Is Pulmonary Arterial Hypertension Really a Late Complication of Systemic Sclerosis? PDF Print E-mail
Thursday, 22 December 2011 20:15
Eric Hachulla, MD, PhD; David Launay, MD, PhD; Luc Mouthon, MD, PhD; Olivier Sitbon, MD, PhD; Alice Berezne, MD; Loïc Guillevin, MD; Pierre-Yves Hatron, MD; Ge´rald Simonneau, MD; Pierre Clerson, MD; and Marc Humbert, MD, PhD;

Pulmonary arterial hypertension (PAH) is a frequent complication in patients with systemic sclerosis (SSc) that has a prevalence of 8 to 12%. Within the last 2 decades, pulmonary fibrosis and PAH have become the leading causes of death in SSc patients. The estimated 3-year survival rate among patients with PAH associated with SSc is approximately 50%. The development of PAH in patients with SSc must be differentiated from that of pulmonary hypertension (PH) associated with severe pulmonary fibrosis. The latter may manifest as a significant reduction of lung volume (FVC 60% predicted and/or total lung capacity [TLC] 60%) and a modest elevation in mean pulmonary artery pressure (mPAP) of 35 mm Hg at rest.

Unlike pulmonary fibrosis, PAH is understood to occur late in the natural course of SSc and more frequently in patients with limited cutaneous SSc (lcSSc). This assertion, however, may benefit from reappraisal. Studies have shown that PAH may be similarly prevalent among patients with diffuse cutaneous SSc (dcSSc) as it is among patients with lcSSc. Robust data estimating the typical duration between the diagnosis of SSc and the diagnosis of PAH are lacking. The aim of this study was to analyze this duration and evaluate the potential relevance of “time of occurrence of PAH” in the course of SSc as a prognostic factor.

Download the full research paper from the link provided below.

 
More articles :

» The Science of Raw Food

In a recent article on , we noted that changing your diet and introducing an abundance of living and raw foods provide a much safer alternative than traditional medicine in restoring the body’s alkalinity, and subsequently ensuring good health....

» Mechanism ID’d for Benefit of Stem Cells in Autoimmunity

Bone marrow mesenchymal stem cells (BMMSCs) activate a mechanism involving coupling of FAS/FAS ligand to induce T cell apoptosis and immune tolerance, according to an experimental study published online April 26 in Cell Stem Cell.To investigate the...

» Adult Stem Cells Are Helping Scleroderma Patients

Dr. Richard Burt and colleagues at Northwestern University have just published a new study in that provides more evidence for the success of adult stem cell transplant in treating System Sclerosis ().Ten patients were treated with their own adult...

» Bosentan Treatment of Digital Ulcers Related to Systemic Sclerosis

Marco Matucci-Cerinic, Christopher P Denton, Daniel E Furst, Maureen D Mayes, Vivien M Hsu, Patrick Carpentier, Fredrick M Wigley, Carol M Black, Barri J Fessler, Peter A Merkel, Janet E Pope, Nadera J Sweiss, Mittie K Doyle, Bernhard Hellmich,...

» Endothelin Receptor Antagonists for the Treatment of Raynaud’s Phenomenon and Digital Ulcers in Systemic Sclerosis

Kait Arefiev, David F. Fiorentino, and Lorinda ChungDivision of Immunology and Rheumatology, Departments of Dermatology and Medicine, Stanford University School of Medicine, Palo Alto VA Health Care System, 3801 Miranda Avenue, Palo Alto, CA 94304,...

» Mixed Connective Tissue Disease May Represent Subset of Systemic Scleroderma

Many patients with (MCTD) may represent a subset of Systemic , rather than a disease involving overlapping connective tissue disorders as is commonly believed, or a subset of Lupus as some have suggested over the years, Dr. Virginia Steen said at...