Journalartikel
Autorenliste: Schaenzer, Anne; Rager, Leonie; Dahlhaus, Iris; Dittmayer, Carsten; Preusse, Corinna; Della Marina, Adela; Goebel, Hans-Hilmar; Hahn, Andreas; Stenzel, Werner
Jahr der Veröffentlichung: 2022
Zeitschrift: Cells
Bandnummer: 11
Heftnummer: 1
eISSN: 2073-4409
Open Access Status: Gold
DOI Link: https://doi.org/10.3390/cells11010109
Verlag: MDPI
Abstract:
Background: In juvenile idiopathic inflammatory myopathies (IIMs), morphological characteristic features of distinct subgroups are not well defined. New treatment strategies require a precise diagnosis of the subgroups in IIM, and, therefore, knowledge about the pathomorphology of juvenile IIMs is warranted. Methods: Muscle biopsies from 15 patients (median age 8 (range 3-17) years, 73% female) with IIM and seven controls were analyzed by standard methods, immunohistochemistry, and transmission electron microscopy (TEM). Detailed clinical and laboratory data were accessed retrospectively. Results: Proximal muscle weakness and skin symptoms were the main clinical symptoms. Dermatomyositis (DM) was diagnosed in 9/15, antisynthetase syndrome (ASyS) in 4/15, and overlap myositis (OM) in 2/15. Analysis of skeletal muscle tissues showed inflammatory cells and diffuse upregulation of MHC class I in all subtypes. Morphological key findings were COX-deficient fibers as a striking pathology in DM and perimysial alkaline phosphatase positivity in anti-Jo-1-ASyS. Vascular staining of the type 1 IFN-surrogate marker, MxA, correlated with endothelial tubuloreticular inclusions in both groups. None of these specific morphological findings were present in anti-PL7-ASyS or OM patients. Conclusions: Morphological characteristics discriminate IIM subtypes in juvenile patients, emphasizing differences in aetiopathogenesis and supporting the notion of individual and targeted therapeutic strategies.
Zitierstile
Harvard-Zitierstil: Schaenzer, A., Rager, L., Dahlhaus, I., Dittmayer, C., Preusse, C., Della Marina, A., et al. (2022) Morphological Characteristics of Idiopathic Inflammatory Myopathies in Juvenile Patients, Cells, 11(1), Article 109. https://doi.org/10.3390/cells11010109
APA-Zitierstil: Schaenzer, A., Rager, L., Dahlhaus, I., Dittmayer, C., Preusse, C., Della Marina, A., Goebel, H., Hahn, A., & Stenzel, W. (2022). Morphological Characteristics of Idiopathic Inflammatory Myopathies in Juvenile Patients. Cells. 11(1), Article 109. https://doi.org/10.3390/cells11010109
Schlagwörter
ANTI-3-HYDROXY-3-METHYLGLUTARYL-COENZYME; Anti-synthetase syndrome; DERMATOMYOSITIS; juvenile; muscle pathology; MYOSITIS; MYOSITIS-SPECIFIC AUTOANTIBODIES; overlap myositis; phenotypes
