Work

Candidate molecular endotypes and dynamic risk prediction in anti-MDA5-positive dermatomyositis-associated interstitial lung disease: a systematic scoping review

Year: 2026
Type: review
Study design: Systematic Review
Abstract: Background: Anti-melanoma differentiation-associated protein 5 antibody-positive dermatomyositis (anti-MDA5+ DM) spans pulmonary states from no detectable interstitial lung disease (ILD) to rapidly progressive ILD (RP-ILD). We mapped molecular programs and prediction evidence to determine how recurrent biological states may support dynamic stratification. Methods: PubMed, Web of Science, Ovid and Embase were searched from inception to 22 August 2026. Two reviewers independently screened records and extracted data. Reporting followed PRISMA-ScR; evidence was synthesized across molecular and prediction domains. Results: Of 4, 166 records, 2, 317 remained after deduplication, 349 underwent full-text assessment, and 95 sources (80 primary studies and 15 syntheses) were included. Type I/III interferon signaling was the most reproducible program. Myeloid inflammatory-remodeling, adaptive activation with lymphocyte or natural-killer-cell loss, vascular-coagulation injury, and immunometabolic disturbance formed additional overlapping axes aligned with pulmonary-state transitions. Clinical and radiomic models showed strong short-term discrimination; harmonized landmarks, calibration, geographic validation, and decision-timed molecular sampling would strengthen clinical use. Conclusion: The evidence supports an integrated map linking recurrent molecular programs to dynamic pulmonary risk. A calibrated clinical-imaging backbone, augmented by parsimonious molecular modules with incremental value and updated at prespecified landmarks, offers a practical framework for prospective endotype validation and treatment-aware prediction in anti-MDA5+ DM-ILD. Systematic review registration: https://doi.org/10.37766/inplasy2026.10.0018, identifier INPLASY2026100018.
Language: English
Subfield: Rheumatology
Field: Medicine
Open Access status: gold
Estimated APC paid: $3,801
OpenAlex ID: W7221065629
FWCI:
Cites: 111
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Related to: 10