Spontaneous Pneumomediastinum in Amylopathic Dermatomyositis - A Rare Manifestation

Vico, Laís De; Canaan, João Paulo; Lanças, Sean Hideo Shirata; Brufatto, Matheus Zanata; Toledo, Joely Cristina Da Silva; Oliveira, Lívia Câmara; Pacheco, Sula Glaucia Lage Drumond; Batista, Andréa de Almeida Peduti; Valente, Luiz Eduardo; Leite, F.V.; Sampaio, Henrique Pereira

doi:10.5151/sbr2019-258

Cited by 1 publication

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“…Similarly, this mechanism can explain the occurrence of pneumomediastinum in DM patients with ILD reported in the literature. 5,6,13,16,[18][19][20][21][22][25][26][27][33][34][35][36][37][38][39] However, it is inadequate in patients who develop pneumomediastinum in the absence of any parenchymatous diseases or ILD, such as our second patient and the cases reported by Shimamoto et al, 20 Yoshida et al, 26 and Tang et al 29 Accordingly, other mechanisms were suggested.…”

Section: Relevant Hypothesis Generationmentioning

confidence: 98%

“…24 Though several case reports, case series, and review articles on DM and pneumomediastinum were reported in the literature, 6,[12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30] only a few cases of DM had both pneumomediastinum and emphysema. 5,6,13,16,[18][19][20][21][22][25][26][27][33][34][35][36][37][38][39] Table 1 summarizes the main clinical features, treatment strategies, and outcomes of these cases. 5,6,13,16,[18][19][20][21][22]…”

Section: Reference To Relevant Literaturementioning

confidence: 99%

“…5,6,13,16,[18][19][20][21][22][25][26][27][33][34][35][36][37][38][39] Table 1 summarizes the main clinical features, treatment strategies, and outcomes of these cases. 5,6,13,16,[18][19][20][21][22][25][26][27][33][34][35][36][37][38][39] Similar to our patients, the clinical course and presentation of the reported cases in the literature were highly variable. Combined pneumomediastinum and emphysema occurred in both males and females, over a wide age range (from 10 to 65 years), and at a varied range of disease duration (as short as one month 5 and up to four years from disease onset) 36 .…”

Section: Reference To Relevant Literaturementioning

confidence: 99%

“…Creatine kinase levels, immunology panel, treatments given, and disease outcomes were also heterogenous as detailed in Table 1. 5,6,13,16,[18][19][20][21][22][25][26][27][33][34][35][36][37][38][39] Comparison to the Current Gold Standard of Care…”

Section: Reference To Relevant Literaturementioning

confidence: 99%

“…Conservative management, mechanical ventilation, steroids, IVIG, and various immunosuppressants have been tried with conflicting results. 5,6,13,16,[18][19][20][21][22][25][26][27][33][34][35][36][37][38][39] The outcome of pneumomediastinum in patients with DM was variable in the literature, ie, corticosteroids and immunosuppressive therapy resulted in complete recovery in some cases, 11 while others had fatal outcomes despite aggressive therapy. 5,14,31,32 In our patients, we adopted treatment strategies as reported in the literature, ie, corticosteroids, 20 IVIG, 35 MMF, 17 and MTX.…”

Section: Reference To Relevant Literaturementioning

confidence: 99%

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Spontaneous Pneumomediastinum and Subcutaneous Emphysema in Dermatomyositis: A Case Series and Literature Review

Subki¹,

Almani²,

Albeity³

et al. 2023

JIR

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Background: Spontaneous pneumomediastinum and subcutaneous emphysema are rare and serious complications of dermatomyositis (DM). Case Presentation: Our article presents two clinically heterogeneous cases of DM who developed pneumomediastinum and subcutaneous emphysema. The first was a 24-year-old lady with a recently diagnosed DM. She developed rapidly progressive pneumonia, interstitial lung disease (ILD), pneumomediastinum, subcutaneous emphysema, and acute respiratory distress syndrome (ARDS). She died despite treatment with steroids and immunosuppressants (methotrexate and mycophenolate mofetil (MMF)). The second was a 30-year-old man diagnosed with amyopathic DM. He developed pneumomediastinum prior to ILD, which worsened over time, and subcutaneous emphysema evolved. However, he recovered completely after corticosteroid, MMF, and rituximab. Conclusion: Spontaneous pneumomediastinum and subcutaneous emphysema may complicate DM. Corticosteroids, immunosuppressants, and respiratory support are the mainstay of management for these conditions. Though they were reported to carry a poor prognostic value, the course and outcome are highly variable among the cases.

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