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Anti-U1 RNP positivity linked to lower mortality rate in patients with CTD- and SSc-associated PAH
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Patients with connective tissue disease and systemic sclerosis who were positive for anti-U1 RNP antibodies had a decreased mortality risk, according to recently published research.
Data from a group of 342 patients with pulmonary arterial hypertension (PAH) and connective tissue disease (CTD) who attended the Royal Free Hospital in London were retrospectively studied. Thirty-six of the patients were positive for anti-U1 RNP antibodies, 14 of the patients had systemic sclerosis (SSc); 10 had systemic lupus erythematosus (SLE); 10 had mixed CTD (MCTD); and two patients had overlapping SSc and SLE.
Analysis showed patients with anti-U1 RNP antibodies tended to be younger and had a shorter CTD duration at the time of pulmonary hypertension (PH) diagnosis compared to patients without these antibodies. Functional impairment was lower in patients with anti-U1 RNP antibodies. More patients with these antibodies were classified as WHO class I or II compared to patients without the antibodies, who were more likely to meet WHO class III or IV criteria. Patients with positive antibody status also had a greater 6-minute walk distance. Mean diffuse lung capacity for carbon monoxide (DLCO) was higher for this group compared to anti-U1 RNP-negative patients.
Cox regression revealed better survival rates among patients with anti-U1 RNP-positivity in a univariate analysis. Antibody status, sex, age at PH diagnosis, WHO functional class, 6-minute walk distance, DLCO, right atrial pressure, oxygen saturation of arterial blood and the percentage of oxygen saturation of mixed venous blood were significantly associated with mortality.
No associations were identified between mortality and the CTD duration at the time of PH diagnosis or mean aortic pressure. Multivariate analysis showed anti-U1 RNP positivity remained significantly associated with better survival. Five-year survival rates were 71%, and 10-year survival rates were 36% for patients with anti-U1 RNP-positivity. Survival rates for negative patients were 41% and 20%, respectively. Investigators found that in patients with SSc, the association was not significant in univariate and multivariate analyses. – by Shirley Pulawski
Disclosures: Sobanski reports the receipt of a fellowship funded by the Association des Sclerodermiques de France, the Societe Nationale Francaise de Medecine Interne, Groupe Pasteur Mutualite, Institut Servier and GlaxoSmithKline. Please see the full study for a list of all other authors’ relevant financial disclosures.
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