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041 _afre
042 _adc
100 1 0 _aDidona, Dario
_eauthor
700 1 0 _a Juratli, Hazem A.
_eauthor
700 1 0 _a Scarsella, Luca
_eauthor
700 1 0 _a Keber, Ursula
_eauthor
700 1 0 _a Eming, RĂ¼diger
_eauthor
700 1 0 _a Hertl, Michael
_eauthor
245 0 0 _aAmyopathic and anti-TIF1 gamma-positive dermatomyositis: analysis of a monocentric cohort and proposal to update diagnostic criteria
260 _c2020.
500 _a24
520 _aBackground: Dermatomyositis (DM) is a group of autoimmune diseases characterized by a variable degree of skin symptoms and myopathy. An amyopathic form of DM (ADM) has been described, and more recently, an anti-TIF-1 gamma-positive subtype, characterized by poikiloderma and associated with a relatively high risk of cancer. Objectives: To characterise a cohort of DM patients. Material and Methods: A cohort of 29 DM patients was followed between January 2004 and March 2019, and investigated for clinical characteristics, pathological features based on electromyography and MRI, laboratory data, and auto-antibody profile. Results: Based on the investigations, DM was shown to be heterogeneous. However, we identified a subgroup of anti-TIF-1 gamma-positive patients who all shared heliotrope erythema. Furthermore, we observed a positive correlation between serum glutamicoxaloacetic transaminase (GOT) and creatine kinase (CK) concentrations in patients with anti-TIF-1 gamma antibodies, which is not found in patients with anti-MDA-5 antibodies. Conclusion: Based on the findings of this study, we propose an update of the Sontheimer et al. diagnostic criteria to improve the sensitivity of diagnosis for ADM. In addition, we describe a significant association between serum GOT and CK levels in DM patients with anti-TIF-1 gamma antibodies, and further highlight the significance of heliotrope rash as a clinical hallmark for this particular subset of patients.
690 _aheliotrope erythema
690 _acreatine kinase (CK)
690 _aamyopathic dermatomyositis (ADM)
690 _aglutamicoxaloacetic transaminase (GOT)
690 _adermatomyositis
690 _aanti-TIF-1 gamma-positive subtype
786 0 _nEuropean Journal of Dermatology | 30 | 3 | 2020-05-01 | p. 279-288 | 1167-1122
856 4 1 _uhttps://shs.cairn.info/revue-european-journal-of-dermatology-2020-3-page-279?lang=fr&redirect-ssocas=7080
999 _c602747
_d602747