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A6488 - Management of Spontaneous Pneumomediastinum in Dermatomyositis with Cyclosporine
Author Block: S. Hapangama1, E. Perez2, A. Adial2, N. Ahmed1, Y. Fuzailov1, R. Gumpeni2; 1Internal Medicine, New-York Presbyterian/Queens, Flushing, NY, United States, 2Pulmonary/Critical Care, New-York Presbyterian/Queens, Flushing, NY, United States.
Introduction: This case discusses a patient with Dermatomyositis (DM) and Interstitial Lung Disease (ILD) presenting with worsening spontaneous pneumomediastinum that was refractory to conventional treatment.
Case Presentation: A 59-year-old female with DM and ILD, managed on prednisone and Mycophenolate Mofetil, presented to the ER with decreasing exercise tolerance, progressive dyspnea at rest, and hypoxia to 85% on ambient air on ambulation.
Initial examination showed an oxygen saturation of 89% on ambient air which improved on nasal cannula and tachypnea to 34 breaths per minute with mild distress. Crepitus was found around the neck extending to the anterior chest, Gottron’s papules were present on the dorsal hand surfaces, diffuse rhonchi/rales in both lung fields. Chest imaging was consistent with prior history of ILD and substantial subcutaneous emphysema, as well as a large pneumomediastinum; there was no evidence of a pneumothorax.The patient was hospitalized and started on methylprednisolone with Mycophenolate Mofetil for her dermatomyositis. By the third day, she remained oxygen dependent without any radiographic improvement. Cyclosporine was added to her treatment regimen. The patient’s oxygenation began to improve along with her dyspnea, successfully being weaned off supplemental oxygen. Subsequent imaging displayed a dramatic decrease in the pneumomediastinum. The patient was eventually discharged on a prednisone taper, Cyclosporine, Mycophenolate Mofetil, and TMP-SMX.
Discussion: Spontaneous pneumomediastinum is a rare complication of Dermatomyositis with ILD. Numerous explanations exist for this adverse effect, the main being that those with Dermatomyositis/ILD have architectural distortion of their lung parenchyma, making it prone to air leakage. However, other reports suggest ischemic vasculopathy involving the bronchial walls and subsequent necrosis with perforation. These processes are postulated to be immune in origin, as CD4 +T-cells have been identified in alveolar walls and bronchoalveolar lavage fluid in such cases. Previous reported cases suggest that Cyclosporine can potentially reduce pneumomediastinum. Cyclosporine inhibits calcineurin in T-cells, leading to inactivation of NFAT gene and activation of TGFβ1 gene which promotes T-cell inhibition. Cyclosporine also down-regulates the expression of CD40L in T-cells that is involved in costimulation and regulation of the T-cell immune response. Thus, Cyclosporine inhibits further T-cell mediated destruction of lung parenchyma. This case suggests that there is potential benefit in Cyclosporine as an adjunct treatment for decreasing the occurrence and severity of spontaneous pneumomediastinum in Dermatomyositis.