Background Pulmonary arterial hypertension (PAH) is a devastating vascular complication of systemic sclerosis (SSc) and is found in 10-15% of cases carrying a severe prognosis. PAH has a dramatic impact on the clinical course and overall survival, being the most common cause of death in patients with this entity. Doppler echocardiography represents the best initial screening tool.
Objectives To determine the prevalence of PAH in mexican patients with systemic sclerosis.
Methods We studied 136 patients who met the American College of Rheumatology classification criteria for Systemic Sclerosis from January to December 2011 in the Internal Medicine Departments of a tertiary Hospital. This is a cross sectional study, that included patients of ages 18 or older with the diagnosis of SSc in the SSc outpatient clinic. We performed a Doppler Echocardiography and determined PAH. We used a descriptive statistical analysis.
Results There were 136 patients and the mean age was 51±13 years: women 125 (91.2%) and men 12 (8.7%). Median disease evolution: 6 years with a range from 1-32 years. The limited cutaneous SSc (lcSSc) was present in 70 patients (50.09%), diffuse cutaneous SSc (dcSSc) in 66 (48.17%) and Scleroderma sine Scleroderma in 1 (0.72%). PAH was found in 59 patients (43.3), of whom 23 (38.9%) had mild form, 27 (45.7%) moderate and 9 (15.2%) had severe PAH. In dsSSC PAH was found in 22 (33.8%) patients and in lcSSc in 36 (51.4%) patients
Conclusions The prevalence of PAH in SSc was 43%. Of our patients with PAH: 15% was severe, 27 moderate and 43% mild. Therefore we should make a prompt diagnosis and start an adequate treatment.
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Vera Lastra O, Porres. Pulmonary arterial hypertension associated with systemic sclerosis: Current diagnosis approach and therapeutic strategies. World J Rheumatol 2012; 28: 2:12-20
Disclosure of Interest None declared