Pulmonary Hypertension in Systemic Sclerosis

The main cause of death in systemic sclerosis is interstitial lung disease, followed by pulmonary hypertension (PH). Pulmonary hypertension is the result of microvasculopathy which is caused by a disrupted healing process of endothelin damage and is featured by vasoconstriction, proliferation of arterial wall, inflammation, and fibrosis. Reclassification of pulmonary hypertension has led to five distinctive groups. In systemic sclerosis, patients may suffer from pulmonary artery hypertension (PAH, group 1), pulmonary hypertension due to interstitial lung disease (group 3), cardiac disease (group 2), and/or thromboembolic pulmonary hypertension (group 4). Patients endure declining performance during exercise, but symptoms may be variable and nonspecific. Diagnosis is made by right heart catheterization. To select patients for this invasive procedure, several screening tools are discussed, including N-terminal pro-brain natriuretic peptide levels, uric acid levels, spirometry and diffusing capacity for carbon monoxide (DCLO), echocardiography (ECG), and the DETECT algorithm. Depending on features such as disease duration, presence of anti-centromere antibodies, and DCLO, three different flow charts for screening are presented. Based on pathophysiology, several medical treatments have been developed like prostanoids, endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and stimulation of the nitric oxide pathway. Combination therapy as well as lung transplantation and supportive therapy is discussed

How to coach student professional development during times of challenges and uncertainties

Abstract Background What we teach our (bio)medical students today may differ from the future context under which they will operate as health professionals. This shifting and highly demanding profession requires that we equip these students with adaptive competencies for their future careers. We aimed to develop a framework to promote and facilitate professional development from day one, guided by self-awareness and self-directed learning. Approach Based on self-directed, transformative and experiential learning, patient involvement and teamwork, we developed a 3-year longitudinal personal-professional development (LPPD) program in the (bio)medical sciences undergraduate curriculum to stimulate self-driven professional development in a variable context. Through group meetings and individual coach consultations, students address topics such as self-awareness, self-directed and lifelong learning, collaboration, well-being and resilience. To drive learning students receive extensive narrative feedback on an essay assignment. Evaluation Experiences and outcomes were evaluated with questionnaires and in-depth interviews. Students and coaches value personal and professional development in a safe learning environment that encourages self-exploration, diversity and connection. Over time, students show more self-awareness and self-directedness and increasingly apply trained skills, resulting in professional identity formation. Students need more clarification to understand the concept of assessment as learning. Implications With the generic content of a longitudinal program embedded in a meaningful environment, the personal and professional development of students can be facilitated and stimulated to face future challenges. When translating to other curricula, we suggest considering the complexity of professional development and the time expenditure needed for students to explore, experiment and practice. An early start and thorough integration are recommended