SESSION TYPE: ILD Case Report Posters
PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION: Acute and rapidly progressing pulmonary fibrosis associated with spontaneous pneumomediastinum in a young woman is an unusual presentation for interstitial lung disease.
CASE PRESENTATION: A 23 YO healthy Caucasian woman presented with retrosternal and back pain preceeded by a viral prodrome consisting of fevers, myalgias, nasal congestion and sore throat. The rest of the review of systems, medications, and social history was noncontributory. On physical exam, her vitals were normal. Her neck exam revealed subcutaneous emphysema and was tender to palpation. The rest of her exam was normal. Laboratory studies: CBC normal except platelets of 98. Electrolytes, renal function, albumin, INR, Troponin, CK and urinalysis were normal. Transaminases were transiently elevated. CT scan of the chest revealed a moderate-size pneumomediastinum, subcutaneous emphysema and early lower lobe predominant ground glass opacities. An esophagram noted no esophageal leak. She was placed on antibiotics and corticosteroids. Hypoxemia developed and chest x-rays showed progression of ground glass infiltrates and consolidation. Repeat CT scan noted enlargement of the pneumomediastinum and bilateral pneumothoraces. There was progression of consolidation throughout the parenchyma. A complete serologic workup and cultures were negative. The patient was intubated and cardiothoracic surgery placed two chest tubes and a mediastinal chest tube. The patient required HFOV but eventually we were not able to maintain oxygenation and ECMO was initiated followed by an open lung biopsy which noted diffuse fibrosis with honeycombing. She was subsequently referred to a transplant center.
DISCUSSION: Spontaneous pneumomediastinum is an uncommon condition. Of those with pre-existing lung conditions, interstitial lung disease is the most common3. There are some connective tissue diseases also associated with pneumomediastinum in which ILD was reported1,3. Further, Goff reports that there is an absence of myositis-specific antibodies in myositis with ILD associated pneumomediastinum1.
CONCLUSIONS: We present a young healthy woman who developed fulminant pulmonary fibrosis refractory to corticosteroids over a 2 week period requiring referral for lung transplantation evaluation and a secondary spontaneous pneumomediastinum. Very few interstitial lung diseases present with concomitant ground glass and pneumomediastinum or pneumothorax.
1) Goff B, Rin P, Cantagrel A, et al. Pneumomediastinum in Interstitial Lung Disease Associated With Dermatomyositis and Polymyositis. Arthritis & Rheumatism 2009; 61(1):108-118
2) Haroon M, McLaughlin P, Henry M, et al. Spontaneous Pneumomediastinum in a Patient With Anti/Centromere Antibody/Positive Limited Scleroderma. J Clin Rheum 2011; 17(1):42- 43
3) Iyer V, Joshi A, Ryu J. Spontaneous Pneumomediastinum: Analysis of 62 Consecutive Adult Patients. Mayo Clin Proc. 2009;84(5):417-421
DISCLOSURE: The following authors have nothing to disclose: Frank Courmier, Juan Sanchez
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