To describe the characteristics and assess the quality of clinical practice guidelines (CPG) for diagnosis and treatment in adult myasthenia gravis (MG) patients.
MG is a rare autoimmune neuromuscular disease with heterogeneous clinical presentation and treatment response. These characteristics and the rapid development of new therapies are approached by evidence-based MG CPG. However, their methodological quality and consistency remain uncertain.
We conducted a scoping review of CPG offering specific recommendations for MG diagnostic and/or treatment in adults. On July 9th, 2025, we searched seven databases and repositories, including PubMed and TripDatabase, for CPGs published or updated in the last six years. We used the Appraisal of Guidelines Research and Evaluation II (AGREE-II) tool to assess methodological quality, focusing on Domain 3 (Rigor of Development).
Ten CPG met inclusion criteria. Guidelines originated from the United States (n=3), Asia (n=3), Europe (n=3), and Latin-America (n=1). The median AGREE-II Domain 3 score was 52.5% (IQR: 31%-60%). Five CPG achieved ≥60% in AGREE-II Domain 3, reporting systematic reviews during development. Among their 112 recommendations, 11 from 1 CPG addressed MG diagnosis. Other two CPG formulated 21 and 5 recommendations for clinical assessment and therapeutic goals in MG patients, respectively. Only one CPG gave two recommendations for pyridostigmine. Three CPG presented 16 recommendations for classic immunosuppressants (oral corticosteroids, cyclosporine, tacrolimus, azathioprine, mycophenolate, and methotrexate) and 9 recommendations for biologics (rituximab, eculizumab, ravulizumab, and efgartigimod) with limited consensus between them. Thymectomy age-indications were conflicted (less than 50, 60 and 65 years old). Finally, 23 recommendations for myasthenic crisis or symptoms exacerbation focused on intensive care, intravenous immunoglobulin, and plasmapheresis.