| Pulmonary Hypertension |
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Prognosis of Pulmonary Hypertension in Systemic Sclerosis |
| Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer |
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Overview of Prognosis of Pulmonary Hypertension in Systemic Sclerosis (Scleroderma) |
| Early diagnosis and treatment substantially improves the prognosis for pulmonary hypertension associated with systemic sclerosis (scleroderma). (Also see: Types of Scleroderma, PH Diagnosis, PH Treatments and Types of Scleroderma: Prognosis and Mortality) |
| Evidence For Pulmonary Arterial Hypertension (PAH) In Very Early And Mild Systemic Sclerosis (SSc). We found no evidence that PAH is a regular feature in this cohort of early/mild, profibrotic SSc patients. If confirmed in larger, multicenter studies, this finding has major impact on clinical practice, because regular, extensive screenings for PAH would not appear necessary in this patient population - in contrast to patients with established SSc. S. Jordan. EULAR 2011 SAT0444. Ann Rheum Dis 2011;70(Suppl3):667. |
| Systemic Sclerosis-associated Pulmonary Arterial Hypertension. Pulmonary arterial hypertension (PAH) is a devastating vascular complication of a number of connective tissue diseases including systemic sclerosis (SSc) where it has a dramatic impact on the clinical course and overall survival and is the single most common cause of death in patients afflicted with this syndrome. Jerome Le Pavec. Am. J. Respir. Crit. Care Med. 2010, March. |
| Researchers revisit pulmonary arterial hypertension survival. Setting out to determine the survival of patients with pulmonary arterial hypertension (PAH), researchers at the University of Chicago Medical Center and their colleagues also discovered that an equation used for more than 20 years to predict survival is outdated. University of Chicago Medical Center. 01/06/10. |
| Risk factors for death and the 3-year survival of patients with systemic sclerosis (SSc): the French ItinérAIR-Sclérodermie study. This 3-year study observed survival and mortality estimates that were comparable with previous reports. Pulmonary artirial hypertension increased the Hazard Ratio for mortality in patients with SSc, justifying yearly echocardiographic screening. Eric Hachulla. Rheumatology 2009 48(3):304-30. January 27, 2009. (Also see: Prognosis) |
| Connective Tissue Disease Associated Pulmonary Arterial Hypertension in the Modern Treatment Era. Survival in SSc-PAH in the modern treatment era is better than in historical series. A significant proportion of patients with mild functional impairment or exercise-induced SSc-PAH have evidence of disease progression. Survival in patients with respiratory disease associated pulmonary hypertension is inferior. R. Condliffe. Am. J. Respir. Crit. Care Med. October, 2008. (Also see: Connective Tissue Disease) |
| Connective tissue associated pulmonary arterial hypertension (PAH). Widespread and timely availability of disease modifying therapies substantially improves the prognosis for patients with systemic sclerosis associated PAH. Early diagnosis is most readily achieved in patients with connective tissue disease associated PAH. PubMed. Lupus. 2006;15(3):138-42. (Also see: PH Diagnosis and PH Treatments) |
Functional Classification of Pulmonary Hypertension |
| ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension. Idiopathic pulmonary arterial hypertension (IPAH) is more prevalent in women and was the most common type of PAH in the French registry. Familial PAH is inherited as an autosomal dominant disease with incomplete penetrance and genetic anticipation. PAH is also associated with congenital heart disease (CHD), connective tissue diseases, drugs and toxins, human immunodeficiency virus (HIV), portal hypertension, hemoglobinopathies, and myeloproliferative disorders. Primary PH formerly encompassed idiopathic, familial, and anorexigen induced PAH. These groups together comprise World Health Organization (WHO) Group I PAH. J Am Coll Cardiol, 2009. |
Markers for Prognosis of Pulmonary Hypertension |
| Pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc) in patients with functional class II dyspnoea: mild symptoms but severe outcome. A majority of patients with mildly symptomatic SSc-PAH in New York Heart Association (NYHA) functional class (FC) II at diagnosis have a severe disease with poor prognosis. Eric Hachulla. Rheumatology. February 8, 2010. |
| Biomarkers of vascular disease in scleroderma. Vascular disease is present in every patient with scleroderma and is a major source of morbidity and mortality. There is a subset of patients who will develop severe and sometimes life-threatening vascular events. (PubMed) Hummers LK. Rheumatology (Oxford). 2008 Oct;47 Suppl 5:v21-2. (Also see: Vascular Disease and Digital Ulcers) |
| Six-minute walk test for the evaluation of pulmonary disease severity in scleroderma patients. Hemoglobin desaturation during a 6 minute walk test (MWT) provides additional information regarding severity of disease in scleroderma patients with pulmonary manifestations. PubMed. Chest. 2007 Jan;131(1):217-22. (Also see:Pulmonary Fibrosis and PH Diagnosis) |
| Hormone can predict pulmonary hypertension and potential death or survival. Measuring levels of a hormone called brain natriuretic peptide in individuals with serious lung disease can predict the presence of pulmonary hypertension and a patient's potential death or survival, regardless of clinical severity or the cause of illness. EurekAlert! 03/31/06. |
| Changes in Causes of Death in Systemic Sclerosis Over the Past 30 Years. Throughout the past 30 years, the frequency of deaths from RC (renal crisis) has dramatically decreased and at the same time the frequency of PF (pulmonary fibrosis) increased. However, only 10% of SSc patients surviving RC have died of PF, which may be because patients with the highest frequency of RC have a low frequency of PF. Virginia Steen. 1052/432. ACR 2004. (Also see: Renal Involvement, Pulmonary Fibrosis, and Types of Scleroderma: Prognosis and Mortality) |
Stories of Prognosis of Pulmonary Hypertension |
| Syl: Systemic Scleroderma/CREST/Pulmonary Hypertension/Sjögren's The physician's assistant noticed that my hands went really blue... |
| Tony B: Systemic Scleroderma with Pulmonary Fibrosis, Pulmonary Hypertension and Raynaud's It has taken me some time to come to terms with this disease... |
