Survival in Pulmonary Hypertension Associated With the Scleroderma Spectrum of Diseases PDF Print E-mail
Thursday, 22 December 2011 20:32
Stephen C. Mathai, Laura K. Hummers, Hunter C. Champion, Fredrick M. Wigley, Ari Zaiman, Paul M. Hassoun, and Reda E. Girgis

Objective. Pulmonary hypertension (PH) is an important cause of mortality in systemic sclerosis (SSc), where it can be isolated (pulmonary arterial hypertension [PAH]) or associated with interstitial lung disease (ILD). This study was undertaken to characterize determinants of survival among SSc patients with either type of PH who received PAH-specific therapy.

Methods. Consecutive SSc patients with PAH or ILD-associated PH confirmed by right heart catheterization were included in the study. Kaplan-Meier and Cox proportional hazards models were used to compare survival between SSc patients with PAH and those with ILD-associated PH and to identify predictors of survival.

Results. Fifty-nine patients (39 with PAH and 20 with ILD-associated PH) were identified. The majority (15 of 20 with ILD-associated PH and 27 of 39 with PAH) received an endothelin receptor antagonist as initial therapy. Median followup time was 4.4 years (range 2.7–7.4 years). Survival was significantly worse in SSc patients with ILD-associated PH than in those with PAH (1-, 2-, and 3-year survival rates 82%, 46%, and 39% versus 87%, 79%, and 64%, respectively; P <0.01 by log rank test). In a multivariable analysis, ILD-associated PH was associated with a 5-fold increase in risk of death compared with PAH. Pulmonary vascular resistance index was also an independent predictor of mortality in the overall cohort (hazard ratio 1.05, P <0.01) and was a significant univariable risk factor in
each group separately. Type of initial PAH therapy and the use of warfarin were not related to survival.

Conclusion. Survival in SSc complicated by PH remains poor despite currently available treatment options. While therapy may be associated with improved survival in PAH compared with historical controls, the prognosis for patients with ILD-associated PH is particularly grim. Early diagnosis and treatment may improve outcomes since worsening hemodynamic factors were associated with reduced survival.

More articles :

» Scleroderma-Related PAH: The Need for Early Diagnosis and Treatment

Pulmonary arterial hypertension (PAH) associated with scleroderma (systemic sclerosis) is an aggressive disorder with a poor prognosis. Effective therapies are available for PAH but patients with scleroderma-associated PAH have a poorer response to...

» More Research Needed to Understand Scleroderma

With an estimated 14 million cases worldwide, you would think more would be known about , a chronic, debilitating disease that can lead to the hardening and tightening of skin and connective tissue. But it seems that we don’t know enough: “We...

» Nutrition & Dietary Supplements

A comprehensive treatment plan for may include a range of complementary and alternative therapies. Individuals with Scleroderma tend to have deficiencies in many vitamins and minerals. Ask your team of health care providers about the best ways to...

» Clinical Correlates Of Sleep Problems In Systemic Sclerosis: The Prominent Role Of Pain

Objective. Problems with sleep are common in patients with SSc and impact daily function. Little research, however, has examined factors associated with sleep disruption in SSc. Therefore, the objective of this study was to investigate...

» Role of N-terminal Brain Natriuretic Peptide in Scleroderma-associated Pulmonary Arterial Hypertension

Mark H. Williams, Clive E. Handler, Raza Akram, Colette J. Smith, Clare Das, Joanna Smee, Devaki Nair, Christopher P. Denton, Carol M. Black and John G. CoghlanFirst published online: April 27, 2006Source:

» Prevention Of Vascular Damage In Scleroderma With Angiotensin-converting Enzyme (ACE) Inhibition

Great strides have been made in identifying and managing the organ-based complications of  systemic sclerosis (SSc). There is no room for the nihilism towards treating this disease that used to be so prevalent. However, there is still...