ArticleRheumatology international2020
Pattern and diagnostic evaluation of systemic autoinflammatory diseases other than familial Mediterranean fever among Arab children: a multicenter study from the Pediatric Rheumatology Arab Group (PRAG).
Article in Rheumatology international, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers, 3 of them syntheses that pooled it.
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Who cites it
12 citing papers in PubMed, 3 syntheses or guidelines pooled it, 18 citations in OpenAlex.
- Prebiotics, Probiotics, and Postbiotics for Acne Vulgaris: A Systematic Review.Dermatology and therapy · 2026Pooled it
- Undifferentiated autoinflammatory diseases: a systematic review.World journal of pediatrics : WJP · 2026Pooled it
- The 2021 EULAR/American College of Rheumatology Points to Consider for Diagnosis, Management and Monitoring of the Interleukin-1 Mediated Autoinflammatory Diseases: Cryopyrin-Associated Periodic Syndromes, Tumour Necrosis Factor Receptor-Associated Periodic Syndrome, Mevalonate Kinase Deficiency, and Deficiency of the Interleukin-1 Receptor Antagonist.Arthritis & rheumatology (Hoboken, N.J.) · 2022Pooled it
- Neurodevelopmental comorbidities in juvenile systemic autoimmune and autoinflammatory diseases.Nature reviews. Rheumatology · 2026Review
- Chronic Non-Bacterial Osteomyelitis in Pediatric Patients: Findings from Multiple Centers in Saudi Arabia.Journal of epidemiology and global health · 2025Article
- Consanguinity and rare monogenic systemic autoinflammatory disorders: implications for prevalence and genetic variability.Pediatric rheumatology online journal · 2025Observational
- The research progress of LACC1.Frontiers in immunology · 2025Review
- From Sand to Excellence: A Deep Dive into Abu Dhabi's Rheumatology Landscape.Mediterranean journal of rheumatology · 2024Review
- Spectrum of auto-inflammatory diseases in Morocco: a monocentric experience.Rheumatology advances in practice · 2023Article
- A purine metabolic checkpoint that prevents autoimmunity and autoinflammation.Cell metabolism · 2022Article
- Chronic recurrent multifocal osteomyelitis. A narrative and pictorial review.Frontiers in immunology · 2022Review
- Consensus clinical approach for a newly diagnosed systemic juvenile idiopathic arthritis among members of the pediatric rheumatology Arab group.International journal of pediatrics & adolescent medicine · 2021Article
Corrections and comments
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Authors and funding
18 authors at 7 institutions in 6 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
To define the spectrum and phenotypic characteristics of systemic autoinflammatory diseases (SAIDs) other than familial Mediterranean fever (FMF) in Arab children and to delineate diagnostic evaluation. Data retrospectively collected on patients with clinical and/or genetically proven SAIDs other than FMF at 10 tertiary Arab pediatric rheumatology clinics from 1990 to 2018. The collected data comprised the clinical findings and diagnostic evaluation including genetic testing, the provided treatment and the accrual damage related to SAIDs. A total of 144 patients (93 female) with a median age at onset of 2.5 (range 0.1-12) years were enrolled. The initial diagnosis was inaccurate in 49.3%. Consanguinity rate among parents was 74.6%. The median time-to-diagnosis for all SAIDs was 2.5 (range 0.1-10) years. There were 104 patients (72.2%) with a confirmed diagnosis and 40 patients with suspected SAIDs. Seventy-two had monogenic and 66 patients with multifactorial SAIDs while six patients had undifferentiated SAIDs. The most frequent monogenic SAIDs were LACC1 mediated monogenic disorders (n = 23) followed by CAPS (12), TRAPS (12), HIDS (12), and Majeed's syndrome (6). The most frequent multifactorial SAIDs was CRMO (34), followed by PFAPA (18), and early onset sarcoidosis (EOS) (14). Genetic analysis was performed in 69 patients; 50 patients had genetically confirmed disease. Corticosteroid used for 93 patients while biologic agents for 96 patients. Overall, growth failure was the most frequent accrual damage (36%), followed by cognitive impairment (13%). There were three deaths because of infection. This study shows a heterogenous spectrum of SAIDs with a high number of genetically confirmed monogenic diseases; notably, LACC1 associated diseases. Hopefully, this work will be the first step for a prospective registry for SAIDs in Arab countries.
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