000 | 02525cam a2200289 4500500 | ||
---|---|---|---|
005 | 20250121154613.0 | ||
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 |