Neumomediastino como complicación de enfermedad pulmonar intersticial en dermatomiositis

Pneumomediastinum as a complication of interstitial lung disease in dermatomyositis

Introducción: la dermatomiositis es una miopatía inflamatoria idiopática, heterogénea de difícil diagnóstico. Se asocia con manifestaciones cutáneas y se considera una enfermedad variable por la presentación clínica, el curso y el pronóstico. Además del músculo y la piel, puede comprometer diferentes órganos y sistemas. Caso clínico: paciente de 56 años con antecedente de dermatomiositis quien ingresó a urgencias con disfagia para sólidos y líquidos, astenia y adinamia, con evidencia de neumomediastino y consolidaciones multilobares en la tomografía del tórax, con mejoría de los síntomas después del manejo con antibiótico e inicio de metotrexate. Discusión:  no se han descrito muchos casos sobre neumomediastino en contexto de enfermedad intersticial asociada con dermatomiositis, por lo que es relevante estudiar esta patología. El paciente no refirió síntomas respiratorios, el estudio endoscópico y el esófagoTAC, no revelaron perforación esofágica por lo que neumología atribuyó el cuadro a efecto Macklin. Conclusión: el neumomediastino espontáneo es una complicación poco común con alta tasa de mortalidad, sin embargo puede ocurrir en pacientes con enfermedad pulmonar intersticial. 

1. DeWane ME, Waldman R, Lu J. Dermatomyositis: Clinical features and pathogenesis. J Am Acad Dermatol. 2020;82(2):267–81. http://dx.doi.org/10.1016/j.jaad.2019.06.1309
2. Chu LL, Rohekar G. Dermatomyositis. Can Med Assoc J. 2019;191(12):E340–E340. http://dx.doi.org/10.1503/cmaj.180947
3. Sun Y, Li DF, Zhang YL, Liang X, Li TF. Characterisation of Disease Patterns of Dermatomyositis with Different Initial Manifestations. Int J Gen Med. 2022;15:6519–28. http://dx.doi.org/10.2147/IJGM.S372658
4. Santos-Briz A, Calle A, Linos K, Semans B, Carlson A, Sangüeza OP, et al. Dermatomyositis panniculitis: a clinicopathological and immunohistochemical study of 18 cases. J Eur Acad Dermatol Venereol. 2018;32(8):1352–9. http://dx.doi.org/10.1111/jdv.14932
5. Sasaki H, Kohsaka H. Current diagnosis and treatment of polymyositis and dermatomyositis. Mod Rheumatol. 2018;28(6):913–921. http://dx.doi.org/10.1080/14397595.2018.1467257
6. Kato M, Ikeda K, Kageyama T, Kasuya T, Kumagai T, Furuya H, et al. Successful Treatment for Refractory Interstitial Lung Disease and Pneumomediastinum With Multidisciplinary Therapy Including Tofacitinib in a Patient With Anti-MDA5 Antibody-Positive Dermatomyositis. J Clin Rheumatol. 2021;27(8S):S574–577. http://dx.doi.org/10.1097/RHU.0000000000000984
7. Charry LP, Alejo A, Cantillo M, Fernández-Ávila DG. Caracterización de pacientes con dermatomiositis en un hospital universitario en Colombia. Rev Colomb Reumatol. 2023;30(1):47–55. https://doi.org/10.1016/j.rcreu.2021.05.019
8. Li C, Liang M, Jiang H, Zhao J, Wu C, Wang Q, Zhang L, Zhao Y. The long-term prognosis of pneumomediastinum associated with dermatomyositis: a two-centre retrospective cohort study. Rheumatology. 2021;60(5):2288–2295. https://doi.org/10.1093/rheumatology/keaa582
9. Zhou C, Gao C, Xie Y, Xu M. COVID-19 with spontaneous pneumomediastinum. Lancet Infect Dis. 2020;20(4):510. https://doi.org/10.1016/S1473-3099(20)30156-0
10. Chen M, Quan C, Diao L, Xue F, Xue K, Wang B, et al. Measurement of cytokines and chemokines and association with clinical severity of dermatomyositis and clinically amyopathic dermatomyositis. Br J Dermatol. 2018;179(6):1334–41. https://doi.org/10.1111/bjd.17079
11. Cerna-López JA, Tejada-Llacsa PJ, Valle-Farfán FA de J, Alarcón GS, Ugarte-Gil MF, Pimentel-Quiroz VR. Rapidly progressive interstitial lung disease-associated hypomyopathic dermatomyositis complicated with pneumomediastinum: A case-based review. Rev Colomb Reumatología. 2023;30(3):277-282. https://doi.org/10.1016/j.rcreu.2021.06.001
12. Yamaguchi K, Yamaguchi A, Itai M, Kashiwagi C, Takehara K, Aoki S, et al. Clinical features of patients with anti-melanoma differentiation-associated gene-5 antibody-positive dermatomyositis complicated by spontaneous pneumomediastinum. Clin Rheumatol. 2019;38(12):3443–50. https://doi.org/10.1007/s10067-019-04729-5
13. Abe K, Furuta S, Kobayashi Y, Sugiyama T, Kagami SI, Nakagomi D, et al. Prognosis of spontaneous pneumomediastinum occurring in dermatomyositis or polymyositis patients with interstitial lung disease according to antimelanoma differentiation-associated gene 5 antibody status: a retrospective cohort study. RMD Open. 2023;9(1):e002770. https://doi.org/10.1136/rmdopen-2022-002770
14. De Giacomi F, Baqir M, Cox CW, Moua T, Matteson EL, Ryu JH. Spontaneous Pneumomediastinum in Connective Tissue Diseases. J Clin Rheumatol. 2019;25(6):239–45. https://doi.org/10.1097/RHU.0000000000000835
15. Yoo H, Hino T, Hwang J, Franks TJ, Han J, Im Y, et al. Connective tissue disease-related interstitial lung disease (CTD-ILD) and interstitial lung abnormality (ILA): Evolving concept of CT findings, pathology and management. Eur J Radiol Open. 2022;9:100419. https://doi.org/10.1016/j.ejro.2022.100419
16. Alemu BN, Yeheyis ET, Tiruneh AG. Spontaneous primary pneumomediastinum: is it always benign? J Med Case Rep. 2021;15(1):157. https://doi.org/10.1186/s13256-021-02701-z
17. Connors GR, Christopher-Stine L, Oddis CV, Danoff SK. Interstitial lung disease associated with the idiopathic inflammatory myopathies: what progress has been made in the past 35 years? Chest. 2010;138(6):1464–74. https://doi.org/10.1378/chest.10-0180
18. Murayama S, Gibo S. Spontaneous pneumomediastinum and Macklin effect: Overview and appearance on computed tomography. World J Radiol. 2014;6(11):850-854. https://doi.org/10.4329/wjr.v6.i11.850
19. Macklin MT, Macklin CC. Malignant interstitial emphysema of the lungs and mediastinum as an important occult complication in many respiratory diseases and other conditions. Medicine. 1944;23(4):281–358.
20. Gutsche M, Rosen GD, Swigris JJ. Connective Tissue Disease-associated Interstitial Lung Disease: A review. Curr Respir Care Rep. 2012;1(4):224–32. https://doi.org/10.1007/s13665-012-0028-7
21. Wu W, Guo L, Fu Y, Wang K, Zhang D, Xu W, et al. Interstitial Lung Disease in Anti-MDA5 Positive Dermatomyositis. Clin Rev Allergy Immunol. 2021;60(2):293–304. https://doi.org/10.1007/s12016-020-08822-5