Pulmonary arterial hypertension associated with autoimmune disease: a single medical center experience.
BACKGROUND
New drugs have significantly improved the prognosis and quality of life of patients with pulmonary arterial hypertension. However, PAH associated with autoimmune disease, particularly progressive sclerosis, remains a very serious problem.


OBJECTIVE
To evaluate whether the course of the disease and survival is significantly different in patients with PAH related to autoimmune disease as compared to other patients with PAH and to determine the prognostic factors in these patients.


METHODS
We retrospectively compared 24 patients with PAH associated with autoimmune disease to 42 patients with other causes of PAH. We focused on the clinical and hemodynamic parameters and on the outcome.


RESULTS
The early mortality rate was slightly higher in patients with PAH associated with autoimmune disease (13% after the first year, 25% after the fifth year). The prognostic factor was a shorter distance on the 6 minute walking distance test (r = 0.2, P= 0.01).


CONCLUSIONS
The early detection of PAH associated with autoimmune disease should encourage earlier and more aggressive treatment than in idiopathic PAH.
Bendayan
Daniele
D
Pulmonary Institute, Rabin Medical Center, Petah Tikva, Israel.
Shitrit
David
D
Kramer
Mordechai R
MR