Case Discussion - PAH Associated with Scleroderma

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A 60 year-old Caucasian woman with associated pulmonary arterial hypertension due to limited scleroderma is on combination therapy with both bosentan 125 mg twice daily and sildenafil 40 mg three times daily states she feels worse than she did at her last visit.  Her pulmonary vasodilator regimen has been stable for one year.  She is also on warfarin, colchicine, allopurinol, mitomycin, and bumetamide 1 mg daily.  Her WHO functional class is now III, compared to II three months prior.  Her systemic blood pressure is 150/88 with a heart rate of 96.  Her baseline oxygen saturation on room air is 92%.  She has jugular venous distension, clear lungs but typical signs of pulmonary hypertension plus an S4 gallop on cardiac examination.  She also has Raynaud's,  sclerodactyly (unchanged) and mild lower extremity edema (new).  Her hemoglobin and liver function tests are normal but her brain natriuretic peptide has increased to 400 from 150 pg/mL.  Serum creatinine is 1.3 mg/dL.  The six-minute walk distance has decreased from 345 to 250 meters but she had a recent flare of her gout and complained of residual podagra.  Echocardiography showed worsening right heart pressures and associated right ventricular enlargement and hypokinesis.  What do you make of her worsening symptoms?  Should you empirically change her treatment regimen?  If so, how?  Does she require additional evaluation in order to make appropriate recommendations?  Let me know your thoughts.  I will then share what we did and the debate will begin!