CHEST Guidelines-A-50-Year-Old-Woman-With-Limited-Scleroderma-Prese (1)

A-50-Year-Old-Woman-With-Limited-Scleroderma-Prese (1)

Pdf Summary

A 50-year-old woman with a history of limited scleroderma, interstitial lung disease (ILD), and pulmonary arterial hypertension presented with worsening shortness of breath. Diagnosed with scleroderma a decade prior, she developed ILD two years ago and was treated with mycophenolate mofetil and low-dose prednisone. Despite stable ILD, her dyspnea worsened, necessitating supplemental oxygen. A right heart catheterization revealed severe pulmonary arterial hypertension. Treatment with intravenous treprostinil was initiated six months prior to her presentation. <br /><br />Upon hospital admission, she was afebrile but exhibited tachycardia, tachypnea, and hypoxemia, requiring high-flow nasal cannula oxygen support. Physical examination showed sclerodactyly, bibasilar inspiratory crackles, and mild edema. Normal blood counts and stable renal and liver function were observed, and cardiac troponins were negative. CT angiography ruled out pulmonary embolism and showed no lung disease progression.<br /><br />The patient’s worsening dyspnea was diagnosed as due to ventilation-perfusion mismatching caused by inhibition of hypoxic pulmonary vasoconstriction through systemic treprostinil. This physiological process, while attempting to optimize oxygen delivery, exacerbates hypoxemia in certain contexts by impairing blood flow distribution between well-ventilated and poorly ventilated lung regions. <br /><br />Systemic vasodilator treatments for pulmonary hypertension often worsen V/Q mismatch. Although lung transplantation is the definitive treatment for this challenging condition, systemic therapies like phosphodiesterase-5 antagonists (e.g., sildenafil) show some promise in improving symptoms. In this case, the patient’s situation improved after carefully tapering off treprostinil and returning to baseline oxygen therapy, but she declined further treatment and opted for palliative care. This case underscores the complexities in managing pulmonary hypertension in patients with chronic lung diseases.

Keywords

scleroderma

interstitial lung disease

pulmonary arterial hypertension

dyspnea

treprostinil

ventilation-perfusion mismatch

hypoxic pulmonary vasoconstriction

sildenafil

palliative care

lung transplantation