| Pulmonary Hypertension |
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Isolated Pulmonary Hypertension in Systemic Sclerosis |
| Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer |
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Overview of Isolated Pulmonary Hypertension in Systemic 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) |
| Post-Menopause is the Main Risk Factor for Developing Isolated Pulmonary Hypertension in Systemic Sclerosis. Postmenopausal condition alone or in combination with HLA-B35 and CREST syndrome is the main risk-factor for developing primary pulmonary hypertension in scleroderma patients. This suggests that hormonal replacement therapy could play a role in preventing isolated PHT in patients with systemic sclerosis. Neuroendocrine Immune Basis of the Rheumatic Diseases II: Proceedings of the Second International Conference, Volume 966, Jan 2006. (Also see: Autoimmunity and Early Menopause) |
| 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. |
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Prevention of Isolated Pulmonary Hypertension in Systemic Sclerosis (Scleroderma) |
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| 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. |
