Clinical aspects
Anti-SAE dermatomyositis: clinical and histologic characteristics from a monocentric Italian cohort
M. Fornaro1, L. Coladonato2, M. Giannini3, A. Napoletano4, F. Girolamo5, D. D'abbicco6, M. Ruggieri7, D.V.A. Sabella8, F. Iannone9
- Unit of Rheumatology, Department of Precision and Regenerative Medicine, Area Jonica (DiMePRe-J), University of Bari, Italy.
- Unit of Rheumatology, Department of Precision and Regenerative Medicine, Area Jonica (DiMePRe-J), University of Bari, Italy.
- 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.
- Unit of Rheumatology, Department of Precision and Regenerative Medicine, Area Jonica (DiMePRe-J), University of Bari, Italy.
- Unit of Human Anatomy and Histology, Department of Translational Biomedicine and Neuroscience “DiBraiN”, University of Bari, Italy.
- Institute of General Surgery "G Marinaccio", Department of Precision and Regenerative Medicine, Area Jonica (DiMePRe-J), University of Bari, Italy.
- Neurochemistry Lab, Department of Translational Biomedicine and Neuroscience “DiBraiN”, University of Bari, Italy.
- Unit of Rheumatology, Department of Precision and Regenerative Medicine, Area Jonica (DiMePRe-J), University of Bari, Italy.
- Unit of Rheumatology, Department of Precision and Regenerative Medicine, Area Jonica (DiMePRe-J), University of Bari, Italy. florenzo.iannone@uniba.it
CER17451
2024 Vol.42, N°2
PI 0295, PF 0301
Clinical aspects
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PMID: 38488098 [PubMed]
Received: 08/01/2024
Accepted : 13/02/2024
In Press: 14/03/2024
Published: 14/03/2024
Abstract
OBJECTIVES:
Multiple myositis-specific antibodies have been identified, each associated with different clinical subsets of dermatomyositis (DM). Anti-SAE associated DM is considered the least studied subset. Our study aimed to evaluate the clinical and histological characteristics of DM patients with anti-SAE antibodies. As reference, patients with anti-Mi2 antibodies associated DM, representing a well-characterised subset, were analysed.
METHODS:
We recorded data from our DM cohort in the INflammatory MYositis REgistry (INMYRE). Patients were divided into two groups: those positive for anti-SAE and those positive for anti-Mi2 antibodies. Clinical characteristics, including skin, muscle, and extra-muscular involvements, were recorded. Available muscle biopsies were compared between the two groups.
RESULTS:
Of 92 DM patients, 10 (10.9%) were positive for anti-SAE and 17 (18.5%) for anti-Mi2. Anti-SAE positive DM patients showed classic DM findings but were characterised by a higher prevalence of skin itching (60% vs. 11.8%, p<0.01), shawl sign (40% vs. 5.9%, p<0.05) and lung involvement (30% vs. 0%, p<0.05) compared to anti-Mi2 positive patients. Furthermore, anti-SAE positive DM patients showed lower creatine kinase levels than those with anti-Mi2 (median [IQR]: 101 [58-647] vs. 1984 [974-3717], p<0.05) and a lower percentage of muscle fibre degeneration and necrosis (1.5%±1.7 vs. 5.9%±3.2, p<0.05) in muscle biopsies. No other differences were observed.
CONCLUSIONS:
Anti-SAE DM represents a disease subset characterised by classic cutaneous involvement often associated with itching, less severe muscle involvement, but potential pulmonary involvement that should always be investigated in these patients.
