Current opinion in pulmonary medicine
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Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a common association adversely affecting quality of life and survival in these patients. We provide herewith recent advances in the understanding and management of PAH-CHD. ⋯ Even with advances in diagnosis and treatment PAH-CHD remains a challenging field requiring lifelong follow-up and meticulous treatment in centres specialized in both CHD and PAH.
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Pulmonary hypertension in sarcoidosis is a well known entity. Sarcoidosis-associated pulmonary hypertension (SAPH) incurs substantial morbidity and mortality. This review examines recent literatures published on epidemiology, prognosis and therapeutic management in SAPH. ⋯ Several registries have highlighted SAPH portends an unfavorable consequence. On the contrary, no published guideline exists to treat SAPH. The precise role of immunosuppressive agents is unclear. The limited evidence favoring use of pulmonary vasodilators arise from small retrospective case series and/or single-center nonrandomized observational studies. Further multicenter randomized research is warranted to better define patient population to treat and how best to treat them.
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To provide an overview of recent studies that could be helpful in a better understanding of rheumatoid arthritis-associated interstitial lung disease (RA-ILD) and to facilitate the clinical management of this severe complication of RA. ⋯ The phenotypical, environmental, and genetic similarities between IPF and RA-ILD have led to a better understanding of the underlying pathogenesis of RA-ILD. Despite the identification of several biomarkers and useful screening tools, several questions remain unanswered regarding the identification of patients with RA at increased risk of ILD and risk of progression. Other substantial gaps are the lack of recommendations for how high-risk patients should be screened and which specific therapeutic strategy should be initiated. International collaborative efforts are needed to address these issues and develop specific recommendations for RA-ILD.
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Treatment options for managing patients with acute pulmonary embolism are rapidly evolving. In this review, we discuss the supporting evidence and implementation strategies for these advanced therapeutic modalities. ⋯ A review of advanced therapies for pulmonary embolism.
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In this review, the authors describe therapeutic strategies for a disease group called progressive fibrosing interstitial lung disease (PF-ILD) and highlight the importance of the definition of progression, prognosis, and treatment response. ⋯ Recent evidence supports the use of antifibrotic therapy in the management of the phenotype progressive non-IPF ILD. Ongoing studies exploring genetic and other molecular biomarkers could identify at-risk individuals or predict treatment response and prognosis (endotypes). This would support the concept of 'treatable traits' in the field of ILD.