Rationale Pulmonary arterial hypertension (PAH) is a known, clinically significant complication of connective tissue diseases (CTDs), and patients with PAH linked to systemic sclerosis (SSc-PAH) have been found to have the highest mortality rates in PAH. The idiopathic inflammatory myopathies are a heterogenous group of CTDs that have been rarely associated with PAH and are often underappreciated by clinicians, and the clinical characteristics and overall prognosis of myositis-associated PAH (MA-PAH) has yet to be fully described. Methods We conducted a single-center, retrospective longitudinal multiple cohort study to investigate the clinical features, treatment responses, and survival of a previously under-reported cohort of CTD-PAH patients with myositis-associated autoantibodies compared to more traditional PAH subgroups of idiopathic pulmonary arterial hypertension (IPAH) and SSc-PAH patients. Patients with PAH confirmed by cardiac catheterization were enrolled from August 2012 to December 2018, and patients were classified based on clinical and serologic data (including myositis autoantibody testing). Patient characteristics were compared between groups using the Chi-square test and one-way ANOVA. Survival analysis was performed utilizing the Kaplan-Meier method and the log-rank test. Results 22 MA-PAH patients were compared to 68 IPAH and 42 SSc-PAH patients. The mean age of the overall cohort was 58 years old, and 104 (79%) of patients were female. IPAH patients were less likely to have anemia or chronic kidney disease and had better lung function than MA-PAH and SSc-PAH patients. There were no significant differences in baseline functional capacity or PAH-specific therapies between the three groups. Hemodynamically, IPAH patients had higher mean pulmonary artery pressures (mPAP) relative to MA-PAH patients and SSc-PAH patients, but otherwise had similar hemodynamic profiles. Estimated survival for the MA-PAH cohort was 91%, 71%, and 71% at one year, three years, and five years, respectively, with no statistically significant difference between the three groups. There was a statistically significant difference in survival if only the IPAH and SSc-PAH cohorts were compared (P=0.05). Conclusions Our findings demonstrate that MA-PAH patients clinically and hemodynamically more closely resemble patients with SSc-PAH, but do not have a statistically significant difference in survival. The significant difference in survival between the SSc-PAH and IPAH groups is similar to what has been previously described.
Background: Cardiovascular disease is the leading cause of death from Cushing’s syndrome (CS). Primary bilateral macronodular adrenal hyperplasia (PBMAH), a rare cause of CS, is clinically distinct from the other common types of CS, but its cardiac characteristics have been poorly studied. Methods: The clinical data, steroid hormones and echocardiographic variables were collected in 17 PBMAH patients. Twenty-one CS patients with cortisol-producing adenoma (CPA) were collected as controls. Results: In the PBMAH group, the proportion of female was lower (35.30 vs 100.00%), the age was older (55.76 ± 2.42 years vs 39.57 ± 2.72 years), and the course of disease was longer [13.00 (5.00, 21.50) years vs 1.58 (1.00, 5.00) years]. The proportion of PBMAH patients with hypertension was higher than that of CPA patients (100.00% vs 61.90%). Plasma cortisol and 24h urinary free cortisol (24h UFC) were lower in PBMAH patients than that in CPA patients [morning cortisol 19.50 (15.35, 24.48) μg/dl vs 28.30 (22.88, 29.89) μg/dl, 24h UFC 106.20 (65.35, 156.58) vs 506.23 (292.53, 712.18) μg]. The PBMAH group had longer right ventricular diameter (24.06 ± 1.23 mm vs 20.48 ± 0.83 mm), left atrial diameter (39.41 ± 1.15 mm vs 32.86 ± 0.76 mm), and a higher rate of diastolic dysfunction (76.50% vs 38.10%) than the CPA group. Univariate regression analysis showed that combination of hypertension and duration of hypertension had significant effects on left atrial diameter (b=6.383, P=0.001; b = 0.276, P<0.001, respectively) and E/A ratios (b=-0.496, P< 0.001; b=0.022, P<0.001, respectively). Multivariate regression analysis showed that corticosterone level had a significant effect on systolic blood pressure (b=6.712, P=0.025). Conclusion: Comparing to the CPA, ventricular diastolic dysfunction was common in PBMAH patients under relatively lower cortisol level. PBMAH had a high corticosterone level which may play a role in the development of hypertension and further heart changes.
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