Hoarseness and laryngeal lesions may be poor prognostic factors for pneumomediastinum in dermatomyositis with interstitial lung disease

Pneumomediastinum (PnM) is a rare complication of interstitial lung disease (ILD) and dermatomyositis (DM) that can be fatal. The relationship of hoarseness, laryngeal lesions with the occurrence and prognosis of PnM in patients with ILD and DM is not clear. In this study the clinical records of patients with ILD and DM were retrospectively reviewed, focusing mainly on the presence of hoarseness, laryngeal lesions and PnM. All published cases of DM that were complicated by PnM in English were also reviewed and analysed. There were 1841 patients with a diagnosis of ILD in which there are 44 patients with DM were identified. There were statistically differences in average age and sex ratio between the patients with or without DM. In all of the 1841 patients there were 11 patients developed into PnM; 8 patients were reported hoarseness. The incidence of PnM and hoarseness are significantly higher in patients with ILD and DM than patients with ILD only (P<0.01). There was a significant difference in prognoses and the occurrence of PnM between patients with and without hoarseness or laryngeal lesions (P<0.01). Twenty-eight case reports of patients diagnosed with PnM in DM were identified in the English literature including our 3 cases. All of the patients with laryngeal lesions died, whereas 3 of the patients with only bronchial lesions survived. To conclude, our results support the concept that hoarseness and laryngeal lesions may be associated with the occurrence and poor prognosis of PnM in patients with DM and ILD.

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