Abstract 12082: Dermatomyositis Associated Pulmonary Fibrosis Masquerading as Severe Aortic Insufficiency

Abstract

Description of Case: A 56-year-old female with asthma and hypertension presented for two weeks of dyspnea, cough, and fevers. Initial workup revealed bilateral ground glass opacities. TTE revealed severe aortic insufficiency, early mitral valve closure, and a LVEF of 65%. The patient underwent SAVR and was started on diuresis but continued to have significant respiratory distress over the next few months, leading to recurrent hospitalizations and requiring home oxygen therapy. Serial imaging revealed worsening of her pulmonary opacities. Due to persistent musculoskeletal and cutaneous symptoms with an elevated CPK, an alternative diagnosis was considered: dermatomyositis associated pulmonary fibrosis. The patient was started on steroids and immunomodulatory therapy with improvement in her symptoms. Discussion: Dermatomyositis (DM) is an inflammatory myopathy characterized by proximal muscle weakness, rash, and other manifestations including ILD and esophageal dysmotility. Mortality in patients with DM is associated with rapid progression of ILD. Patients may also have myocardial involvement and heart failure. Patients with presumed cardiogenic respiratory failure that does not improve with HF optimization should undergo further workup and consideration of a broader differential diagnosis. Unexplained elevated CPK disproportionate to troponins, and persistent hypoxia in patients with multisystem involvement should trigger an autoimmune work up. If such a process is identified, patients may have a rapid improvement in their symptomatology and excellent prognosis following immunomodulatory therapy. If left undiagnosed, it can cause multi-system failure, including significant myocarditis and heart failure.