Egyptian guideline on the management of paediatric, adolescent, and adult patients with idiopathic inflammatory myopathy and its major subgroups

Abstract

Background
The Idiopathic Inflammatory Myopathies (IIM) are a heterogeneous group of autoimmune diseases characterised by muscle inflammation (myositis), interstitial lung disease (ILD), and skin manifestations and many systemic manifestations, with high morbidity and functional disability. This document presents the first national, evidence-informed consensus guidelines for the diagnosis, treatment, and long-term surveillance of Inflammatory Myopathy, in paediatric, adolescent and adult Egyptian patients.


Methods
Twenty-two key clinical questions were identified by the scientific committee according to the Patient/ Population, Intervention, Comparison, Outcomes, and Timing (PICOT) approach. The Literature Review team performed a systematic review to summarize evidence advocating the diagnosis, assessment, and management of IIM. Subsequently, recommendations were formulated. The level of evidence was determined for each section using the Oxford Centre for Evidence-based Medicine (CEBM) system. A two-round Delphi process was conducted with 13 experts. All rounds were conducted online. A consensus was achieved on the direction and the strength of the recommendations.


Results
An online questionnaire was sent to expert panel who participated in the two rounds (response rate 100%). At the end of round 2, a total of 22 recommendation items stratified as 12 recommendations for diagnosis and assessment, and 10 recommendations for management. Consensus was reached (90% of respondents strongly agreed or agreed) on the wording of all 22 recommendations.


Conclusion
This study successfully culminated in the development of the first national, evidence-informed consensus guidelines for the diagnosis, treatment, and long-term surveillance of Idiopathic Inflammatory Myopathy (IIM) in Egyptian patients across all age groups.