Clinical characteristics and survival of Japanese patients with connective tissue disease and pulmonary arterial hypertension: a single-centre cohort

Abstract

The major PAH-CTD population in Japan suffers from MCTD or SLE with anti-U1RNP antibody, in contrast to PAH-CTD patients in the USA and Europe. Modern PAH treatment has improved survival rates, but long-term outcomes are still unsatisfactory. Independent predictors of mortality indicate that early diagnosis and the prompt use of PAH drugs should improve survival.

Predictors of Favorable Responses to Immunosuppressive Treatment in Pulmonary Arterial Hypertension Associated With Connective Tissue Disease

“…In this prospective cohort, all consecutive CTD patients with PAH were enrolled upon PAH diagnosis between 1970 and 1990 (pre-pulmonary vasodilator era) and after 2000 (modern treatment era). 12 PAH was diagnosed based on the following criteria: (1) mean pulmonary arterial pressure (mPAP) ≥25 mmHg at rest by right heart catheterization (RHC); (2) exclusion of left-sided heart disease, defined as pulmonary arterial wedge pressure >15 mmHg; (3) exclusion of advanced interstitial lung disease (ILD), defined as a predicted forced vital capacity <70%; and (4) exclusion of chronic thromboembolic pulmonary hypertension, based on a lack of apparent thromboembolism detected by ventilation or perfusion lung scanning. 10 From the entire cohort of 96 patients, 30 patients eligible for inclusion in the study were selected based on the following criteria: (1) SLE, MCTD, or primary SS as the underlying CTD, according to well-established classification criteria; 13-15 (2) those who received GC consisting of ≥0.5 mg/kg per day prednisolone (or its equivalent) for at least 2 weeks as the first-line IS treatment, with or without immunosuppressants; and (3) those who were followed for at least 3 months after enrollment.…”

Is Elevated Circulating Galectin-3 Level A Predictor of Pulmonary Artery Hypertension Development and Progression?

“…Compared with IPAH patients and familial PAH individuals, patients with PAH associated with CTD have an older age of onset and exhibited worse prognosis in survival [6] Survival of patients with respiratory disease-related or congenital heart disease-related PAH in the modern treatment era is better than CTD-APAH [6,7]. Although clinical features, hemodynamic parameters, echocardiography patter, multi-spiral computer tomography findings, exercise capacity, and anti-nuclear antibody profiles were found as powerful factors predicted a development of PAH due to several diseases [8], the reliability, sensitivity, specificity and predictive value are derived from PAH patients with different comorbidities and specific complications associated with CTD, congenital heart disease and respiratory disease might be unacceptable [9]. In this context, taken into consideration pathophysiological heterogeneity of PAH to risk stratification based on biological markers (N-terminal pro-brain natriuretic peptide, red cell distribution width, soluble endoglin, growth differentiation factor-15, interleukin-6, soluble vascular endothelial growth factor receptor-1, C-reactive protein, pentraxin 3) reflected several faces of nature evolution of the disease might be useful and appears to be attractive [10][11][12][13].…”

Ankylosing Spondylitis Associated with Pulmonary Arterial Hypertension

“…The onset of PAH in patients with CTD is common, with an incidence ranging from 0.5 to 15% according to the findings of right heart catheterization (3,4). In cohort studies conducted in the USA and Europe, the majority of patients with PAH associated with CTD (CTD-PAH) have been found to have systemic sclerosis (5-7), whereas systemic lupus erythematosus (SLE) and mixed connectivetissue disease (MCTD) are more common in Japanese patients with PAH (8).…”