Rendu-Osler-Weber Disease with High Pulmonary Hypertension and Interstitial Lung Disease

A 64-year-old female with a family history of hereditary hemorrhagic telangiectasia (HHT) was hospitalized due to complaints dyspnea during light physical exertion and leg edema. HHT diagnosed at 20 y.o., recurrent nasal bleeding started age 52, bleedings severity aggravated by not completely compensated hypertension. At the 60, after massive hemorrhage, she noted onset dyspnea, edema, ascites. Diuretics iron preparations improved her well-being, but from that period onward heart failure worsened each blood loss. The last major before present hospitalization (Hgb 67 g/l), which symptoms significantly deteriorated. Echocardiography showed preserved left ventricular ejection fraction, revealed high pulmonary hypertension (systolic artery pressure 69 mmHg). Chest computed tomography (CT) contrast no evidence embolism, interstitial lung lesions were detected. Pulse therapy glucocorticosteroids did result in positive dynamics control CT scan, allowed reject separate disease. As cardiotropic diuretic therapy, as well correction anemia, patient's condition improved. Macitentan administered, patient refused it because one possible side effects anemia. year later diedfrom acute progression According literature, can have significant impact on prognosis requires timely diagnosis treatment. Interstitial are manifestation underlying disease does require special