Pulmonary Fibrosis and the Antisynthetase Syndrome: a Case Report

Main Article Content

Analia Canepa
https://orcid.org/0000-0002-9275-6467
Santiago Moyano
https://orcid.org/0000-0003-4200-3480
María Florencia Artola
Martín Rebella
https://orcid.org/0000-0002-1925-4022
Manuel Baz

Abstract

Antisynthetase syndrome is a rare disease that usually presents with different degrees of muscle, skin, joint and lung involvement depending on the profile of autoantibodies involved in its pathogenesis. Lung involvement can be serious and have a progressive fibrosing pattern with important prognostic and therapeutic implications, so its early detection through timely screening based on high-resolution chest tomography and respiratory functional study is essential. The clinical case of a patient with a diagnosis of anti-Jo1 positive inflammatory myopathy refractory to multiple immunosuppressive treatments that establish subacute respiratory failure is presented, leading to the diagnosis of fibrosing interstitial lung disease and presenting a good response to treatment with nintedanib.

Article Details

Section

Casos clínicos

How to Cite

Pulmonary Fibrosis and the Antisynthetase Syndrome: a Case Report. (2025). Respirar, 17(2), 201-206. https://doi.org/10.55720/respirar.17.2.11

References

Alberti ML, Wolff V, Reyes F et al. Enfermedad pulmonar intersticial asociada a miositis: características clínicas y factores relacionados con la mejora de la función pulmonar. Un estudio de cohorte multicéntrico latinoamericano. Clínica Reumatol 2022;18: 293–29. Doi: 10.1016/j.reuma.2020.12.002

Betteridge Z, Tansley S, Shaddick G et al. Frequency, mutual exclusivity and clinical associations of myositis autoantibodies in a combined European cohort of idiopathic inflammatory myopathy patients. J Autoinmune 2019;101:48–55. Doi: 10.1016/j.jaut.2019.04.001

Zamarrón-de Lucas E, Gómez Carrera L, Bonilla G et al. Síndrome antisintetasa: análisis de 11 casos. Med Clin (Barc) 2017;148(4):166–169. Doi: 10.1016/j.medcli.2016.11.021

Nuno-Nuno L. Síndrome antisintetasa. Tratado SER de diagnóstico y tratamiento de las enfermedades autoinmunes sistémicas. Cap 37. Editorial Médica Panamericana, 2018; pp. 250-254.

Gardenia R, Sola MF, Giardullo C et al. Síndrome antisintetasa: reporte de un caso. Rev Argent Reumatol vol.33 no.1 2022;33:1. Doi: 10.47196/rar.v33i1.372

Zanatta E, Cocconcelli E, Castelli G et al. Interstitial lung disease with and without progressive fibrosing phenotype in patients with idiopathic inflammatory myopathies: data from a large multicentric cohort. RMD Open 2023;9:e003121. Doi: 10.1136/rmdopen-2023-003121

Flaherty KR, Wells AU, Cottin V et al. Nintedanib in Progressive Fibrosing Interstitial Lung Diseases. N Engl J Med 2019;381(18):1718-1727. Doi: 10.1056/NEJMoa1908681.

Similar Articles

You may also start an advanced similarity search for this article.

Most read articles by the same author(s)