Diagnosis of pulmonary arterial hypertension in a patient with systemic sclerosis.
BACKGROUND
A 42-year-old woman with limited cutaneous systemic sclerosis presented with rapid-onset dyspnea on exertion, which had developed over the previous 8 weeks. She had not experienced any dyspnea before this period. Transthoracic Doppler echocardiography performed 6 months before presentation demonstrated an estimated right ventricular systolic pressure of 32 mmHg. Lung function tests also performed at that time revealed a decreased diffusion capacity for carbon monoxide of 54% and normal lung volumes, and high-resolution CT scan of the lungs was normal.


METHODS
Physical investigation, CBC, analysis of C-reactive protein and pro-brain natriuretic peptide, transthoracic Doppler echocardiography, six-minute walk test, lung function tests including diffusion capacity for carbon monoxide, right heart catheter, high-resolution CT scan, and ventilation/perfusion scan.


METHODS
Pulmonary arterial hypertension associated with limited cutaneous systemic sclerosis.


RESULTS
Treatment with oral anticoagulation therapy and the endothelin-receptor antagonist bosentan. Monitoring of adverse effects of bosentan therapy was performed using liver function tests.
Distler
Jörg H W
JH
Department of Internal Medicine 3, University of Erlangen-Nuremberg, Hanover, Germany.
Hoeper
Marius M
MM
Distler
Oliver
O
D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use; D000077300:Bosentan; D004417:Dyspnea / Q000209:etiology*; D004452:Echocardiography; D005080:Exercise Test; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D020097:Natriuretic Peptide, Brain / Q000097:blood; D012129:Respiratory Function Tests; D012595:Scleroderma, Systemic / Q000150:complications*; D013449:Sulfonamides / Q000627:therapeutic use
D000959:Antihypertensive Agents; D013449:Sulfonamides; D020097:Natriuretic Peptide, Brain; D000077300:Bosentan