Multifactorial Pulmonary Hypertension in Systemic Sclerosis

Cureus. 2020 Jul 11;12(7):e9144. doi: 10.7759/cureus.9144.

Abstract

Pulmonary hypertension is a progressive disease often associated with multifactorial etiology. The impact of multiple causes contributing to rapid progression of the disease, to our knowledge has not been thoroughly reviewed in literature. The cause of pulmonary hypertension is often implied from pre-existing comorbidities. A diagnostic and management challenge exists when simultaneous presence of multiple plausible causes exist. Studies evaluating the rapid progression of symptoms in multifactorial pulmonary hypertension to this effect are lacking. We present a case of pulmonary arterial hypertension (PAH) in a patient with rapidly progressing symptoms to highlight the need for an early and thorough diagnostic workup.

Keywords: interstitial lung disease; multifactorial pulmonary hypertension; pulmonary arterial hypertension; scleroderma; systemic sclerosis.

Publication types

  • Case Reports