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Allenbach, Yves; Leroux, Gaëlle; Suárez-Calvet, Xavier; Preusse, Corinna; Gallardo, Eduard; Hervier, Baptiste; Rigolet, Aude; Hie, Miguel; Pehl, Debora; Limal, Nicolas; Hufnagl, Peter; Zerbe, Norman; Meyer, Alain; Aouizerate, Jessie; Uzunhan, Yurdagul; Maisonobe, Thierry; Goebel, Hans-Hilmar; Benveniste, Olivier; Stenzel, Werner
The American journal of pathology, March 2016, Volume: 186, Issue: 3Journal Article
The anti-melanoma differentiation-associated gene 5 (MDA5) autoantibody is specifically associated with dermatomyositis (DM). Nevertheless, anti–MDA5+ -patients experience characteristic symptoms distinct from classic DM, including severe signs of extramuscular involvement; however, the clinical signs of myopathy are mild or even absent. The morphological and immunological features are not yet described in adulthood. Data concerning the pathophysiology of anti-MDA5 DM are sparse; however, the importance of the interferon (IFN) type I pathway involved in DM has been shown. Our aim was to define morphological alterations of the skeletal muscle and the intrinsic immune response of anti–MDA5-positive DM patients. Immunohistological and RT-PCR analysis of muscle biopsy specimens from anti-MDA5 and classic DM were compared. Those with anti-MDA5 DM did not present the classic features of perifascicular fiber atrophy and major histocompatibility complex class I expression. They did not show significant signs of capillary loss; tubuloreticular formations were observed less frequently. Inflammation was focal, clustering around single vessels but significantly less intense. Expression of IFN-stimulated genes was up-regulated in anti-MDA5 DM; however, the IFN score was significantly lower. Characteristic features were observed in anti-MDA5 DM and not in classic DM patients. Only anti-MDA5 DM showed numerous nitric oxide synthase 2–positive muscle fibers with sarcoplasmic colocalization of markers of regeneration and cell stress. Anti–MDA5-positive patients demonstrate a morphological pattern distinct from classic DM.
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