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Respiratory failure in systemic sclerosis

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Abstract

Systemic sclerosis (SSc) can lead to dyspnea and respiratory failure through multiple mechanisms, making a precise diagnosis particularly challenging, especially amid the current COVID-19 pandemic. In this report, we present a case involving a 26-year-old female who had previously undiagnosed SSc. She experienced acute respiratory failure necessitating orotracheal intubation. Following an extensive evaluation, the patient exhibited skin thickening, kidney failure, thrombocytopenia, microangiopathic anemia, and an antinuclear antibody with a nuclear fine speckled pattern at a titer of 1:320. A diagnosis of SSc complicated by scleroderma renal crisis (SRC) was established. The patient’s condition improved after undergoing hemodialysis, receiving an angiotensin-converting enzyme inhibitor, and undergoing cyclophosphamide treatment. Subsequently, she demonstrated sustained improvement during a follow-up period of 20 months.

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Acknowledgements

JIVDL and ASF collected the data, analyzed the results, and wrote the manuscript; PDSB, RM, ACMR, RMRP, and APLA designed the study, analyzed the results, critically reviewed the manuscript, and contributed to the final version.

Funding

This research did not receive any specific grants from funding agencies in the public, commercial, or not-for-profit sectors.

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Authors and Affiliations

  1. Division of Rheumatology, Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Av. Dr. Arnaldo, 455, 3O. Andar, Cerqueira César, Sao Paulo, SP, CEP: 01246-903, Brazil

    Joaquim Ivo Vasques Dantas Landim, Andre Silva Franco, Percival Degrava Sampaio-Barros, Renata Miossi, Ana Cristina Medeiros-Ribeiro, Rosa Maria R. Pereira & Ana Paula Luppino Assad

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  1. Joaquim Ivo Vasques Dantas Landim
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  2. Andre Silva Franco
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Correspondence to Joaquim Ivo Vasques Dantas Landim.

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Compliance with ethical standards

All the procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee. The patient signed an informed consent form authorizing the use of the image and access to her electronic medical record.

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Rosa Maria R. Pereira: In memoriam.

RMRP is deceased.

Special thanks to Dr. Ricardo Manoel de Oliveira and the RDO Diagnósticos Médicos laboratory for conducting the anti-RNA-polymerase III assay.

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Landim, J.I.V.D., Franco, A.S., Sampaio-Barros, P.D. et al. Respiratory failure in systemic sclerosis. Rheumatol Int (2023). https://doi.org/10.1007/s00296-023-05482-4

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