Pulmonary arterial hypertension in limited cutaneous systemic sclerosis: a distinctive vasculopathy.

Pulmonary arterial hypertension in systemic sclerosis: a distinctive endotheliopathy?

We read with great interest the recent article by OVERBEEK et al. [1] on the histological differences in pulmonary vessels from patients with pulmonary arterial hypertension (PAH) related to systemic sclerosis (SSc) versus idiopathic PAH (IPAH). The study demonstrates that pulmonary pathological features in patients suffering from limited cutaneous SSc (lcSSc) differ from the ones seen in IPAH,...

Different right ventricular contractility in limited cutaneous systemic sclerosis- associated pulmonary arterial hypertension and idiopathic pulmonary arterial hypertension

Increased plasma soluble CD40 ligand concentrations in systemic sclerosis and association with pulmonary arterial hypertension and digital ulcers.

BACKGROUND Increased expression of CD40L has been reported on activated CD4+ T lymphocytes in systemic sclerosis. CD40L can be expressed in soluble form (sCD40L). OBJECTIVE To compare sCD40L concentrations in patients with systemic sclerosis and healthy controls. METHODS Quantitative sandwich ELISA was used to measure plasma sCD40L in systemic sclerosis (n = 50) and matched healthy controls...

Connective tissue disease-associated pulmonary arterial hypertension

Although rare in its idiopathic form, pulmonary arterial hypertension (PAH) is not uncommon in association with various associated medical conditions, most notably connective tissue disease (CTD). In particular, it develops in approximately 10% of patients with systemic sclerosis and so these patients are increasingly screened to enable early detection. The response of patients with systemic sc...

EUROPEAN RESPIRATORY UPDATE Scleroderma lung disease

S ystemic sclerosis (SSc) is a systemic autoimmune disease that is characterised by endothelial dysfunction resulting in a small-vessel vasculopathy, fibroblast dysfunction with resultant excessive collagen production and fibrosis, and immunological abnormalities. The classification of SSc is subdivided based on the extent of skin involvement into diffuse cutaneous sclerosis (dcSSc), limited cu...