Spontaneous pneumomediastinum in dermatomyositis: a case series and literature review

Abstract

OBJECTIVES:To describe a case series of spontaneous pneumomediastinum in dermatomyositis and to review the literature. METHODS:This was a retrospective single-center case series, reporting 9 patients with pneumomediastinum and defined dermatomyositis, followed from 2005 to 2017. RESULTS: Median age of patients: 33 years; cutaneous and pulmonary involvement in all cases; constitutional symptoms in 88.8% of patients; involvement of the joints in 11.1%, gastrointestinal tract in 44.4%, and muscles in 77.7%; subcutaneous emphysema was observed in 55.5% and pneumothorax in 11.1%, respectively. Muscle weakness was observed in 77.7% of cases and with a median level of serum creatine phosphokinase of 124U/L. Drawing on results for our literature review, the overall analysis showed that the risk factors associated with spontaneous pneumomediastinum were: (a) a history of interstitial pneumopathy; (b) normal or low levels of muscle enzymes; (c) previous use of systemic glucocorticoid; (d) over 50% of patients had subcutaneous emphysema; (e) high mortality as a consequence of severity of the interstitial lung disease. CONCLUSIONS: Our case series revealed that pneumomediastinum is a rare complication in dermatomyositis that occurs in patients with a history of interstitial pneumopathy and may be accompanied by subcutaneous emphysema and pneumothorax.

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

“…11 Several cases of spontaneous pneumomediastinum were reported in patients with DM in the literature. 6,[12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30] Similar to our patients, pneumomediastinum was reported in cases of DM and amyopathic DM, 11 occurred at a widely variable range of disease duration, 6,[12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30] and was associated with variable outcomes from complete recovery 11 to death. 5,14,31,32 Pneumomediastinum occurred prior to (as our second patient), coinciding with, or after (as our first patient) pulmonary involvement.…”

“…Subcutaneous emphysema has also been reported in approximately 55.5% of cases of DM. 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.…”

“…More than half of the cases of pneumomediastinum develop subcutaneous emphysema. 24 Though the vast majority of the cases occur in the context of pneumomediastinum…”

“…42 Many risk factors have been reported to increase the risk of developing pneumomediastinum in DM, such as male gender, younger age at disease onset, presence of ILD, presence of cutaneous vasculopathy, normal or mildly elevated CK levels, amyopathic DM, initial forced vital capacity ≤60, Hamman-Rich-like presentation, neutrophilic bronchoalveolar lavage, and corticosteroid administration. 6,[10][11][12]24,30,43 To improve the prognosis of DM patients who developed pneumomediastinum, the categorization of ILD based on imaging findings and clinical features is suggested to predict survival. As for diagnostic procedures, it was suggested that further research should focus on identifying associated antibodies in dermatomyositis that would facilitate earlier diagnosis.…”