ABSTRACT
BACKGROUND: Systemic juvenile idiopathic arthritis (sJIA) is a diagnosis of exclusion. The complex nature and clinical variety of the disease, as well as the vast clinical variation of disease presentation, may lead to difficulties in disease detection and subsequent delays in treatment. AIM: To provide a consensus guidance on the management of newly diagnosed sJIA patients among pediatric rheumatologists in Arab countries. METHODS: This work was conducted in two phases. The first phase utilized an electronic survey sent through an email invitation to all pediatric rheumatologists in Arab countries. In the second phase, a Task Force of ten expert pediatric rheumatologists from Arab countries met through a series of virtual meetings. Results obtained in phase one were prioritized using a nominal group and Delphi-like techniques in phase two. RESULTS: Seven overarching principles and a set of recommendations were approved by the Task Force to form the final consensus. CONCLUSION: This is the first consensus on a clinical approach for pediatric rheumatic diseases among Arab pediatric rheumatologists. It is presented as a guidance on the clinical approach to sJIA that requires further evidence, and future updates are anticipated.
ABSTRACT
BACKGROUND: Characteristics of critical Severe Acute Respiratory Syndrome-related Coronavirus 2 (SARS-CoV-2) infection in children is not well understood. This study described the clinical characteristics of children admitted to intensive care units (ICU) and explored factors associated with the need for invasive ventilation or mortality. METHODS: A multicenter, retrospective, cohort study was conducted over eight medical centers, including all patients younger than 18 years of age and admitted to the ICU due to a direct consequence of coronavirus disease 2019 (COVID-19). Patients who were admitted to the ICU for any alternate reason and tested positive for SARS-CoV-2 by screening test, and patients who were admitted due to multi-inflammatory syndrome in children, were excluded. Demographic, laboratory, imaging, and clinical data were collected. Descriptive statistics were used to compare survivors and non-survivors. Fine and Gray's hazard model was used to estimate the association between clinical variables and ICU death. RESULTS: During the study period, 25 pediatric COVID-19 patients received care in the ICUs. The median age was 2.78 years (IQR 0.21-8.51), and 60% were male. Only three patients were reported to be previously healthy at admission. Nine (36%) patients required invasive mechanical ventilation, including two were on extracorporeal membrane oxygenation. Four (16%) patients died during ICU care. In univariate analysis, the presence of comorbidity (HR 0.0001; 95%CI 0.00001-0.00016), platelets count (HR 0.99; 95% CI 0.98-0.99), elevated procalcitonin (HR 1.05; 95%CI 1.016-1.09), and circulatory compromise (HR 16.34; 95%CI 1.99-134.35), all at the time of ICU admission, were associated with in-ICU mortality. CONCLUSION: Our findings suggest that children admitted to the ICU with SARS-CoV-2 infection, generally, have a favorable outcome. Low platelets count, elevated procalcitonin, presence of comorbidity, and shock at the time of ICU admission were associated with death. This study may shed more light on the disease dynamics of critical pediatric COVID-19.
Subject(s)
COVID-19/mortality , COVID-19/therapy , Critical Illness , Adolescent , Child , Child, Preschool , Comorbidity , Female , Humans , Infant , Intensive Care Units , Male , Respiration, Artificial , Retrospective StudiesABSTRACT
OBJECTIVE: To report the cumulative articular and extraarticular damage in Arab children with juvenile idiopathic arthritis (JIA) and to identify variables that correlate with disease damage. METHODS: We conducted a multicenter, cross-sectional study among 14 pediatric rheumatology centers from 7 Arab countries. JIA patients who met the International League of Associations for Rheumatology classification criteria and had a disease duration of >1 year were enrolled. Disease activity status was assessed using the Juvenile Arthritis Multidimensional Assessment Report. Disease damage was assessed by the Juvenile Arthritis Damage Index, articular (JADI-A) and extraarticular (JADI-E). RESULTS: A total of 702 (471 female) JIA patients with a median age of 11.3 years (interquartile range [IQR] 8.0-14.0 years) were studied. Median age at disease onset was 5 years (IQR 2.0-9.0 years) and the median disease duration was 4 years (IQR 2.0-7.0 years). The most frequent JIA categories were oligoarticular JIA (34.9%), polyarticular JIA (29.5%), and systemic JIA (24.5%). Clinical remission was achieved in 73.9% of patients. At the last clinic visit, 193 patients experienced joint damage, with a mean ± SD JADI-A score of 1.7 ± 4.5, while 156 patients had extraarticular damage, with a mean ± SD JADI-E score of 0.5 ± 1.1. Patients with enthesitis-related arthritis had the highest JADI-A score. JADI-A correlated significantly with the presence of a family history of JIA. JADI-A and JADI-E had a significant correlation with long disease duration. CONCLUSION: Cumulative damage was common in this Arab JIA cohort, and consanguinity and JIA in a sibling were frequent findings and were associated with a greater cumulative damage.
