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

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...

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Main Authors: H. Lotfy, M. H. Abu-Zaid, S. Salah, M. El Gaafary, H. Abdulhady, H. Salah, E. Abd El-Latif, Y. Farag, M. Eissa, S. Esam Maher, A. Radwan, Amira T. El-Shanawany, B. M. Medhat, D. El Mikkawy, D. M. Mosa, G. El Deriny, M. Mortada, N. S. Osman, N. A. Fouad, N. E. Elkaraly, S. S. Mohamed, S. A. Tabra, W. A. Hassan, H. Abu Shady, Y. Amer, S. I. Nasef, Salwa Galal, Y. El Miedany
Format: Article
Language:English
Published: SpringerOpen 2022-06-01
Series:Egyptian Rheumatology and Rehabilitation
Subjects:
Online Access:https://doi.org/10.1186/s43166-022-00135-z
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author H. Lotfy
M. H. Abu-Zaid
S. Salah
M. El Gaafary
H. Abdulhady
H. Salah
E. Abd El-Latif
Y. Farag
M. Eissa
S. Esam Maher
A. Radwan
Amira T. El-Shanawany
B. M. Medhat
D. El Mikkawy
D. M. Mosa
G. El Deriny
M. Mortada
N. S. Osman
N. A. Fouad
N. E. Elkaraly
S. S. Mohamed
S. A. Tabra
W. A. Hassan
H. Abu Shady
Y. Amer
S. I. Nasef
Salwa Galal
Y. El Miedany
author_facet H. Lotfy
M. H. Abu-Zaid
S. Salah
M. El Gaafary
H. Abdulhady
H. Salah
E. Abd El-Latif
Y. Farag
M. Eissa
S. Esam Maher
A. Radwan
Amira T. El-Shanawany
B. M. Medhat
D. El Mikkawy
D. M. Mosa
G. El Deriny
M. Mortada
N. S. Osman
N. A. Fouad
N. E. Elkaraly
S. S. Mohamed
S. A. Tabra
W. A. Hassan
H. Abu Shady
Y. Amer
S. I. Nasef
Salwa Galal
Y. El Miedany
author_sort H. Lotfy
collection DOAJ
description 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.
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spelling doaj.art-8713d8995de74de08f802da28ac98bc92022-12-22T02:32:22ZengSpringerOpenEgyptian Rheumatology and Rehabilitation1110-161X2090-32352022-06-0149111710.1186/s43166-022-00135-zEgyptian evidence-based consensus on clinical practice guidelines for the diagnosis and treat-to-target management of macrophage activation syndrome in childrenH. Lotfy0M. H. Abu-Zaid1S. Salah2M. El Gaafary3H. Abdulhady4H. Salah5E. Abd El-Latif6Y. Farag7M. Eissa8S. Esam Maher9A. Radwan10Amira T. El-Shanawany11B. M. Medhat12D. El Mikkawy13D. M. Mosa14G. El Deriny15M. Mortada16N. S. Osman17N. A. Fouad18N. E. Elkaraly19S. S. Mohamed20S. A. Tabra21W. A. Hassan22H. Abu Shady23Y. Amer24S. I. Nasef25Salwa Galal26Y. El Miedany27Cairo UniversityRheumatology and Rehabilitation Department, Faculty of Medicine, Tanta UniversityCairo UniversityAin Shams UniversityAin Shams UniversityCairo UniversityAlexandria UniversityCairo UniversityCairo UniversityMinia UniversitySohag UniversityMenoufia UniversityCairo UniversityAin Shams UniversityMansoura UniversityAlexandria UniversityZagazig UniversityAssiut UniversityFayoum UniversitySuez Canal UniversityCairo UniversityRheumatology and Rehabilitation Department, Faculty of Medicine, Tanta UniversityBenha UniversityCairo UniversityZagazig UniversitySuez Canal UniversityAin Shams UniversityAin Shams UniversityAbstract 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.https://doi.org/10.1186/s43166-022-00135-zMacrophage activation syndromeTreat to targetEgyptian guidelines MASAlgorithm
spellingShingle H. Lotfy
M. H. Abu-Zaid
S. Salah
M. El Gaafary
H. Abdulhady
H. Salah
E. Abd El-Latif
Y. Farag
M. Eissa
S. Esam Maher
A. Radwan
Amira T. El-Shanawany
B. M. Medhat
D. El Mikkawy
D. M. Mosa
G. El Deriny
M. Mortada
N. S. Osman
N. A. Fouad
N. E. Elkaraly
S. S. Mohamed
S. A. Tabra
W. A. Hassan
H. Abu Shady
Y. Amer
S. I. Nasef
Salwa Galal
Y. El Miedany
Egyptian evidence-based consensus on clinical practice guidelines for the diagnosis and treat-to-target management of macrophage activation syndrome in children
Egyptian Rheumatology and Rehabilitation
Macrophage activation syndrome
Treat to target
Egyptian guidelines MAS
Algorithm
title Egyptian evidence-based consensus on clinical practice guidelines for the diagnosis and treat-to-target management of macrophage activation syndrome in children
title_full Egyptian evidence-based consensus on clinical practice guidelines for the diagnosis and treat-to-target management of macrophage activation syndrome in children
title_fullStr Egyptian evidence-based consensus on clinical practice guidelines for the diagnosis and treat-to-target management of macrophage activation syndrome in children
title_full_unstemmed Egyptian evidence-based consensus on clinical practice guidelines for the diagnosis and treat-to-target management of macrophage activation syndrome in children
title_short Egyptian evidence-based consensus on clinical practice guidelines for the diagnosis and treat-to-target management of macrophage activation syndrome in children
title_sort egyptian evidence based consensus on clinical practice guidelines for the diagnosis and treat to target management of macrophage activation syndrome in children
topic Macrophage activation syndrome
Treat to target
Egyptian guidelines MAS
Algorithm
url https://doi.org/10.1186/s43166-022-00135-z
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