Friday, July 24, 2009

Pulmonary hypertension in patients with systemic sclerosis and its variants

In patients with systemic sclerosis (SSc) pulmonary hypertension (PH) portends a poor prognosis, and even worse if it’s associated with interstitial lung disease (ILD) according to this study. According to this Medscape commentary on the study, the investigators suggest identifying such patients, as they may be candidates for early lung transplantation.

Background:

Traditional thinking holds that limited SSc such as CREST syndrome is associated more with PH while diffuse SSc is associated more with ILD. [1]

Such traditional clinical classifications are flawed, and serologic classifications based on autoantibodies (anti-topoisomerase, anti-centromere, anti-RNP, etc.) may be more useful. [2]

As noted in this review, PH may be idiopathic or have disease associations (connective tissue diseases, congenital heart diseases, portal hypertension, HIV infection, drugs) and be associated with pulmonary diseases including COPD, although it is sometimes unclear in the latter case whether it is due to hemodynamic effects of the lung disease itself or overlap with another disorder of the pulmonary vasculature.

Although the use of warfarin was not associated with improved survival in the above mentioned study, the guidelines suggest its use in patients with idiopathic PH.

2 comments:

Karen said...

Hi, I'm Karen. Since my mother was diagnosed with Pulmonary Arterial Hypertension four months ago, I've decided to make a blog to raise awareness of PAH. Perhaps you'd consider linking to my blog - it's http://hypertension-pulmonary.blogspot.com/

Karen said...

Hi, I'm Karen. Since my mother was diagnosed with Pulmonary Arterial Hypertension four months ago, I've decided to make a blog to raise awareness of PAH. Perhaps you'd consider linking to my blog - it's http://hypertension-pulmonary.blogspot.com/