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Clinical aspects

 

Clinical, histologic and prognostic features of clinically amyopathic dermatomyositis

1, 2, 3, 4, 5, 6, 7, 8, 9, 10

 

  1. Unit of Rheumatology, Department of Precision and Regenerative Medicine, Area Jonica (DiMePRe-J), University of Bari, Italy.
  2. Unit of Human Anatomy and Histology, Department of Translational Biomedicine and Neuroscience “DiBraiN”, University of Bari, Italy.
  3. Physiologie et Explorations Fonctionnelles Musculaires, University Hospital of Strasbourg; UR3072 “Mitochondrie, Stress Oxydant Et Protection Musculaire”, Centre de Recherche en Biomédecine, University of Strasbourg; and Centre de Référence des Maladies Auto-immunes Systémiques Rares, University Hospital of Strasbourg, France.
  4. Unit of Rheumatology, Department of Precision and Regenerative Medicine, Area Jonica (DiMePRe-J), University of Bari, Italy.
  5. Unit of Rheumatology, Department of Precision and Regenerative Medicine, Area Jonica (DiMePRe-J), University of Bari, Italy.
  6. Unit of Rheumatology, Department of Precision and Regenerative Medicine, Area Jonica (DiMePRe-J), University of Bari, Italy.
  7. Institute of General Surgery "G Marinaccio", Department of Precision and Regenerative Medicine, Area Jonica (DiMePRe-J), University of Bari, Italy.
  8. Neurochemistry Lab, Department of Translational Biomedicine and Neuroscience “DiBraiN”, University of Bari, Italy.
  9. Neurochemistry Lab, Department of Translational Biomedicine and Neuroscience “DiBraiN”, University of Bari, Italy.
  10. Unit of Rheumatology, Department of Precision and Regenerative Medicine, Area Jonica (DiMePRe-J), University of Bari, Italy. florenzo.iannone@uniba.it

CER17218
2024 Vol.42, N°2
PI 0288, PF 0294
Clinical aspects

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PMID: 38488091 [PubMed]

Received: 19/10/2023
Accepted : 08/02/2024
In Press: 14/03/2024
Published: 14/03/2024

Abstract

OBJECTIVES:
To characterise clinical amyopathic dermatomyositis (CADM) from a clinical, histological, and prognostic perspective.
METHODS:
We retrospectively recorded data from our DM cohort. Patients were categorised into three groups: classic DM, hypomyopathic DM (HDM), characterised by normal muscle strength and evidence of muscle involvement in laboratory tests and/or instrumental examinations and CADM, featured by normal muscle strength and unremarkable findings in both laboratory tests and instrumental examinations. Available muscle biopsies from each group were also compared.
RESULTS:
Our cohort included 63 DM (69.2%), 12 HDM (13.2%) and 16 CADM (17.6%) patients. Compared to DM, CADM patients were younger at onset and diagnosis (45.5±17 vs. 57±18, and 46±17 vs. 58±18 years, respectively; p<0.05). They were more likely to test positive for anti-MDA5 (37.5% vs. 4.8%) and anti- TIF1-γ (31.3% vs. 6.3%), had a higher incidence of arthritis (37.5% vs. 12.6%) and interstitial lung disease (ILD) (43.8% vs. 15.9%) (all comparisons with p<0.05). Muscle biopsies were available for 44 DM, 7 CADM, and 11 HDM patients, revealing similar sarcolemma MHC-I expression rates. Five-year survival rates were comparable across groups (DM: 74.6%, CADM: 75%, HDM: 83.3%). Cox analysis indicated the main mortality predictors in overall cohort were ILD (HR: 3.57, CI: 1.11-11.5) and cancer (HR: 3.67, CI: 1.17-11.5), not CADM (HR: 1.46, CI: 0.33-6.68).
CONCLUSIONS:
CADM patients differ in disease onset, autoantibody profiles, joint and lung involvement. While laboratory and instrumental tests have not shown muscle involvement in CADM, many muscle biopsies have shown MHC-I overexpression.

DOI: https://doi.org/10.55563/clinexprheumatol/kgpnbq

Rheumatology Article