Raynaud's phenomenon and pulmonary arterial hypertension

Abstract

Sir-We have read with interest the paper of Kasparian, et al. 1 about Raynaud´s phenomenon (RP) in systemic lupus erythematosus and its correlation with elevated pulmonary arterial systolic pressure (PASP). We present the results of our study in patients with scleroderma and the correlation observed between severity of RP and PASP with exercise.We performed exercise echocardiography in 49 patients with systemic scleroderma. In eight patients, the flow of tricuspid regurgitation could not be measured, so the final sample included 41 patients, 27% with diffuse form and 73% with limited form. The PASP was calculated using maximum speed of tricuspid insufficiency according to common practice (PASP = 4v 2 + right atrial pressure) at peak of maximal exercise using a cycloergometer according to World Heart Organization´s protocol. We defined severe RP if ulcers or digital necrosis was present or if it was necessary to treat with bosentan, sildenafil or prostaglandin. To evaluate the possible effect of these treatments on PASP, a drug-free interval of at least 3 months was required. All patients had a normal PASP at rest defined as a PASP <35 mmHg. A nonparametric Mann-Whitney test was used for ecocardiographic comparisons between severe and nonsevere RP and Chi-square test for categorical data comparisons.RP was present in 97.5% of patients and was considered severe in eight patients (19.5%). In this latter group, the PASP at exercise was significantly higher than in patients with nonsevere RP (54.3 mmHg vs 41.6 mmHg; P < 0.05). In a group of patients with the highest PASP (>65 mmHg), the prevalence of severe RP was 3/4 (75%) patients. Right heart catheterization only was performed in the group of patients with PASP >65 mmHg at exercise, and in all cases, the pulmonary arterial medial pressure at rest was normal.

Clinical characteristics and survival of patients with three major connective tissue diseases associated with pulmonary hypertension: A study from China

“…Mechanistically, Raynaud's phenomenon is associated with systolic and diastolic dysfunction of the acral arteriole. When it affects the pulmonary artery, the condition is called ‘pulmonary Raynaud's phenomenon’ ( 24 ). The persistent vasospasm of pulmonary arterioles may result in pulmonary vascular remodeling and increased pulmonary vascular resistance ( 24 ).…”

“…When it affects the pulmonary artery, the condition is called ‘pulmonary Raynaud's phenomenon’ ( 24 ). The persistent vasospasm of pulmonary arterioles may result in pulmonary vascular remodeling and increased pulmonary vascular resistance ( 24 ). In the present study, Raynaud's phenomenon appeared to be more common in the group of CTD-PH non-survivors compared with survivors.…”

The Clinical Presentation of Systemic Lupus Erythematosus

“…Evidence of severe restrictive or obstructive disease in SLE is unusual, although this can be the major manifestation in some variant forms of the disease [112] and is suspected when the carbon monoxide diffusing capacity is very low (DLCO).…”