Spontaneous Pneumomediastinum in Connective Tissue Diseases

Federica De Giacomi, Misbah Baqir, Christian W. Cox, Teng Moua, Eric L. Matteson, Jay H. Ryu

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)239-245
Number of pages7
JournalJournal of Clinical Rheumatology
Volume25
Issue number6
DOIs
StatePublished - Sep 1 2019

Fingerprint

Mediastinal Emphysema
Connective Tissue Diseases
Thorax
Interstitial Lung Diseases
Tomography
Chest Tubes
Dermatomyositis
Pneumothorax
Radiography
Curriculum
Drainage

Keywords

  • connective tissue diseases
  • dermatomyositis
  • emphysema
  • mediastinal
  • pneumomediastinum
  • polymyositis

ASJC Scopus subject areas

  • Rheumatology

Cite this

Spontaneous Pneumomediastinum in Connective Tissue Diseases. / De Giacomi, Federica; Baqir, Misbah; Cox, Christian W.; Moua, Teng; Matteson, Eric L.; Ryu, Jay H.

In: Journal of Clinical Rheumatology, Vol. 25, No. 6, 01.09.2019, p. 239-245.

Research output: Contribution to journalArticle

De Giacomi, F, Baqir, M, Cox, CW, Moua, T, Matteson, EL & Ryu, JH 2019, 'Spontaneous Pneumomediastinum in Connective Tissue Diseases', Journal of Clinical Rheumatology, vol. 25, no. 6, pp. 239-245. https://doi.org/10.1097/RHU.0000000000000835
De Giacomi, Federica ; Baqir, Misbah ; Cox, Christian W. ; Moua, Teng ; Matteson, Eric L. ; Ryu, Jay H. / Spontaneous Pneumomediastinum in Connective Tissue Diseases. In: Journal of Clinical Rheumatology. 2019 ; Vol. 25, No. 6. pp. 239-245.
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abstract = "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.",
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AU - Cox, Christian W.

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AU - Matteson, Eric L.

AU - Ryu, Jay H.

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N2 - 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.

AB - 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.

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KW - polymyositis

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