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Review
. 2025 Jun 1;64(6):3288-3302.
doi: 10.1093/rheumatology/keaf116.

Treatment guidelines for idiopathic inflammatory myopathies in adults: a comparative review

Julie J Paik  1 , Victoria P Werth  2   3 , Hector Chinoy  4   5 , Karim R Masri  6 , Amruta Jambekar  7 , Feza Hasan  7 , Cecilia E Borlenghi  8 , David A Gold  9
Affiliations
Review

Treatment guidelines for idiopathic inflammatory myopathies in adults: a comparative review

Julie J Paik et al. Rheumatology (Oxford). .

Abstract

Myositis, or idiopathic inflammatory myopathy, encompasses a group of autoimmune diseases with broad-spectrum clinical presentations, with a common presentation of muscle weakness and inflammation. The management of myositis presents significant challenges due to the rarity and variability of the disease and lack of large-scale, randomized controlled trials. Due to limited evidence available from smaller studies as well as variation in treatment practices across geographical regions and disease subtypes, available published treatment recommendations vary significantly. There is a need, therefore, to develop multidisciplinary consensus-driven guidelines that appropriately reflect the diverse and complex nature of the disease. This comparative review presents an in-depth analysis of existing myositis treatment guidelines from diverse organizations, highlighting similarities and key differences in diagnoses, treatment and management recommendations. We propose that there is a need for developing globally unified, consensus-driven standardized set of guidelines for effective myositis management.

Keywords: dermatomyositis; guidelines; idiopathic inflammatory myopathy; myositis; treatment recommendations.

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Figures

None
Graphical abstract
Figure 1.
Figure 1.
Myositis subtypes: clinical and demographic characteristics. aCutaneous features include heliotrope, shawl sign, Gottron’s papules, etc. Autoantibody target molecules: CCAR1: cell division cycle and apoptosis regulator 1; cN1A: cytosolic 5′-nucleotidase 1A; EJ: glycyl-tRNA synthetase; YRS/HA: tyrosyl-tRNA synthetase; HMGCR: 3-hydroxy-3-methyl-glutaryl-coenzyme A reductase; Jo1: anti-histidyl-transfer RNA synthetase; KS: asparaginyl-tRNA synthetase; Ku: Ku70/Ku80 DNA-binding protein; MDA5: melanoma differentiation-associated protein 5; Mi2: helicase protein; NXP2: nuclear matrix protein 2; OJ: isoleucyl-tRNA synthetase; PL7: threonyl-tRNA synthetase; PL12: alanyl-tRNA synthetase; PM/Scl: PM-scleroderma; Ro52: Ro52 polypeptide; SAE: small ubiquitin-like modifier activating enzyme; Sp4: specificity protein 4; SRP: signal recognition particle; TIF1γ: transcriptional intermediary factor 1 gamma; U1RNP: U1 ribonucleoprotein; ZO: phenylalanyl-tRNA synthetase; ADM: amyopathic dermatomyositis; ILD: interstitial lung disease
Figure 2.
Figure 2.
Classification criteria of myositis over time. ADM: amyopathic dermatomyositis; ASyS: antisynthetase syndrome; ENMC: European Neuromuscular Centre; IMNM: immune-mediated necrotizing myopathy
See this image and copyright information in PMC

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