Prognosis of Pulmonary Hypertension in Systemic Sclerosis

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Overview of PH Prognosis
Functional Classification of PH
Markers for Prognosis of PH
Personal Stories of Prognosis of PH


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)

Survival protection by bodyweight in isolated scleroderma–related pulmonary artery hypertension. The results suggest body mass index decline should be contrasted, lung diffusion is useful for screening and with stroke volume index and mixed venous saturation for assessing prognosis and treatment. PubMed, Intern Emerg Med, 04/06/2016.

Sex disparities in systemic sclerosis (SSc)–associated pulmonary arterial hypertension (PAH): a cohort study. Sex disparities appear to exist in the frequency of PAH, time to PAH diagnosis, PAH disease duration and SSc disease burden. PubMed, Arthritis Res Ther, 2016 Jan 27;18(1):30.

Long-term survival of patients with pulmonary arterial hypertension (PAH). Patients with PAH who achieve recovery to WHO-FC I or II without use of intravenous epoprostenol have similar survival to those who reach the same WHO-FC with use of intravenous epoprostenol. BMC Research Notes, June 2014; 7: 359. (Also see Pulmonary Hypertension Treatments)

Pulmonary Hypertension (PH) Surveillance. Continued surveillance helps us understand and address the evolving trends in hospitalization and mortality associated with PH and PH-associated conditions, especially regarding sex, age, and race/ethnicity disparities. Chest, 2014;146(2):476-495.

Prediction of pulmonary complications and long term survival in systemic sclerosis (SSc). This study provides data on long-term outcome of SSc and the timing and frequency of major organ complications. PubMed, Arthritis Rheumatol, 03/03/2014. (Also see Pulmonary Involvement)

Left Ventricular Ejection Time (LVET) in Acute Heart Failure Complicating Precapillary Pulmonary Hypertension. Shortened LVET at ICU admission was a prognostic factor in patients with precapillary PH with heart failure. Chest, 2013;144(5):1512-1520.

Survival in systemic sclerosis(SSc)-associated pulmonary arterial hypertension (PAH) in the modern management era. SSc-associated PAH remains a devastating disease and age, male gender, and cardiac index were the main prognosis factors. Early detection of less severe patients should be a priority. PubMed, Ann Rheum Dis, 2013 Dec 1;72(12):1940-6.

Survival remains poor for systemic sclerosis-associated pulmonary hypertension. Despite the frequent assertion that survival has improved in recent years for patients who have pulmonary hypertension associated with systemic sclerosis, a meta-analysis that allowed pooling of data from numerous small studies shows otherwise. Rheumatology News, 09/05/2013.

Survival in Systemic Sclerosis with Pulmonary Arterial Hypertension Has Not Improved in the Modern Era. Despite an improvement in clinical status, unlike patients with pulmonary arterial hypertension, the mortality in systemic sclerosis has not improved since the introduction of epoprostenol. CHEST Journal, June 6 2013. (Also see Flolan/Prostacyclin/Epoprostenol)

Survival and prognostic factors in systemic sclerosis (SSc)-associated pulmonary hypertension (PAH). This analysis confirms a poor pooled 3-year survival rate of 52% (47-58) in SSc-associated PH. PubMed, Arthritis Rheum, 2013 Jun 5.

Demographics and Outcomes of Patients Diagnosed With Pulmonary Hypertension (PAH) With Pulmonary Capillary Wedge Pressures (PCWP) 16 to 18 mm Hg. Insights from the REVEAL Registry. Patients with PCWP 16 to 18 mm Hg who were diagnosed and treated for PAH were older, heavier, and more likely to have comorbidities associated with left ventricular diastolic dysfunction. Five-year survival rates were similarly low for all PCWP subgroups. CHEST,January 2013, Vol 143, No. 1 (Also see REVEAL Registry)

Epidemiology and risk factors for pulmonary hypertension (PH) in systemic sclerosis (SSc). The ultimate objective in understanding the risk of developing PH in SSc is early diagnosis and early initiation of appropriate therapy with the hope for improved outcomes for patients with SSc-PH. PubMed, Curr Rheumatol Rep, 2013 Jan;15(1):302. (Also see Prognosis in Systemic Sclerosis)

Pulmonary hypertension deaths and hospitalizations on the rise. Death rates throughout the reporting period 1990-2008 have been higher for blacks than for whites. In addition, a higher rate of hospitalization was observed in the Northeast than in other regions of the United States. MedicalXpress. 10/22/12.

Functional Classification of Pulmonary Hypertension

Functional Classification of Severe Pulmonary Hypertension. The higher the class the more severe the disease and the greater the urgency for the patient to be accurately diagnosed and started on therapy for pulmonary hypertension. Pulmonary Hypertension RN.

Markers for Prognosis of Pulmonary Hypertension

Aberrant immune response with consequent vascular and connective tissue remodeling – causal to scleroderma and associated syndromes? Therapeutic helminth infection or treatment with parasite–derived response modifiers could be promising new management tools for autoimmune connective tissue diseases. PubMed, Curr Opin Rheumatol, 2016 Nov;28(6):571-6.

The role of asymmetric dimethylarginine (ADMA) alone and in combination with N–terminal pro–B–type natriuretic peptide as a screening biomarker for systemic sclerosis–related pulmonary arterial hypertension (SSc–PAH): A case control study. In this small study, use of ADMA in combination with NT–proBNP produced excellent sensitivity and specificity for the non–invasive identification of SSc–PAH. PubMed, Clin Exp Rheumatol, 05/23/2016.

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.

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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