Selexipag treatment in patients with systemic sclerosis-associated pulmonary arterial hypertension in clinical practice, a case series.

Objective: To describe the efficacy and safety in all patients with systemic sclerosis-associated pulmonary arterial hypertension who started selexipag between 09-2016 and 06-2018 in two pulmonary arterial hypertension expert centers. Methods: All patients with systemic sclerosis-associated pulmonary arterial hypertension diagnosed by right heart catheterization and treated with selexipag were included. Every 12 weeks, treatment effect was assessed by (1) the opinion of the expert team and (2) the abbreviated risk assessment, consisting of functional class, six-minute walking distance, and N-terminal prohormone of brain natriuretic peptide level at baseline and during follow-up. Side effects and adverse events were registered. Results: We included 13 systemic sclerosis-associated pulmonary arterial hypertension patients, 10 patients were female, median age (interquartile range) of 68 (58-75) years, median systemic sclerosis disease duration of 7.4 (4.7-13.5) years, and median pulmonary arterial hypertension duration of 4 (2.5-7.5) years. Two patients discontinued selexipag within 4 weeks due to side effects. The remaining 11 patients had a median follow-up duration of 48 (interquartile range = 24-72) weeks. Two patients died (one pulmonary arterial hypertension-related, the other systemic sclerosis-related). According to the expert team, 8 of 11, 9 of 10, and 5 of 7 patients stabilized or improved at 12, 24, and 48 weeks, respectively. According to the abbreviated risk assessment at study end, 3 of 11 patients had 1 low-risk criterion. No previously unrecorded side effects were reported. Conclusion: Adding selexipag to background therapy in a high-risk cohort of systemic sclerosis-associated pulmonary arterial hypertension patients provided sustained stabilization of symptoms with an acceptable safety profile. Improvement was reached in only two of our patients. Further research should focus on systemic sclerosis-associated pulmonary arterial hypertension patients treated with multiple targeted treatments, preferably these patients should be prospectively followed in international registries.

The Course of Obstructive Sleep Apnea Syndrome in Patients With Acromegaly During Treatment.

BACKGROUND: Obstructive sleep apnea syndrome (OSAS) is common in active acromegaly and negatively influences quality of life, morbidity, and mortality. This prospective study with 3 predetermined timepoints and a standardized treatment protocol investigates changes in sleep parameters during the first 2.5 years of acromegaly treatment. METHODS: Before initiation of acromegaly treatment (medical pretreatment followed by surgery), polysomnography (PSG) was performed in 27 consecutive patients with treatment-naive acromegaly. PSG was repeated after 1 year (N = 24) and 2.5 years (N = 23), and anthropometric and biochemical parameters were obtained. RESULTS: At baseline, 74.1% of the patients was diagnosed with OSAS. The respiratory disturbance index (RDI; P = 0.001), oxygen desaturation index (ODI; P = 0.001), lowest oxygen saturation (LSaO2; P = 0.007) and the Epworth Sleepiness Scale (ESS; P < 0.001) improved significantly during treatment, with the greatest improvement in the first year. After 2.5 years of treatment, all patients had controlled acromegaly. Of the 16 patients with repeated PSG and OSAS at baseline, 11 (68.8%) were cured of OSAS. Changes in RDI, ODI, LSaO2, and ESS correlated with insulin-like growth factor 1 levels. CONCLUSION: OSAS has a high prevalence in active acromegaly. There is a substantial decrease in prevalence and severity of OSAS following acromegaly treatment, with the largest improvement during the first year. Most patients recover from OSAS following surgical or biochemical control of the acromegaly. Therefore, a PSG is advised after diagnosis of acromegaly. When OSAS is present, it should be treated and PSG should be repeated during acromegaly treatment.

Extensive pulmonary sarcoid reaction in a patient with BMPR-2 associated idiopathic pulmonary arterial hypertension.

Pulmonary arterial hypertension is a progressive life-threatening disease characterized by vascular remodeling. There is evidence that varied immune mechanism play an important role in progression of pulmonary hypertension.  We describe a case of a 35-year-old woman with idiopathic pulmonary arterial hypertension (IPAH) and a novel BMPR2 mutation, who underwent a successful lung transplantation.  Extensive granulomatous inflammation was seen in the resected lungs. The granulomatous inflammation found in the histology supports  a sarcoid-like reaction due to pulmonary hypertension in the context of the BMPR2 mutation.

The CareWell in Hospital program to improve the quality of care for frail elderly inpatients: results of a before-after study with focus on surgical patients.

BACKGROUND: The objective of this study was to evaluate implementation of an innovative intervention designed to prevent complications and stimulate early rehabilitation among frail elderly inpatients. METHODS: The program was implemented in April 2011. A mixed-methods process evaluation and before-after study were performed. Primary effect outcomes included incidence of hospital-acquired delirium, cognitive decline, and decline in activities of daily living (ADL) during hospital stay. Secondary endpoints included ADL performance 3 months postdischarge, readmission, and caregiver burden. RESULTS: One hundred ninety-one preintervention and 195 postintervention patients aged 70 years or older were included. Overall, no significant differences in primary endpoints were found. Mean ADL between discharge and follow-up improved (3.2 vs 5.7, P = .058). Caregivers rated burden of care lower at 3 months postdischarge (.5 vs -.6, P = .049). CONCLUSIONS: The CareWell in Hospital program was implemented satisfactorily. Although the low baseline delirium incidence (11%), higher comorbidity, and an increasing learning curve during a restricted implementation period potentially influenced the overall effects, this integrated care program may have beneficial effects on outcomes among frail elderly surgical patients.

Increasing evidence for gender differences in chronic obstructive pulmonary disease.

The prevalence of chronic obstructive pulmonary disease (COPD) among women is increasing and differences in both the management of COPD and the results of treatment between men and women have been noted. This article investigates the reasons for this increase in prevalence and the differences in natural history and COPD management between male and female patients. The main reason for the rise in prevalence of COPD in women is increased tobacco use. An additional factor is the greater susceptibility of women to damage from smoke and air pollution. The health-related quality of life is worse in women when compared with men with the same severity of disease. In addition, nutritional status is often worse in women. The most important treatment for COPD is to stop smoking. Women appear to be more dependent on cigarettes than men, and have greater difficulties stopping smoking, especially when they live with a partner who smokes. Rehabilitation is an effective treatment for both male and female COPD patients, but the focus is different: women need more emotional support and social interaction to achieve the best results.