Egyptian evidence-based consensus on clinical practice guidelines for the diagnosis and treat-to-target management of macrophage activation syndrome in children

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.