Peer-reviewed article
Egyptian evidence-based consensus on clinical practice guidelines for the diagnosis and treat-to-target management of macrophage activation syndrome in children
Lotfy H, Abu-Zaid M, Salah S, El Gaafary M, Abdulhady H et al. ·
Summary
Written by AI, not yet AICS's view · claude-haiku-4-5-20251001Egyptian paediatric experts developed evidence-based clinical guidelines for diagnosing and treating macrophage activation syndrome (MAS), a life-threatening inflammatory condition in children. Using a Delphi consensus method, 23 specialists reviewed the medical literature and agreed on 19 recommendations covering diagnosis and management. The guidelines include algorithms to help clinicians recognise and treat MAS early, potentially improving survival outcomes for affected children.
Key points
- 19 consensus recommendations were developed: 11 for diagnosis and 8 for management
- Agreement with recommendations ranged from 86.9% to 95.7% across all three Delphi rounds
- All clinical standards achieved consensus with ≥75% of experts strongly agreeing or agreeing
- Management algorithms were developed to support a treat-to-target approach
- The guidelines address a gap in literature by presenting the first T2T (treat-to-target) approach for MAS
Limitations
This is a consensus document based on expert opinion and literature review rather than a clinical trial or intervention study. It cannot demonstrate whether implementing these guidelines actually improves patient outcomes or survival rates in clinical practice.
Abstract
Background Macrophage activation syndrome (MAS) is a severe life-threatening hyperinflammatory state with uncontrolled activation and proliferation of macrophages and T-lymphocytes. MAS has variable causes and risk factors. Early diagnosis and optimum management could be lifesaving. Our aim was to develop a consensus, evidence-based recommendations for the diagnosis, evaluation, and treat-to-target management of pediatric MAS. This study was carried out to achieve an Egyptian expert consensus on a treat-to-target management strategy for MAS using the Delphi technique. The multistep process strategy was used in developing a consensus, evidence-based treatment guidelines for MAS, started by developing 7 key clinical questions by a scientific committee according to the Patient/Population, Intervention, Comparison, and Outcomes (PICO) approach. The core leadership team identified pediatric rheumatology clinicians and researchers throughout Egypt. To generate evidence for MAS management, an evidence-based, systematic literature review was done. To obtain a consensus, the Delphi procedure (3 rounds) was used. Results Twenty-three expert panel participated in the 3 rounds with a response rate of 100%. A total of 19 recommendations, categorized into 2 sections (11 in the diagnosis section and 8 in management), were obtained. The agreement with the recommendations (ranks 7–9) ranged from 86.9 to 95.7%. The consensus was reached (i.e., ≥75% of respondents strongly agreed or agreed) on all the clinical standards. Algorithms for management have been also developed. Conclusion This was an expert, consensus recommendation for the diagnosis and treat to target of MAS, based on the best available evidence and expert opinion. The guidelines fill a gap in the literature as it presents a T2T approach for MAS.