Spontaneous pneumomediastinum (SP) is a rare illness that has been linked to connective tissue disorders (CTDs), most notably dermatomyositis and polymyositis. Researchers wanted to investigate this link by examining the features of patients with CTD and SP.

Researchers identified 25 individuals with CTD and SP who were assessed between January 1997 and December 2016. Clinicoradiologic features, therapy, and results were retrieved, and chest computed tomography scans were examined.

Among 25 participants with CTD and SP, the median age at the time of SP incidence was 55, and 60% were female, 37% had a smoking history, 67% had symptomatic spontaneous pneumomediastinum, 18  had a known CTD diagnosis, and 20 had radiologic indications of interstitial lung disease. Chest radiography and chest computed tomography were used to diagnose spontaneous pneumomediastinum in 20% of patients and chest computed tomography in the other instances. In 22 instances, expectant observation alone was used to treat spontaneous pneumomediastinum. Four patients experienced simultaneous pneumothorax, with one requiring chest tube drainage. During the median follow-up of 13 months, there were no deaths attributable to SP. At 1 year, the cumulative survival rate was 52%, and at 2 years, it was 40%.

A spontaneous pneumomediastinum is a rare form of CTD that frequently arises in the setting of interstitial lung disease. Although SP appears to have a rather benign short-term course, the presence of SP in CTD patients may be a bad prognostic indicator.