Seminars in respiratory and critical care medicine
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Severe asthma affects 5 to 10% of the asthma population but consumes a disproportionate amount of the global asthma budget (~50%) due to unscheduled health care utilization in primary care, hospitalizations due to severe exacerbations, and the costs of pharmacotherapy. A key challenge in managing severe asthma is to identify appropriate groups of patients that will respond best to existing and evolving therapies. Recent advances in our understanding of how to classify severe asthma using multivariate taxonomical approaches have provided a unique model of a stratified medicines approach. ⋯ A similar paradigm can be applied to other domains in severe asthma such as airway hyperresponsiveness, which may now be treated with the first mechanical therapy in airways disease (bronchial thermoplasty). At the same time it is important for the clinician to recognize and treat comorbid factors that make asthma difficult to manage such as poor adherence to medication, rhinosinusitis, and psychological comorbidity. Therefore it is of vital importance to develop a multidisciplinary approach to the management of severe asthma that is best applied within specialist centers with experience and wider access to national and international severe asthma networks.
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Semin Respir Crit Care Med · Dec 2012
Aspirin-exacerbated respiratory disease: update on pathogenesis and desensitization.
Aspirin-exacerbated respiratory disease (AERD) is a unique syndrome of airway inflammation that frequently occurs in patients with nasal polyposis, chronic sinusitis, and asthma. These patients tend to have progressive and recalcitrant sinus disease requiring frequent surgical intervention and in many cases systemic corticosteroids. Much about the pathogenesis of AERD remains unclear, but environmental factors likely play a prominent role in its development. ⋯ Given the prevalence and usefulness of aspirin and NSAID therapy in primary care clinics, an accurate diagnosis should be made in all patients. Desensitization is an effective treatment option for many patients. Recent advances have made this procedure considerably safer and outpatient aspirin desensitization is now the standard of care.
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Asthma is a heterogeneous disease, with wide variability in pathology, natural history, and response to therapy. Historically, treatment of asthma relied almost exclusively on clinical judgment and pulmonary function tests (spirometry or peak flows), despite the limitations of both. Physiological tests are one step removed from what the clinician needs to know, namely, the underlying activity and whether it is amenable to additional or alternative treatment. ⋯ Financial constraints applied by health providers and funding agencies have limited the use of induced sputum analysis and exhaled nitric oxide to date. However, evaluation of candidate biomarkers has provided important insights in clinical practice and in research settings. At the very least, existing techniques should have a regular place in severe asthma clinics, if not more widely, where heterogeneity is the norm and not all asthma is what it seems.
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Semin Respir Crit Care Med · Oct 2012
ReviewLymphangioleiomyomatosis: new concepts in pathogenesis, diagnosis, and treatment.
Lymphangioleiomyomatosis (LAM) is a slowly progressive lung disease that is associated with mutations in tuberous sclerosis complex genes, infiltration of the pulmonary parenchyma and lymphatics with neoplastic smooth muscle cells, extensive tissue remodeling and architectural distortion of the lung, and tumors of the chest and abdomen, including lymphangiomyomas and angiomyolipomas. LAM occurs in women in the general population and in patients of both genders with tuberous sclerosis. ⋯ The molecular basis of LAM has been extensively characterized over the past decade, resulting in the development of a targeted therapy. This article reviews emerging approaches to the diagnosis and treatment of LAM.