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

A-50-Year-Old-Woman-With-Limited-Scleroderma-Prese

Pdf Summary

A 50-year-old woman with a history of limited scleroderma (SSc) complicated by interstitial lung disease (ILD) and pulmonary arterial hypertension presented with acute on chronic shortness of breath. Her medical journey with these conditions began 10 years earlier, with worsening symptoms over time despite treatment. A right heart catheterization indicated severe pulmonary arterial hypertension. Despite treatment, including an up-titration of IV treprostinil over six months, her respiratory condition remained concerning.<br /><br />Physical examination revealed sclerodactyly, a holosystolic murmur, bibasilar inspiratory crackles, and mild pitting edema. Diagnostic studies, including a CT angiography of the chest and a 2D echocardiogram, showed stable interstitial lung disease but significant markers of right ventricular systolic pressure (RVSP). <br /><br />The primary diagnosis in this case was ventilation-perfusion mismatching caused by systemic treprostinil inhibiting hypoxic pulmonary vasoconstriction (HPV). Normally, HPV is a physiological response to alveolar hypoxia, directing blood flow to better-oxygenated areas to match ventilation with perfusion. Chronic activation of HPV, however, can be harmful, particularly in diffuse lung disease, often leading to pulmonary hypertension (PH) and right ventricular failure.<br /><br />While attempting to manage her condition, systemic vasodilators like treprostinil can inadvertently worsen V/Q mismatching by inhibiting HPV, thus exacerbating hypoxemia. Despite this, the optimal treatment for PH in chronic lung disease remains underdetermined, with lung transplantation being the only definitively effective therapy. Treatments like sildenafil and inhaled prostacyclin therapies have shown some promise, though large-scale studies are needed.<br /><br />In this case, the patient's condition improved when IV treprostinil was gradually discontinued, enabling a return to her baseline oxygen requirement. However, she refused further therapies and opted for palliative care. Pulmonary hypertension in chronic lung diseases like SSc-ILD typically suggests a poor prognosis, highlighting the complex nature of managing such conditions.

Keywords

scleroderma

interstitial lung disease

pulmonary arterial hypertension

treprostinil

ventilation-perfusion mismatch

hypoxic pulmonary vasoconstriction

right ventricular failure

sildenafil

lung transplantation

palliative care