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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
Overview of PH Prognosis
Functional Classification of PH
Markers for Prognosis of PH
Personal Stories of Prognosis of PH
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 )
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
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...
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