Pneumomediastinum in interstitial lung disease associated with dermatomyositis and polymyositis

Abstract

Objective. Spontaneous pneumomediastinum is a rare complication of dermatomyositis (DM) and polymyositis (PM).The aim of this study was to characterize this complication and determine its prognostic factors. Methods. We retrospectively collected a multicenter series of PM/DM cases complicated by pneumomediastinum. We analyzed all published cases and combined those that were exploitable with ours for an investigation of the factors associated with poor survival. Results. We collected 11 PM/DM cases complicated by interstitial lung disease and pneumomediastinum. Five of the 9 DM patients had clinically amyopathic DM without muscle weakness and high serum creatine kinase levels. The outcome was favorable in 7 of these patients and 6 had no sequelae. In total, ϳ25% of our patients of the 21 analyzable cases studied died within 1 month. With a median followup of 240 days, the cumulative estimated Kaplan-Meier survival rate was 64% at 1 year and 55% at 2 years. Poor survival was associated with absence of muscle weakness (P ‫؍‬ 0.02), initial low vital capacity (P ‫؍‬ 0.006), and initial low carbon monoxide diffusion capacity (P ‫؍‬ 0.04). Conclusion. In this first large series of patients with connective tissue disease complicated by pneumomediastinum to be reported, most patients had DM and half amyopathic DM, as in previous reports. Pneumomediastinum may occur before DM diagnosis and may thus reveal DM with minimal or no muscle involvement. Death was associated with an absence of muscle weakness and severe pulmonary involvement before the onset of pneumomediastinum. Corticosteroids and immunosuppressive therapy can result in complete recovery, as in half our cases.

A Fatal Complication of Dermatomyositis: Spontaneous Pneumomediastinum

“…Le Goff et al (6) reported that the prevalence of pneumomediastinum in myositis is approximately 2.2%. In most cases, pneumomediastinum occurs within the first year of DM onset or polymyositis symptoms.…”

“…The overall mortality rate noted by this review was 34%, whereas 25% of deaths were attributed to respiratory distress. Poor prognostic factors included the absence of muscle weakness, decreased vital capacity, and carbon monoxide diffusing capacity prior to the development of pneumomediastinum and high-dose corticosteroid treatment (6,7). On the other hand, Iyer et al (3) found that patients with spontaneous pneumomediastinum most commonly had ILD as a pre-existing lung disease.…”

Raynaud�s Phenomenon and Recurrent Spontaneous Mediastinal Emphysema: One case

“…Semptomların ortaya çıkışı ile SMA gelişimi arasındaki ortalama zaman 10,5 ay olarak belirtilmiştir. 10 Diğer yandan RF akciğer parankimindeki damarlarda vazokonstriksiyon yaparak interstisyel basıncı azaltmış ve böylece alveollerin yırtılmasına neden olmuş olabilir. 1,11 Tong ve arkadaşlarının yaptığı bir çalışmada pnömomedi-astinum ile komplike olan DM ve PM hastaları değerlendi-rilmiş ve toplam 447 olgunun 4'ünde pnömomediastinum tespit edilmiştir.…”

“…Literatürde SMA'nın rekürrensi nadir bir durumdur. [7][8][9][10][11][12][13][14][15][16][17][18] Iyer ve arkadaşları takip ettikleri 62 hastanın sadece 1'inde rekürrens tespit etmişlerdir. 5 Olgumuz bu yönüyle de nadir görülen bir vakadır.…”

Simultaneous Pneumatosis Cystoides Intestinalis and Pneumomediastinum in a Patient with Systemic Sclerosis

“…As well as such diseases, numerous precipitating factors exist, such as vomiting, coughing, asthma exacerbation, and exercise. Among patients with CTD, dermatomyositis (DM) or amyopathic DM is most frequently complicated by pneumomediastinum 6 . However, it is rare in patients with SSc, with only 3 such cases reported in the English-language literature 1,2,3 .…”