Pulmonary arterial hypertension (PAH) is a rare disease characterised by pulmonary vascular remodelling and elevated pulmonary pressure, which eventually leads to right heart failure and death. Registries worldwide have noted a female predominance of the disease, spurring particular interest in hormonal involvement in the disease pathobiology. Several experimental models have shown both protective and deleterious effects of oestrogens, suggesting that complex mechanisms participate in PAH pathogenesis. In fact, oestrogen metabolites as well as receptors and enzymes implicated in oestrogen signalling pathways and associated conditions such as BMPR2 mutation contribute to PAH penetrance more specifically in women. Conversely, females have better right ventricular function, translating to a better prognosis. Along with right ventricular adaptation, women tend to respond to PAH treatment differently from men. As some young women suffer from PAH, contraception is of particular importance, considering that pregnancy in patients with PAH is strongly discouraged due to high risk of death. When contraception measures fail, pregnant women need a multidisciplinary team-based approach. This article aims to review epidemiology, mechanisms underlying the higher female predominance, but better prognosis and the intricacies in management of women affected by PAH.
BackgroundThe impact of pulmonary rehabilitation (PR) on survival in patients with fibrotic interstitial lung disease (ILD) is unknown. Given the challenges conducting a large randomised controlled trial, we aimed to determine whether improvement in 6-minute walk distance (6MWD) was associated with better survival.MethodsThis retrospective, international cohort study included patients with fibrotic ILD participating in either inpatient or outpatient PR at 12 sites in 5 countries. Multivariable models were used to estimate the association between change in 6MWD and time to death or lung transplantation accounting for clustering by centre and other confounders.Results701 participants (445 men and 256 women) with fibrotic ILD were included. The mean±SD ages of the 196 inpatients and 505 outpatients were 70±11 and 69±12 years, respectively. Baseline/changes in 6MWD were 262±128/55±83 m for inpatients and 358±125/34±65 m for outpatients. Improvement in 6MWD during PR was associated with lower hazard rates for death or lung transplant on adjusted analysis for both inpatient (HR per 10 m 0.94, 95% CI 0.91 to 0.97, p<0.001) and outpatient PR (HR 0.97, 95% CI 0.95 to 1.00, p=0.042). Participation in ≥80% of planned outpatient PR sessions was associated with a 33% lower risk of death (95% CI 0.49% to 0.92%).ConclusionsPatients with fibrotic ILD who improved physical performance during PR had better survival compared with those who did not improve performance. Confirmation of these hypothesis-generating findings in a randomised controlled trial would be required to definitely change clinical practice, and would further support efforts to improve availability of PR for patients with fibrotic ILD.
Lung cancer is the most common cancer worldwide. It also has the highest malignancy-associated mortality rate. Treatment options are limited by cancer and tumor heterogeneity, resistance to treatment options, and an advanced stage at time of diagnosis, all of which are common. Statins are a class of lipid-lowering medications that have been studied for their antitumor effects in various types of cancers. Multiple mechanisms have been proposed to explain their observed off-target effects. Most of these hypotheses focus largely on statin-induced upregulation of proapoptotic signaling pathways and mediators, and the downregulation of antineoplastic factors secondary to statin use. Preclinical and clinical studies support their use for conferring a mortality benefit and improving treatment effect in some chemotherapy-resistant subtypes of lung cancer. However, their exact mechanism of action, class-dependent effect, dose-dependent effect, potential use as adjuvant chemotherapeutics, and markers of statin-sensitivity in specific lung cancer subtypes remain areas of ongoing investigation. Herein, we review the latest literature pertinent to the role statins can play in the management of lung cancers.
Peripartum cardiomyopathy (PPCM) is a rare but potentially fatal disease. The management of PPCM is individualized, and an early diagnosis is instrumental in the institution of an appropriate management plan. Here, we present a dramatic case of PPCM that was managed with a period of mechanical circulatory support. ( Level of Difficulty: Beginner. )
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