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Spontaneous Pneumomediastinum in Connective Tissue Diseases

De Giacomi, Federica MD*; Baqir, Misbah MBBS; Cox, Christian W. MD; Moua, Teng MD; Matteson, Eric L. MD§; Ryu, Jay H. MD

JCR: Journal of Clinical Rheumatology: September 2019 - Volume 25 - Issue 6 - p 239–245
doi: 10.1097/RHU.0000000000000835
Original Articles

Background Spontaneous pneumomediastinum (SP) is an uncommon disorder but has been described in cases with connective tissue diseases (CTDs), most commonly dermatomyositis and polymyositis. We aimed to explore this relationship by analyzing the characteristics of CTD patients with SP.

Methods Using a computer-assisted search, we identified 25 patients with CTD and SP evaluated between January 1997 and December 2016 at our institution. Clinicoradiologic characteristics, treatment, and outcomes were extracted and chest computed tomography studies analyzed.

Results We identified 25 patients with CTD and SP. Median (range) age at SP occurrence was 55 (18–82) years, and 60% of the patients were women. Smoking history was present in 37%. Spontaneous pneumomediastinum was symptomatic in 56% of patients. Eighteen patients (72%) had a known CTD diagnosis, and 20 patients (80%) manifested radiologic evidence of interstitial lung disease. Spontaneous pneumomediastinum diagnosis was achieved with chest radiography in 20% of cases and chest computed tomography in the other cases. Spontaneous pneumomediastinum was managed with expectant observation alone in 22 cases (88%). Four patients (16%) had concomitant pneumothorax, 1 of whom required chest tube drainage. There were no deaths attributable to SP during the median (range) follow-up of 13 (0–174) months. Cumulative survival was 52% at 1 year and 40% at 2 years.

Conclusions Spontaneous pneumomediastinum is an uncommon manifestation of CTD and usually occurs in the presence of interstitial lung disease. Although SP seems to be associated with a relatively benign short-term course, occurrence of SP in CTD patients may be a poor prognostic factor.

In this study, we describe the risk factors, presentation, and clinical course and follow-up of patients with connective tissue disease who presented with spontaneous pneumomediastinum.

From the *Department of Health Science, University of Milan-Bicocca, Clinica Pneumologica, AO San Gerardo, Monza, Italy

Department of Radiology

Division of Pulmonary and Critical Care Medicine

§Division of Rheumatology, Mayo Clinic, Rochester, MN.

The authors declare no conflict of interest.

Correspondence: Misbah Baqir, MBBS, Division of Pulmonary and Critical Care Medicine, Mayo Clinic, 200 First St SW, Rochester, MN 55905. E-mail:

Online date: June 19, 2018

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