LUNG (PULMONARY): MAIN MENU
Lung (Pulmonary) Overview
Aspiration Pneumonia
Oxygen Therapy
Pleural Effusion
Pulmonary Fibrosis
Pulmonary Hypertension
Preventive Care
Pulmonary Support Resources
Pulmonary Hypertension: MAIN MENU
What is PH?
Primary PH
Isolated PH
Secondary PH
Exercised Induced PH
PH Disease Correlations
Symptoms
Diagnosis
Prognosis
Treatments
Research Registries
Research
Patient Support Organizations
Personal Stories
Isolated 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 Isolated PH/PAH
Predictors of Isolated PH/PAH
Prevention of Isolated PH/PAH
Overview of Isolated Pulmonary Hypertension in Systemic Sclerosis (Scleroderma)
In systemic sclerosis (scleroderma), pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH) often occurs secondary to severe pulmonary fibrosis, kidney failure, or cardiomyopathy with diastolic dysfunction. When it occurs by itself, not secondary to such other causes, it is called isolated pulmonary hypertension.
Isolated pulmonary hypertension in scleroderma. Isolated PHT occurs as a late-stage complication in >11% of patients with limited scleroderma approximately 20 years after the first symptoms of scleroderma. Susceptible patients were characterized by the presence of multiple telangiectasia, reduced nailfold capillary density, digital ulceration, gross reduction of diffusing capacity for carbon monoxide and echocardiographic evidence of elevated pulmonary artery pressure. There is a mean survival of 2.5 years from symptomatic onset of PHT. The early use of endothelin-1 receptor antagonists may change the natural history of this fatal complication. PubMed. Intern Med J. 2005 Jan;35(1):28-33. (Also see: Types of Scleroderma and Limited Scleroderma.)
Pulmonary arterial hypertension in systemic sclerosis: clinical manifestations, pathophysiology, evaluation, and management. It is increasingly recognized that significant pulmonary arterial hypertension (PAH) develops in more than 15% of patients with systemic sclerosis (SSc). As this complication of SSc may occur even in the absence of overt interstitial lung disease (isolated PAH), it has been likened to primary PAH and is attributable to intrinsic vascular pathology that is the hallmark of SSc. PubMed. Treat Respir Med. 2004;3(6):339-52.
Predictors of Isolated Pulmonary Hypertension in Systemic Sclerosis (Scleroderma)
Early detection of PH risk in scleroderma patients is necessary, since early treatment may modify or possibly even prevent PH. Predictors of Isolated Pulmonary Hypertension in Patients With Systemic Sclerosis and Limited Cutaneous Involvement. Patients with isolated PHT have, at some point, rapidly progressive dyspnea on exertion, leading to right heart failure and death from hypoxemia and ventricular arrhythmia. As medications are developed that have the potential to modify or even prevent PHT, it is important to identify, early in the course of SSc, those patients who are most likely to develop PHT. Decreasing DLCO is an excellent predictor of the subsequent development of isolated PHT in limited scleroderma. The DLCO may be significantly decreased for many years prior to the diagnosis of PHT. The presence of autoantibodies and the PAP may also be helpful predictors.Virginia Steen and Thomas A. Medsger, Jr. Arthritis & Rheumatism, Vol. 48, No. 2, February 2003, pp 516–522.
Prevention of Isolated Pulmonary Hypertension in Systemic Sclerosis (Scleroderma)
Hormone replacement therapy may prevent isolated PH in scleroderma patients. Hormone replacement therapy may prevent the development of isolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement. Twenty patients (32.8%) received HRT for a mean of 6.7±3.7 years. None of these patients developed iPHT after a mean of 7.2±3.5 years from menopause, whereas eight out of 41 patients not receiving HRT (19.5%) developed iPHT. HRT administration may be effective in SSc post-menopausal women, preventing the development of iPHT. Scandinavian Journal of Rheumatology, 006, vol. 35, no6, pp. 468-471.
Sclerosing panniculitis is associated with pulmonary hypertension in systemic sclerosis. Our results suggest that thrombosis caused by venous hypertension of the leg may be the main cause of PH in patients with SSc and sclerosing panniculitis. Sclerosing panniculitis may be a useful marker of PH in patients with SSc. PubMed. Br J Dermatol. 2005 Sep;153(3):579-83.
Keep on Surfing!
Go to Pulmonary Hypertension: Prognosis
Contact ISN. We are a full-service nonprofit agency.
Email: isn@sclero.org or
or post a message in our Sclero Forums !
Shop, Join or Donate Now
(Also see: Contact ISN )
International Scleroderma Network (ISN)
7455 France Ave So #266
Edina, MN 55435-4702
USA
Toll Free Hotline in U.S. 1-800-564-7099
Direct Line 952-831-3091 (U.S.)
(We are also known as the Scleroderma from A to Z web site.)
© Copyright 1998-2008 International Scleroderma Network
All Rights Reserved