: Online published version of an accepted article before publication in the final form. Journal Name: International Journal of Case Reports and Images (IJCRI) Type of Article: Clinical Images Title: Successful treatment of pneumomediastinum in a patient with interstitial lung disease due to anti-synthetase syndrome. A case report and review of the literature Authors: Roy Cho, Erhan H. Dincer, Rade Tomic, Hyun Kim doi: To be assigned Early view version published: May 13, 2017 How to cite the article: Roy Cho, Erhan H. Dincer, Rade Tomic, Hyun Kim. Successful treatment of pneumomediastinum in a patient with interstitial lung disease due to antisynthetase syndrome. A case report and review of the literature. International Journal of Case Reports and Images (IJCRI). Forthcoming 2017. Disclaimer: This manuscript has been accepted for publication. This is a pdf file of the. The is an online published version of an accepted article before publication in the final form. The proof of this manuscript will be sent to the authors for corrections after which this manuscript will undergo content check, copyediting/proofreading and content formatting to conform to journal s requirements. Please note that during the above publication processes errors in content or presentation may be discovered which will be rectified during manuscript processing. These errors may affect the contents of this manuscript and final published version of this manuscript may be extensively different in content and layout than this. Page 1 of 6
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 TYPE OF ARTICLE: Clinical Images TITLE: Successful treatment of pneumomediastinum in a patient with interstitial lung disease due to anti-synthetase syndrome. A case report and review of the literature Authors and contributions: Roy Cho 1, Erhan H. Dincer 1, Rade Tomic 1, Hyun Kim 1 AFFILIATIONS: 1 M.D, Assistant Professor, University of Minnesota, Department of Pulmonary, Allergy, Critical Care and Sleep Medicine CORRESPONDING AUTHOR DETAILS Roy Cho, MD Assistant Professor, University of Minnesota, Department of Pulmonary, Allergy, Critical Care and Sleep Medicine Email: choxx548@umn.edu Short Running Title: Successful treatment of pneumomediastinum in antisynthetase syndrome Guarantor of Submission: The corresponding author is the guarantor of submission. 24 25 26 27 28 29 30 31 Page 2 of 6
32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60 61 62 63 TITLE: Successful treatment of pneumomediastinum in a patient with interstitial lung disease due to anti-synthetase syndrome. A case report and review of the literature CASE REPORT A 35-year-old woman with interstitial lung disease (ILD) due to AS presented with cough, hoarseness and facial swelling. Chest CT demonstrated significant PNM (Figure 1). Bronchoscopy and esophagram were unremarkable. We began highconcentration oxygen (10Liters/min with non-rebreather face mask for 12-hours/day), withheld mycophenolate mofetil, reduced prednisone and began dextromethorphan. After 1-month, there was complete resolution of her symptoms and PNM. DISCUSSION Pneumomediastinum (PNM) is a rare complication of anti-synthetase syndrome (AS) with only three case reports since 1986 [1-3]. The associated 1-month mortality is 25%, which highlights the need for effective management [4]. The management of PNM in ILD is based on case reports and experience forming expert opinion; however, the best management requires understanding the pathogenesis [5]. Several mechanisms of PNM in ILD have been proposed including the Macklin effect, worsening vasculitis leading to airway rupture, and weakened alveolar walls from immunosuppression [6]. In this case, reducing immunosuppression suggested that weakened alveolar walls was a factor and using high-concentration oxygen and cough suppressant decidedly contributed to the full recovery. Notably, this is the second case in the literature that has reported success using high-concentration oxygen for the treatment of PNM [7]. CONCLUSION We report a case of PNM in a patient with AS who was successfully treated with reduction in immunosuppression, high-concentration oxygen, and cough suppression. Keywords: anti-synthetase syndrome, dermatomyositis, pneumomediastinum, interstitial lung disease Page 3 of 6
64 65 66 67 68 69 70 71 72 73 74 75 76 77 78 79 80 81 82 83 84 85 86 87 88 89 90 91 92 93 94 95 CONFLICT OF INTEREST There was no conflict of interest among the authors. AUTHOR S CONTRIBUTIONS Roy Cho Group 1: Substantial contributions to conception and design, acquisition of data, or analysis and interpretation of data Group 2: Drafting the article or revising it critically for important intellectual content Group 3: Final approval of the version to be published Rade Tomic Group 1: Final approval of the version to be published Hyun Kim Group 1: Substantial contributions to conception and design, acquisition of data, or analysis and interpretation of data Group 2: Drafting the article or revising it critically for important intellectual content Group 3: Final approval of the version to be published Erhan H. Dincer and Rade Tomic were involved with the final review of the manuscript REFERENCES 1. Bradley JD. Spontaneous pneumomediastinum in adult dermatomyositis. Ann Rheum Dis 1986; 45:780-752. 2. Carmody E, McNicholl J, Chadwick G, et al. Prolonged spontaneous pneumomediastinum in adult dermatomyositis. Ann Rheum Dis 1987;46-566. 3. Cicuttini FM, Fraser KJ. Recurrent pneumomediastinum in adult dermatomyositis. J Rheumatol 1989; 16:384-386. 4. Ma X, Chen Z, Hu W, et al. Clinical and serological features of patients with dermatomyositis complicated by spontaneous pneumothorax. Clin Rheumatol. 2016; 35:489-493. Page 4 of 6
96 97 98 99 100 101 102 103 104 105 106 107 108 109 110 111 5. Tang R, Millett CR, and Green JJ. Amyopathic dermatomyositis complicated by pneumomediastinum. JCAD. 2013; 6(3):40-43. 6. Macklin MT, Macklin CC. Malignant interstitial emphysema of the lungs and mediastinum as an important occult complication in many respiratory diseases and other conditions: an interpretation of the clinical literature in the light of laboratory experiment. Medicine. 1944; 23:281-358. 7. Patel A, Kesler B, and Wise R. Persistent pneumomediastinum in interstitial fibrosis associated with rheumatoid arthritis: treatment with high-concentration oxygen. Chest. 2000; 117:1809-1813. FIGURE LEGEND Figure 1: Chest computed tomography (CT) at the level of the neck, heart and lung base demonstrates subcutaneous air and pneumomediastinum (Left). After 1-month of high-concentration oxygen, anti-tussives and reduction in immunosuppression; there was complete resolution (Right). 112 113 114 115 116 117 118 119 120 121 122 123 124 125 126 127 Page 5 of 6
128 FIGURE 129 130 131 132 133 134 135 Figure 1: Chest computed tomography (CT) at the level of the neck, heart and lung base demonstrates subcutaneous air and pneumomediastinum (Left). After 1-month of high-concentration oxygen, anti-tussives and reduction in immunosuppression; there was complete resolution (Right). Page 6 of 6