| LUNG (PULMONARY): MAIN MENU |
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| Pulmonary Hypertension: MAIN MENU |
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| Prognosis of Pulmonary Hypertension in Systemic Sclerosis (Scleroderma) |
| This page was written by Shelley Ensz, and has not been medically edited. Scleroderma (SD) affects everyone differently. Just because something is listed here does not mean an individual patient will ever experience it. See 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) |
| 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 |
| A New Classification of Pulmonary Hypertension. Includes World Health Organization (WHO) Functional Classification of Pulmonary Hypertension. Pulmonary Hypertension Association. |
| Markers for Prognosis of Pulmonary Hypertension |
| 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. |
| Lung and Kidney Involvement in Diffuse and Limited Cutaneous Systemic Sclerosis After 5-26 Years of Disease Duration - Study of 184 Patients. After at least 5, up to 26 years of disease duration, patients with diffuse cutaneous systemic sclerosis had significantly higher frequency of lung and kidney involvement compared to patients with limited cutaneous systemic sclerosis. Extent of skin involvement was a good predictor of more severe disease in patients with disease duration of 5, and longer than 5 years. S. Damjanov. FRI0115 EULAR 2005. Also see: Types of Scleroderma and Kidney Involvement) |
| 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... |
