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Amyopathic Dermatomyositis Associated With BOOP, Pneumomediastinum, and Subcutaneous Emphysema: A Case Report FREE TO VIEW

Paulo Albuquerque, MD; Rodrigo Chaves, MD; Larissa Souza, MD; Francisco Bezerra Neto
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Medicine, UFRN, Natal, RN, Brazil

Chest. 2015;148(4_MeetingAbstracts):653A. doi:10.1378/chest.2278901
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SESSION TITLE: Miscellaneous Global Case Reports

SESSION TYPE: Global Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: The Dermatomyositis (DM) along with polymyositis belong to the group of so-called idiopathic inflammatory myopathies. Diseases that has a predilection for involvement of the skin and muscles with variable muscle involvement. The lung may be involved in four ways: diffuse alveolitis, with cough and rapidly progressive dyspnea, found in patients with anti-synthetase syndrome; progressively more slowly, as myopathy masks the severity of lung involvement (the most frequent); aspiration pneumonia and an asymptomatic form in which there is only the radiographic findings and / or functional corresponding to the interstitial disease. Spontaneous pneumomediastinum has been reported as a rare complication

CASE PRESENTATION: A 35-year-old man diagnosed with DM amyopathic with obliterative bronchiolitis with organizing pneumonia (BOOP) complicated with subcutaneous emphysema and pneumomediastinum, with irregular history of fever, significant weight loss, alopecia, ulcers oral, dry cough with progressive dyspnea on moderate effort, erythematous-violaceous lesions, scaly, painful in fingers and extensor surfaces of elbows, and eyelids accompanied by severe muscle weakness , since 2013. Facies had swollen to the examination with nasal voice. Chest X-ray and High-resolution computed tomography (HRCT) revealed subcutaneous emphysema with pneumomediastinum and fibrotic interstitial lung disease (Figure 1). Electromyography of four members with the result of nonspecific myopathy. Lung biopsy which revealed BOOP and NSIP. He was diagnosed with DM amyopathic with BOOP and spontaneous pneumomediastinum and subcutaneous emphysema. Chosen pulse therapy 1g IV for 3 days of methylprednisolone associated with cycles of cyclophosphamide for pulmonary disease and oxygen therapy as an aid in the reabsorption of pneumomediastinum and subcutaneous emphysema.

DISCUSSION: Bradley described the first case of pneumomediastinum associated with MD in 1986. In English literature and french were reported 25 cases. Therefore, in patients with PM/DM and respiratory symptoms, ILD should always be included in the differential diagnosis. A pattern of nonspecific interstitial pneumonia (NSIP), with or without areas of consolidation (suggestive of organizing pneumonia), is usually revealed on HRCT scans. Usual approach to therapy for PM/DM-related ILD is to use a combination of glucocorticoids and a steroid-sparing agent . We use cyclophosphamide for cases with clinically severe or rapidly progressive ILD, like in this patient. The pneumomediastinum is a rare complication characterized by the presence of air in the mediastinal tissues outside the esophagus, tracheobronchial tree, or pericardium, which may be accompanied by subcutaneous emphysema and pneumothorax and is associated with worse prognosis. Its etiopathogenesis remains unclear, but the following mechanisms have been raised in the literature: pulmonary vasculopathy leading to necrosis and rupture of the tracheobronchial tree, corticosteroids causing tissue fragility and increasing the risk of infections and pulmonary fibrosis with ruptured cysts and subpleural bubbles.

CONCLUSIONS: Polymyositis and dermatomyositis are classified as idiopathic inflammatory myopathies. Interstitial lung disease is rare and is described in the literature in about 10% of cases, but result in significant morbidity and mortality. In a patient with dermatomyositis complaining of dyspnea an active investigation is mandatory for infections, cancer, complications related to disease activity or side effect of treatment. Although pneumomediastinum is rare occurrence in dermatomyositis need to be recognized and treated promptly to prevent further morbidity and mortality of the patient. Acute fibrinous and organizing pneumonia can either be idiopathic or be associated with an underlying or concomitant condition, such as collagen vascular disease and drug reaction

Reference #1: Barvaux VA, Van Mullem X, Pieters TH, Houssiau FA. Persistent pneumomediastinum and dermatomyositis: a case report and review of the literature. Clin Rheumatol 2001; 20 (5): 359-61. [Links]

Reference #2: Masrouha KZ, Kanj N, Uthman I. Late-onset pneumomediastinum in dermatomyositis. Rheumatol Int 2009; 30 (2): 291-2. [Links]

Reference #3: Bradley JD. Spontaneous pneumomediastinum in adult dermatomyositis. Ann Rheum Dis 1986; 45 (9): 780-2.

DISCLOSURE: The following authors have nothing to disclose: Paulo Albuquerque, Rodrigo Chaves, Larissa Souza, Francisco Bezerra Neto

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