Clinics in chest medicine
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Cigarette smoking is a major preventable cause of morbidity and mortality. It is the major risk factor for chronic obstructive pulmonary disease in the developed world. Smoking is a chronic relapsing disease. ⋯ All clinicians should be comfortable with the use of nicotine replacement therapy, bupropion, and varenicline. Second-line therapies can be used by those familiar with their use. Effective use of these medications requires their integration into an effective management plan, which is likely to be a long-term undertaking, involving several cycles of remission and relapse.
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Chronic obstructive pulmonary disease (COPD) is currently defined as a common preventable and treatable disease that is characterized by persistent airflow limitation that is usually progressive and associated with an enhanced chronic inflammatory response in the airways and the lung to noxious particles or gases. Exacerbations and comorbidities contribute to the overall severity in individual patients. The evolution of this definition and the diagnostic criteria currently in use are discussed. COPD is increasingly divided in subgroups or phenotypes based on specific features and association with prognosis or response to therapy, the most notable being the feature of frequent exacerbations.
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Alpha1-antitrypsin (AAT) deficiency was first described in 1963 together with its associations with severe early-onset basal panacinar emphysema. The genetic defects leading to deficiency have been elucidated and the pathophysiologic processes, clinical variation in phenotype, and the role of genetic modifiers have been recognized. ⋯ The only recognized specific therapeutic strategy is regular infusions of the purified plasma protein, and evidence confirms its efficacy in protecting the lung (at least partially). Early detection and modification of lifestyle remains crucial to the management of AAT deficiency.
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Clinics in chest medicine · Dec 2013
ReviewStandard nonspecific therapies in the management of pulmonary arterial hypertension.
Recent advances in pulmonary arterial hypertension (PAH) research have created a new era of PAH-specific therapies. Although these therapeutics have revolutionized PAH therapy, their innovation was predated by supportive but nonspecific medical therapies adapted from their use in more common cardiopulmonary diseases. ⋯ Expert opinion continues to support the use of adjunct therapies based on current pathologic understandings of PAH combined with some evidence extrapolated from small studies. This article discusses why these therapies continue to play an important role in the treatment of patients with PAH.
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Clinics in chest medicine · Dec 2013
Review Historical ArticleEpidemiology of pulmonary arterial hypertension.
Changes in the epidemiology of pulmonary arterial hypertension (PAH) have resulted from changes in classification schemes and an increased emphasis on diagnosis because of the availability of effective therapies. The terms primary pulmonary hypertension and secondary pulmonary hypertension are considered inappropriate, confusing, and should not be used. Recent registries of patients with PAH have provided improved data regarding prognosis in the era of advanced therapies.