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.