Abstract

SANCHEZ ROMAN, Julio et al. Diagnosis and treatment of pulmonary hypertension in patients with scleroderma. Insuf. card. [online]. 2012, vol.7, n.1, pp.16-28. ISSN 1852-3862.

Pulmonary arterial hypertension (PAH) associated with systemic autoimmune diseases is the most common variant of PAH, behind only the idiopathic forms. Within those, which is associated with systemic sclerosis is also the most frequent (between 16 and 54% according to different series). Patients with scleroderma (or systemic sclerosis) can have virtually all variants of pulmonary hypertension in support of a classification of Dana Point (2008), currently in effect. PAH (related to alterations in the pulmonary vascular bed itself and without other apparent cause) is detected in a variable percentage ranging between 5 and 20% of patients with scleroderma. This variant is characterized by a worse prognosis than that seen in patients with idiopathic PAH due of the frequent comorbidity in these patients and the impact of the autoimmune process and profibrotic on the heart and great vessels lung. However, the fact that patients with PAH associated with systemic sclerosis belong to a known risk group, allows earlier detection and, therefore, the establishment of treatment at earlier stages, circumstances have greatly improved its survival and quality of life. The algorithms for diagnosis and treatment of these patients have their own variants and are similar to those used for idiopathic pulmonary hypertension. Attempts to use immunosuppressive drugs have proved quite ineffective.

Keywords : Pulmonary arterial hypertension; Systemic autoimmune diseases; Systemic sclerosis.