AN UNUSUAL CASE OF BRONCHIECTASIS IN A PATIENT WITH DERMATOMYOSITIS

Abstract

TYPE: Case Report TOPIC: Diffuse Lung Disease INTRODUCTION: Bronchiectasis is a progressive lung disease defined by dilatation of the bronchi in the setting of chronic airway infection and/or inflammation. It is common pulmonary manifestation in certain rhumatologic disorders, however, it has never been reported in association with inflammatory myopathies. Here we present a case of bronchiectasis in a patient with dermatomyositis. CASE PRESENTATION: A 71 year old African American woman with history of dermatomyositis presented with productive cough for two years. She was diagnosed with dermatomyositis 20 years prior, treated with immunosuppressives and no current active disease. She had no history of smoking, recurrent respiratory infection or occupational exposure. Her Chest CT demonstrated bibasilar and upper lobes cylindrical bronchiectasis (figure). Labratory tests indicated normal CBC, CMP, Muscle enzyms, Serum IgE levels to Aspergillus, Alpha-1antitrypsin and negative ANA, RF, Anti-CCP and TB tests. Patient was started on acapella therapy and albuterol as needed with improvement in her symptoms. DISCUSSION: Bronchiectasis often develops in the setting of infections, ciliary clearance impairment, immunodeficiencies, allergic bronchopulmonary aspergillosis and Alpha1-antitrypsin deficiency. It is an unusual manifestation in inflammatory myopathies where Interstitial Lung Diseases (ILDs) are the most common pattern of lung involvement. ILDs often present with dyspnea, dry cough and CT findings of basal reticular abnormalities or honeycombing, as oppose to our patient's presentation and CT findings of diffuse cylindrical bronchiectasis without reticular abnormalities. With all other etiologies ruled out, it was suspected her dermatomyositis to be the underlying etiology of bronchiectasis. CONCLUSIONS: It is important to recognize and suspect atypical lung presentations in patients with inflammatory myopathies. DISCLOSURE: Nothing to declare. KEYWORD: Bronchiectasis