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Pneumomediastinum in dermatomyositis: association with cutaneous vasculopathy
H Kono1, S Inokuma, H Nakayama
1Department of Allergy and Immunological Diseases, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo 113-8677, Japan.
Objectives:
To study the pathogenesis of pneumomediastinum in polymyositis/dermatomyositis (PM/DM).
Patients And Methods:
The clinical records of 48 patients with PM/DM were reviewed, focusing mainly on the presence of pneumomediastinum and cutaneous vasculopathy, and the chest radiographic changes. A patient with pneumomediastinum with a characteristic change in his bronchus is described in detail. Case reports of pneumomediastinum in PM/DM in English publications are reviewed.
Results:
Among the 48 patients with PM/DM, pneumomediastinum was observed as a complication in four patients with DM and none of the patients with PM. Three of the four patients with pneumomediastinum, but only six of the 44 patients without this complication, had associated cutaneous vasculopathy. There was a significant association of pneumomediastinum with cutaneous vasculopathy (p = 0.02) and younger age (p = 0.04), but not with the prevalence of lung disease. A 30 year old man (patient 1) with DM, who had interstitial pneumonitis and skin ulceration due to vasculopathy, developed pneumomediastinum. Fibreoptic bronchoscopy showed white plaques on the bronchial mucosa, which were confirmed by microscopic examination as representing subepithelial necrosis. A literature review showed 13 cases of DM but no patient with PM with pneumomediastinum.
Conclusions:
In patient 1, bronchial necrosis due to vasculopathy was strongly suspected as being responsible for the pneumomediastinum. The results suggest that pneumomediastinum was associated not with interstitial pneumonitis but with the complication of vasculopathy appearing as skin lesions in DM.
Insights
Pneumomediastinum in polymyositis/dermatomyositis (PM/DM) is linked to cutaneous vasculopathy, not lung disease. Bronchial necrosis due to vasculopathy is a suspected cause in dermatomyositis patients.
Area of Science:
- Rheumatology and Pulmonary Medicine
- Pathogenesis of Inflammatory Myopathies
- Rare Complications of Autoimmune Diseases
Background:
- Polymyositis/dermatomyositis (PM/DM) are autoimmune diseases affecting muscles and skin.
- Pneumomediastinum, air in the chest cavity, is a rare complication.
- Understanding its pathogenesis in PM/DM is crucial for patient management.
Observation:
- A retrospective review of 48 PM/DM patients identified pneumomediastinum in 4 dermatomyositis (DM) cases.
- Pneumomediastinum showed a significant association with cutaneous vasculopathy (p=0.02) and younger age (p=0.04).
- Bronchial mucosal examination revealed subepithelial necrosis in a patient with DM and pneumomediastinum.
Findings:
- Pneumomediastinum occurred exclusively in DM patients, not in polymyositis (PM) patients.
- The complication was significantly associated with skin vasculopathy, suggesting a link between systemic vascular involvement and pneumomediastinum.
- Interstitial pneumonitis was not significantly associated with pneumomediastinum.
Implications:
- Bronchial necrosis secondary to vasculopathy is a likely cause of pneumomediastinum in DM.
- This finding highlights the importance of monitoring for vascular complications in DM patients.
- Further research into the mechanisms linking vasculopathy to airway compromise in PM/DM is warranted.