ArticleAnnals of the rheumatic diseases2014
Validation of the auto-inflammatory diseases activity index (AIDAI) for hereditary recurrent fever syndromes.
Article in Annals of the rheumatic diseases, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 77 papers, 6 of them syntheses that pooled it.
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Who cites it
77 citing papers in PubMed, 6 syntheses or guidelines pooled it, 155 citations in OpenAlex.
- Mevalonate kinase deficiency: an updated clinical overview and revision of the SHARE recommendations.Frontiers in immunology · 2024Pooled 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
- Effectiveness and Safety of JAK Inhibitors in Autoinflammatory Diseases: A Systematic Review.Frontiers in medicine · 2022Pooled it
- Consensus protocols for the diagnosis and management of the hereditary autoinflammatory syndromes CAPS, TRAPS and MKD/HIDS: a German PRO-KIND initiative.Pediatric rheumatology online journal · 2020Guideline
- 2016 American College of Rheumatology/European League Against Rheumatism criteria for minimal, moderate, and major clinical response in adult dermatomyositis and polymyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative.Annals of the rheumatic diseases · 2017Guideline
- 2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Juvenile Dermatomyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative.Annals of the rheumatic diseases · 2017Guideline
- Canakinumab for Treatment of Adult-Onset Still's Disease to Achieve Reduction of Arthritic Manifestation (CONSIDER): phase II, randomised, double-blind, placebo-controlled, multicentre, investigator-initiated trial.Annals of the rheumatic diseases · 2020Trial
- [Young adults with autoinflammation-What can rheumatologists expect?]Zeitschrift fur Rheumatologie · 2026Review
- Association of Hemoglobin Level and Diagnostic Delay with Left Atrial Volume Index in Patients with Familial Mediterranean Fever: A Cross-Sectional Observational Study.Journal of clinical medicine · 2026Article
- Clinical and laboratory manifestations and treatment of children withWorld journal of clinical pediatrics · 2026Article
- Premenstrual syndrome and inflammatory activity in adolescent familial Mediterranean fever.Rheumatology (Oxford, England) · 2026Article
- Evaluation of clinical responses to probiotics and colchicine in PFAPA patients based on autoinflammatory diseases activity index (AIDAI) scores.Pediatric research · 2026Article
- Practical considerations for monitoring patients with familial Mediterranean fever: review of evidence and expert opinion.Frontiers in immunology · 2026Review
- Diagnosis, management, and monitoring of interleukin-1 mediated diseases in Central and Eastern Europe: real-world data.Pediatric rheumatology online journal · 2025Article
- Colchicine treatment in PFAPA: how long should we wait for the clinical response?European journal of pediatrics · 2025Article
- Review
- Familial Mediterranean Fever; Recent Advances, Future Prospectives.Diagnostics (Basel, Switzerland) · 2025Review
- Insights into familial Mediterranean fever: Chronic disease correlations with arthralgia and current health status of patients with familial Mediterranean fever in Jordan.Rheumatology and immunology research · 2025Article
- Disease activity score for still's disease.Clinical rheumatology · 2024Article
- RETRACTED: The Superiority of Compressed Colchicine Tablets over Coated Colchicine Tablets for Familial Mediterranean Fever.Medicina (Kaunas, Lithuania) · 2024Article
17 more citing papers are in PubMed but not listed here.
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Authors and funding
14 authors at 8 institutions in 8 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectivesTo validate the Auto-Inflammatory Diseases Activity Index (AIDAI) in the four major hereditary recurrent fever syndromes (HRFs): familial Mediterranean fever (FMF), mevalonate kinase deficiency (MKD), tumour necrosis factor receptor-associated periodic syndrome (TRAPS) and cryopyrin-associated periodic syndromes (CAPS).
methodsIn 2010, an international collaboration established the content of a disease activity tool for HRFs. Patients completed a 1-month prospective diary with 12 yes/no items before a clinical appointment during which their physician assessed their disease activity by a questionnaire. Eight international experts in auto-inflammatory diseases evaluated the patient's disease activity by a blinded web evaluation and a nominal group technique consensus conference, with their consensus judgement considered the gold standard. Sensitivity/specificity/accuracy measures and the ability of the score to discriminate active from inactive patients via the best cut-off score were calculated by a receiver operating characteristic analysis.
resultsConsensus was achieved for 98/106 (92%) cases (39 FMF, 35 CAPS, 14 TRAPS and 10 MKD), with 26 patients declared as having inactive disease and 72 as having active disease. The median total AIDAI score was 14 (range=0-175). An AIDAI cut-off score ≥9 discriminated active from inactive patients, with sensitivity/specificity/accuracy of 89%/92%/90%, respectively, and an area under the curve of 98% (95% CI 96% to 100%).
conclusionsThe AIDAI score is a valid and simple tool for assessing disease activity in FMF/MKD/TRAPS/CAPS. This tool is easy to use in clinical practice and has the potential to be used as the standard efficacy measure in future clinical trials.
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