Late onset inflammatory myopathy associated with Anti-Ro52 antibodies without lung involvement: A case report
Keywords:Inflammatory myopathies, Dermatomyositis, Anti-Ro52, Systemic neoplasm, Interstitial lung disease
Introduction. Idiopathic inflammatory myopathies are a heterogeneous group of autoinmune diseases. Clinically they are characterized by symmetric muscle weakness and sometimes systemic involvement. They present neurophysiological and histological signs of chronic muscle inflammation, as well as elevation of muscle enzymes. Specific autoantibodies are useful biomarkers for diagnosis. We present the case of a patient with dermatomyositis (DM) and positive anti-Ro52 antibodies without systemic activity at diagnosis.
Clinical case. An 87-year-old woman with a history of basal cell carcinoma in the left malar region and hypothyroidism, both under control. Presented a progressive disease which began 6 months prior to the evaluation by our department, characterized by fatigue, weakness of proximal muscles in the four extremities, associated with myalgia in the pelvic girdle. Over the course of 2 months, showed difficulty performing instrumented activities of daily living. At the fifth month, she manifested dysphagia. On physical examination, symmetric weakness was documented, predominantly proximal, without alteration of the muscle stretch reflexes or sensory involvement. Laboratory analysis showed CK elevation (2974 U/L). A panel of antibodies was made, finding anti Ro52 +++. Body PET revealed hypometabolism in the proximal muscles of the four extremities, with no evidence of neoplastic activity. A muscle biopsy was performed, observing findings compatible with DM. Management began with methylprednisolone pulses, presenting symptomatic improvement.
Discussion and Conclusions: The association of anti-Ro52 antibodies and DM is rare, occurring in up to 26% in overlapping syndromes. The presentation of interstitial lung disease and cáncer have a poor prognosis
Lundberg IE, Fujimoto M, Vencovsky J, Aggarwal R, Holmqvist M, Christopher-Stine L, Mammen AL, Miller FW. Idiopathic inflammatory myopathies. Nat Rev Dis Primers. 2021 Dec 2;7(1):86. DOI: 10.1038/s41572-021-00321-x
Mariampillai K, Granger B, Amelin D, Guiguet M, Hachulla E, Maurier F, Meyer A, Tohmé A, Charuel JL, Musset L, Allenbach Y, Benveniste O. Development of a New Classification System for Idiopathic Inflammatory Myopathies Based on Clinical Manifestations and Myositis-Specific Autoantibodies. JAMA Neurol. 2018 Dec 1;75(12):1528-1537. DOI: 10.1001/jamaneurol.2018.2598
Yoo IS, Kim J, The Role of Autoantibodies in Idiopathic Inflammatory Myopathies, J Rheum Dis 2019; 26(3): 165-178 URL: https://www.jrd.or.kr/journal/view.html?uid=1392&vmd=Full
Satoh M, Tanaka S, Ceribelli A, Calise SJ, Chan EK. A Comprehensive Overview on Myositis-Specific Antibodies: New and Old Biomarkers in Idiopathic Inflammatory Myopathy. Clin Rev Allergy Immunol. 2017 Feb;52(1):1-19. DOI: 10.1007/s12016-015-8510-y
Sabouri A, Christopher-Stine L, Kafaie J, Inflammatory Myopathies. Clinicopathologic classification can aid understanding and guide treatment. Practical Neurology, 2021 URL: https://practicalneurology.com/articles/2021-july-aug/inflammatory-myopathies
Yang SH, Chang C, Lian ZX. Polymyositis and dermatomyositis - challenges in diagnosis and management. J Transl Autoimmun. 2019 Oct 8;2:100018. DOI: 10.1016/j.jtauto.2019.100018
DeWane ME, Waldman R, Lu J. Dermatomyositis: Clinical features and pathogenesis. J Am Acad Dermatol. 2020 Feb;82(2):267-281. DOI: 10.1016/j.jaad.2019.06.1309
Waldman R, DeWane ME, Lu J. Dermatomyositis: Diagnosis and treatment. J Am Acad Dermatol. 2020 Feb;82(2):283-296. DOI: 10.1016/j.jaad.2019.05.105
Bielsa Marsol I. Dermatomiositis [Dermatomyositis]. Reumatol Clin. 2009 Sep-Oct;5(5):216-22. DOI: 10.1016/j.reuma.2008.09.004
Cobos GA, Femia A, Vleugels RA. Dermatomyositis: An Update on Diagnosis and Treatment. Am J Clin Dermatol. 2020 Jun;21(3):339-353. DOI: 10.1007/s40257-020-00502-6
How to Cite
Copyright (c) 2022 Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
September 2022-present © Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez. Open access articles under the terms of the Creative Commons Attribution-NonCommercial 4.0 International (CC BY-NC 4.0) license, which permits use, distribution and reproduction in any medium, provided the original work is properly cited. No commercial re-use is allowed.
January-September 2022 © The authors. Open access articles under the terms of the Creative Commons Attribution-NonCommercial 4.0 International (CC BY-NC 4.0) license, which permits use, distribution and reproduction in any medium, provided the original work is properly cited. No commercial re-use is allowed.
January 2014-December 2021 © Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez. Open access articles under the terms of the Creative Commons Attribution 4.0 International (CC BY 4.0) license, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.