Publications by authors named "Sulaiman Al-Mayouf"
Article Synopsis
- A set of quality indicators (QIs) was created to enhance care for children with juvenile idiopathic arthritis (JIA) in the Asia-Pacific region.
- The development process involved a systematic literature review and input from a Delphi panel of pediatric rheumatologists and stakeholders, narrowing down from 32 initial QIs to a final list of 22.
- These QIs cover six areas of care, focusing on improving access, clinical assessments, medication management, screening, counseling, and patient satisfaction to ultimately boost health outcomes for children with JIA.
Objectives: To develop an easy-to-use and efficient clinical score to identify monogenic lupus based on clinical presentations and to stratify patients who may benefit from confirmatory molecular genetic testing.
Methods: A comprehensive literature review identified 55 distinct items across 12 clinical and laboratory domains, narrowed down to the top ten by a panel of 12 expert paediatric rheumatologists with 80% consensus. The proposed score was tested in a pilot study on 10 patients with monogenic lupus and 30 control subjects with various autoimmune and autoinflammatory diseases.
Background: Systemic lupus erythematosus (SLE) is a chronic inflammatory multisystemic disease. Monitoring disease activity thoughtout the disease course is important for effective management and assessment of disease outcome.
Objective: To assess whether the pan-immune inflammation value (PIIV) at diagnosis could predict organ involvement and disease activity in childhood SLE (cSLE) patients after 12 months of disease onst.
Article Synopsis
- The study focuses on investigating autoinflammatory interferonopathy in Saudi children, characterized by early-onset fevers and high type-I IFN response, aiming to document genotype-phenotype connections and identify novel genetic findings.* -
- Researchers analyzed medical records of 20 patients, revealing that the majority showed symptoms within the first two years of life, with a high rate of family history and consanguinity among affected families.* -
- Genetic testing revealed various conditions linked to specific gene mutations, with several patients diagnosed with Aicardi-Goutières syndrome and other rare interferonopathy conditions; half of the identified genetic variants were novel.*
Objective: To report the frequency of selected autoantibodies and their associations with clinical features in Arab children with monogenic lupus.
Methods: This study was retrospective single-center study of genetically confirmed monogenic lupus cases at childhood lupus clinic at King Faisal Specialist Hospital and Research Center, from June 1997 to July 2022. We excluded familial lupus without genetic testing and patients with insufficient data.
Practice Patterns and Approach to Childhood Lupus Nephritis in Saudi Arabia.
Saudi J Kidney Dis Transpl
November 2023
Renal involvement of systemic lupus erythematosus needs aggressive treatment. Despite the development of multiple international guidelines, differences in practices exist. This study aimed to explore the current practices of pediatric rheumatologists and nephrologists for the diagnosis, management, and monitoring of lupus nephritis (LN) in Saudi Arabia through a survey.
View Article and Find Full Text PDFArticle Synopsis
- - The study aimed to establish and validate specific cutoff values for the systemic Juvenile Arthritis Disease Activity Score 10 (sJADAS10) to differentiate between various disease activity levels in children with systemic juvenile idiopathic arthritis.
- - Researchers used data from 400 patients across multiple countries, applying different methods to determine these cutoffs, ensuring robust validation through comparison of physician assessments.
- - The identified cutoffs were found to effectively separate inactive disease, minimal disease activity, moderate disease activity, and high disease activity, making them reliable for clinical use and research purposes.
Objective: To assess the performance of the new EULAR/ACR criteria, particularly for early detection of cSLE, in comparison to the SLICC criteria among the pediatric population in multiple centers in Saudi Arabia.
Methods: We conducted a retrospective study that enrolled pediatric patients up to the age of 14 years who've been diagnosed with SLE and followed in pediatric rheumatology clinics at 9 multi-tertiary hospitals in Saudi Arabia from 2010 to 2021 as a case group and were compared to a similar group of pediatric patients who've had defined rheumatological diseases other than SLE with a positive ANA titer (≥1:80) as controls. In total, 245 patients were included and distributed as 129 cases (diagnosed by expert pediatric rheumatologists) versus 116 patients in the control group.
Article Synopsis
- The study evaluated the effectiveness of two sets of classification criteria for Granulomatosis with Polyangiitis (GPA) in pediatric patients, comparing the ACR/EULAR criteria with the Ankara 2008 criteria.
- Data from 77 pediatric patients with GPA were analyzed, revealing high sensitivity (94.8%) and specificity (95.3%) for the Ankara criteria, while the ACR/EULAR criteria showed similar rates (89.6% sensitivity and 96.3% specificity).
- The results indicate that both classification criteria are reliable and perform similarly in diagnosing GPA in children.
Biologic agents are increasingly being used to treat adult patients with systemic lupus erythematosus (SLE). However, the available data on biologic agents' use in childhood-onset SLE (cSLE) remains limited. To collate available evidence related to the efficacy and safety of using biologic agents in cSLE.
View Article and Find Full Text PDFBackground: Burnout is frequent among physicians and seems to be underestimated among rheumatologists.
Objective: To estimate the frequency of burnout in a sample of rheumatologists practising in the Arab countries and determine its associated factors.
Methods: This was a descriptive cross-sectional study conducted by the Arab League of Associations for Rheumatology (ArLAR research group) using an anonymous electronic questionnaire set up on the Google Forms platform.
Article Synopsis
- The study aimed to validate the Pediatric Behçet's Disease criteria using an evidence-based method, involving 210 pediatric patients with various conditions.
- After evaluations by blinded experts, a consensus was reached for 66.2% of patients, identifying 24 as confirmed Behçet's Disease and 10 as probable.
- The PEDBD criteria showed high specificity but lower sensitivity compared to other criteria, highlighting the need for further research with larger international cohorts to assess their effectiveness.
Objective: To report the differences in phenotypic characteristics, disease course, and outcome in monogenic and sporadic childhood lupus (SC-lupus) from a single tertiary childhood lupus clinic.
Methods: A descriptive, observational, cross-sectional study was conducted. Data were retrospectively collected at the last follow-up visit on patients with monogenic lupus proven by genetic variants and SC-lupus seen between June 1997 and July 2022.
The Arab League of Associations for Rheumatology (ArLAR) Research Group (ARCH) conducted this study to investigate the number of current practicing rheumatologists in the Arab countries, to estimate the projected number of rheumatologists in 10 years, and to evaluate the current workload, practice profile, consultation waiting time, and geographical mobilities of these rheumatologists. This cross-sectional survey study was conducted in 16 Arab countries in two parts. The first survey was addressed nominally to national societies to estimate the current and projected workforce.
View Article and Find Full Text PDFGenetic defects of result in a wide range of syndromic histiocytosis that encompasses H syndrome. Patients with variants typically have hyperpigmentation, hypertrichosis, hepatosplenomegaly, sensorineural hearing loss, diabetes mellitus, and hypogonadism. Herein, we identify a novel phenotype in a girl presenting with clinical and laboratory findings similar to systemic juvenile arthritis and hyperferritinemia.
View Article and Find Full Text PDFObjectives: To describe the phenotypic, genetic, and outcome characteristics of large-vessel vasculopathy (LVV) in childhood associated with genetic variants. Additionally, a systematic literature review was conducted to delineate the differences between LVV with and without genetic variants.
Methods: The medical records of all children with LVV seen between January 2000 and September 2022 at our institution were retrospectively reviewed for demographic, clinical and genetic data, and outcomes at the last follow-up visit.
Background: The type II transmembrane protein fibrinogen-like protein 2 (FGL2) plays critical roles in hemostasis and immune regulation. The C-terminal immunoregulatory domain of FGL2 can be secreted and is a mediator of regulatory T (Treg) cell suppression. Fgl2 mice develop autoantibodies and glomerulonephritis and have impaired Treg cell function.
View Article and Find Full Text PDFAdenosine deaminase 2 deficiency (DADA2), a rare and potentially fatal systemic autoinflammatory disease, is characterized by low or lack of ADA2 activity due to ADA2 mutations. DADA2 symptoms are variable and include vasculitis, immunodeficiency, and cytopenia. Minimal data are available from Saudi Arabia.
View Article and Find Full Text PDFObjective: The aim of this paper is to present the AutoInflammatory Disease Alliance (AIDA) international Registry dedicated to Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic (VEXAS) syndrome, describing its design, construction, and modalities of dissemination.
Methods: This Registry is a clinical, physician-driven, population- and electronic-based instrument designed for the retrospective and prospective collection of real-life data. Data gathering is based on the Research Electronic Data Capture (REDCap) tool and is intended to obtain real-world evidence for daily patients' management.
Objective: This paper points out the design, development and deployment of the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to pediatric and adult patients affected by Undifferentiated Systemic AutoInflammatory Diseases (USAIDs).
Methods: This is an electronic registry employed for real-world data collection about demographics, clinical, laboratory, instrumental and socioeconomic data of USAIDs patients. Data recruitment, based on the Research Electronic Data Capture (REDCap) tool, is designed to obtain standardized information for real-life research.
Objective: To evaluate the application of the EULAR/ACR-2019 criteria to monogenic lupus patients and compare its performance against the SLICC-2012 criteria.
Methods: In a multicenter retrospective cohort study, consecutive patients with monogenic lupus from three tertiary lupus clinics were enrolled. The diagnosis of monogenic lupus was based on the expert physician's opinion or fulfilling the SLICC-2012 criteria.
Epidemiology and demographics of juvenile idiopathic arthritis in Africa and Middle East.
Pediatr Rheumatol Online J
December 2021
Article Synopsis
- Juvenile Idiopathic Arthritis (JIA) affects about 3 million children worldwide, with higher prevalence in girls and diverse influences from ethnicity and socioeconomic factors in Africa and the Middle East.
- Limited studies on JIA epidemiology in these regions highlight a lack of recent data, revealing a lower prevalence of JIA compared to global estimates, with oligoarticular arthritis as the most common subtype.
- Significant challenges in managing JIA exist, including the need for better data, increased disease awareness, local treatment guidelines, and more pediatric rheumatologists to address the unmet medical needs of affected patients.
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.