.4 pg/ml) compared to the control (76.5 ± 33.01 pg/ml) (p < 0.0001) and was comparable between those with and without APS. VEGF was signficantly higher in those with a positive anti-ds DNA (n = 53) (471.8 ± 431.7 pg/ml) compared to those with a negative test (223.9 ± 234.8 pg/ml) (p = 0.005). The serum VEGF level signficantly correlatied with the SLEDAI (r = 0.34, p = 0.001) and steroid dose (r = 0.27, p = 0.02). On regression analysis, VEGF was not a signficant predictor of disease activity (p = 0.46). A cut off value of 126 pg/ml showed a good sensitivity (72%) and specificity (60%) predicting anti-dsDNA positivity (p = 0.02). Conclusion: Serum VEGF was remarkably increased in SLE patients with no special relation to APS and may be considered a potential marker of disease activity. Further insights on its relation with anti-ds DNA and genotypic expression in SLE are warranted. Keywords: Vascular endothelial growth factor, Systemic lupus erythematosus, Anti-phospholipid syndrome, SLEDAI, SLICC DI, Anti-ds DN

Juvenile and juvenile-onset systemic lupus erythematosus patients: Clinical characteristics, disease activity and damage

Background: The diagnosis of systemic lupus erythematosus (SLE) in children is challenging. The heterogeneous manifestations and disease impact on the child’s growth highlight the importance of timely diagnosis and management. Objective: The aim of the work was to assess and compare the clinical characteristics, disease activity and damage between children with juvenile SLE (JSLE) and adult patients with juvenile-onset (JO-SLE). Patients and methods: 78 SLE patients; 26 children (JSLE) and 52 JO-SLE adults were included in this study. Disease activity was assessed using the SLE Disease Activity Index (SLEDAI) and organ damage using the Systemic Lupus International Collaborating Clinics (SLICC) index. Results: The mean age of the JSLE children was 13.25 ± 2.09 years and 23.17 ± 4.26 years for JO-SLE cases. Age at disease-onset and female gender tended to be higher in JO-SLE cases than in children with JSLE. There was a significantly higher frequency of serositis, nephritis and hematological involvement in the JO-SLE (57.7%, 76.9%, 73.1%, respectively) compared to the JSLE cases (15.4%, 30.8%, 30.8%, respectively) (p < 0.001 for all). The erythrocyte sedimentation rate, creatinine and proteinuria were significantly increased in JO-SLE while alkaline phosphatase was higher in JSLE cases. In JO-SLE cases, SLEDAI significantly increased (5.96 ± 6.18 vs 3.12 ± 1.97; p = 0.003) and the SLICC tended to increase compared to the JSLE children. More JO-SLE cases received hydroxychloroquine and azathioprine. Conclusion: The existence of differences in clinical phenotype has been confirmed, between JSLE and JO-SLE especially as regards serositis, nephritis and heamatological affection. The disease damage was comparable which denotes that the maximum organ involvement occurs in childhood with an almost stationary course

Damage in rheumatic diseases: Contemporary international standpoint and scores emerging from clinical, radiological and machine learning

In rheumatic diseases, damage is a major concern and reflects irreversible organ scarring or tissue degradation. Quantifying damage or measuring its severity is an indispensable concern in determining the overall outcome. Damage considerably influences both longterm prognosis and quality of life. Rheumatic diseases (RD) represent a significant health burden. Organ damage is consistently associated with increased mortality. Monitoring damage is critical in the evaluation of patients and in appraising treatment efficacy. Proper assessment and early detection of damage paves way for modifying the disease course with effective medications and regimens may reduce organ damage, improve outcomes and decrease mortality. With the exception of systemic lupus erythematosus and vasculitis, most RDs lack an established damage index making it an ongoing demand to develop effective scores and prediction models for damage accrual early in the disease course. A better understanding of machine learning with the increasing availability of medical large data may facilitate the development of meaningful precision medicine for patients with RDs. An updated spectrum of clinical and radiological damage scores and indices as well as the role of machine learning are presented in this review for the key RDs