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Pulmonary arterial hypertension in systemic sclerosis (scleroderma): Definition, classification, risk factors, screening, and prognosis

INTRODUCTION

Pulmonary complications of systemic sclerosis (SSc) are common, and are the leading cause of SSc-related death. The most common pulmonary manifestations of SSc are pulmonary hypertension (PH), interstitial lung disease (ILD), and any combination thereof.

The classification, definition, risk factors, screening, and prognosis of SSc-associated pulmonary arterial hypertension (PAH), specifically group 1 PAH, are reviewed here. Group 2 PH and group 3 PH are discussed separately. (See "Pulmonary hypertension associated with interstitial lung disease" and "Treatment of pulmonary hypertension in adults", section on 'Group 2 PH' and "Treatment of pulmonary hypertension in adults", section on 'Group 3 PH'.)

The evaluation and diagnosis of lung disease in SSc as well as clinical manifestations, diagnosis, and treatment of SSc-associated pulmonary arterial hypertension are discussed elsewhere. (See "Evaluation for and diagnosis of lung disease in systemic sclerosis (scleroderma)" and "Clinical manifestations of systemic sclerosis (scleroderma) lung disease" and "Pulmonary vascular disease in systemic sclerosis (scleroderma): Treatment" and "Prognosis and treatment of interstitial lung disease in systemic sclerosis (scleroderma)".)

WHO CLASSIFICATION

Many different forms of pulmonary hypertension (PH) occur in systemic sclerosis (SSc). The World Health Organization (WHO) classifies patients with PH into five groups, as shown in the table (table 1) [1]. SSc is most often associated with group 1 pulmonary arterial hypertension (PAH) and group 3 PH, and less commonly with group 2 PH. Due to the varied and sometimes mixed etiology underlying PH in SSc, the precise classification of the type of PH can be challenging.

The following is a brief description of the WHO classification of PH:

                  

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Literature review current through: Sep 2014. | This topic last updated: Apr 29, 2014.
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