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 :

» Study Suggests Systemic Sclerosis Is an Independent Risk Factor for Atherosclerosis

A new study by researchers in Hong Kong suggests that is an independent determinant for moderate to severe coronary calcification or . Conventional cardiovascular risk factors such as age and hypertension predispose patients with systemic sclerosis...

» Liver Autoantibodies in Patients with Scleroderma

Thelma L. Skare & Renato M. Nisihara & Osvaldo Haider & Pedro M. Azevedo & Shirley R. R. UtiyamaReceived: 5 January 2010 / Revised: 13 August 2010 / Accepted: 26 September 2010# Clinical Rheumatology 2010Association between...

» A System Out of Breath: How Hypoxia Possibly Contributes to the Pathogenesis of Systemic Sclerosis

T. W. van Hal, L. van Bon, and T. R. D. J. RadstakeReceived 20 May 2011; Revised 18 August 2011; Accepted 7 September 2011Systemic sclerosis (SSc) is typified by vascular alterations and immunological disturbances and fibrosis of the skin and...

» Patients With Scleroderma May Not Exhibit Increased Corneal Thickness

in patients with systemic sclerosis may not differ significantly from those of healthy patients, contrary to prior hypothesis, a study found."Because [] is characterized by increased deposition of and other connective tissue components in the skin...

» The Role of Intravenous Immunoglobulin Preparations in the Treatment of Systemic Sclerosis

Marta Baleva and Krasimir NikolovReceived 12 June 2011; Revised 28 August 2011; Accepted 28 August 2011Scleroderma is progressive autoimmune disease associated with severe disability. The major underlying pathological process in Scleroderma is...

» New Promising Therapy Against Systemic Sclerosis

There is a new path to defeat systemic sclerosis, also called because of the hardening of the skin of the patients (from Greek skleros, "hard", and derma, "skin"). This path involves the B-cell of the immune system, so far only considered "innocent...