Abstract
The authors present the case of a 76-year-old female patient with progressive decrease in proximal muscle strength, fatigue, dyspnea, diffuse hand edema and painful triphasic Raynaud’s phenomenon. Anti-SRP and anti-SSA antibodies were detected, muscle biopsy revealed changes consistent with necrotizing myopathy and capillaroscopy had findings compatible with systemic sclerosis. High-resolution chest computed tomography revealed interstitial lung disease with a non-specific interstitial pneumonia pattern. Lung function tests demonstrated a forced vital capacity 93% and a diffusing capacity for carbon monoxide of 65% predicted. After multidisciplinary discussion, she was diagnosed with immune-mediated necrotizing myopathy/systemic sclerosis overlap syndrome with pulmonary involvement. Initially, dual immunomodulation therapy with high-dose steroids and intravenous immunoglobulin was started, but after 4 weeks, the patient had clinical and analytical deterioration. At this time, she was started on rituximab, with an excellent and sustained response at both muscle and lung, sustained after 12 months.
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CM prepared the first version of the manuscript. Manuscript revision was performed by all the authors. All authors gave final approval of the version to be published.
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Carolina Mazeda and Rita Cunha declare that they have no conflict of interest. Renata Aguiar has received honoraria for speaking from UCB and Bial. Pedro Gonçalo Ferreira has received honoraria for speaking from Boehringer-Ingelheim, Roche, Medinfar, Glaxo, Novartis, Menarini and Mundipharma. Pedro Gonçalo Ferreira has received honoraria for consultancy from Boehringer-Ingelheim and Roche. Anabela Barcelos has received honoraria for speaking at symposia/position on advisory board: MSD, Novartis, Lilly, Pfizer. Carolina Mazeda is the corresponding author. She takes responsibility for the integrity of the data and data analysis accuracy.
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Mazeda, C., Cunha, R., Ferreira, P.G. et al. Myopathy associated with anti-signal recognition particle antibodies with pulmonary involvement and response to rituximab. Rheumatol Int 42, 1265–1269 (2022). https://doi.org/10.1007/s00296-021-04904-5
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DOI: https://doi.org/10.1007/s00296-021-04904-5