“…37 The diagnosis is often followed by repeated hospitalizations and a 5-year mortality as high as 50%. 37 CT is useful to identify airway and lung abnormalities, guide bronchoscopy and monitor treatment responses. Early diagnosis and aggressive treatment with simultaneous use of pulmonary vasodilators (sildenafil, bosentan), aerosolized fibrinolytics, mucolytics, and inhaled steroids can improve the outcome of these patients, although the duration of the efficacy of treatment remains unknown.…”