Current opinion in pulmonary medicine
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To summarize current knowledge of health-related quality of life (HRQL) assessment in idiopathic pulmonary fibrosis (IPF), with an emphasis on reviewing available data on HRQL instruments, operationalizing HRQL assessment in IPF research and interventions that show promise for improving HRQL in patients with IPF. ⋯ IPF is a devastating disease. Because there is no cure, an improved understanding of how this disease affects patients' lives is needed. Rigorously developed and carefully administered instruments are needed to assess those effects and to measure the impact of interventions aimed at improving the lives of patients with IPF.
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Pulmonary hypertension is a serious complication of sarcoidosis. This review discusses clinical characteristics of patients with sarcoid-associated pulmonary hypertension (SAPH) and pitfalls in the diagnosis, and highlights potential therapies. ⋯ Patients who complain of persistent dyspnea should be screened for the presence of pulmonary hypertension. The prognosis of SAPH is poor and it is prudent to consider referral of these patients for lung transplantation. In some patients with SAPH, treatment with anti-inflammatory agents and pulmonary vasodilators can lower pulmonary arterial pressures, improve dyspnea and functionality, and enhance overall quality of life.
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Idiopathic pulmonary fibrosis (IPF) is a progressive, invariably fatal condition with a median survival from diagnosis of only 3 years. Despite improvements in disease understanding, challenges remain in establishing a diagnosis and predicting prognosis in individual patients. Furthermore, limited understanding of the key pathogenetic mechanisms driving disease is hampering development of new therapies. This review outlines progress that has been made in applying systems biology to IPF and the insights into disease pathogenesis, diagnosis and monitoring that this research is providing. ⋯ Use of molecular research techniques in large populations of well-phenotyped patients is leading to major advances in understanding of IPF. As new treatments for IPF emerge, it is to be hoped that careful application of these findings will enable the targeting of therapy to individuals based on the predominant mechanisms driving progression of their disease.
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To summarize and highlight recent advances in the field of the diagnosis of pulmonary sarcoidosis. ⋯ The finding of noncaseating granulomas remains crucial to the diagnosis of sarcoidosis. The methods described here markedly enhance the diagnostic yield of tissue sampling along with low risks of complications.
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The interstitial lung diseases (ILDs) frequently result in considerable disability and reduced survival in affected patients. Unfortunately, they are often poorly responsive to available therapies. Comorbidities, both pulmonary and nonpulmonary, frequently accompany ILDs and contribute to adverse outcomes. ⋯ The identification and treatment of comorbidities may improve morbidity and potentially impact mortality in patients with ILD. A high index of suspicion and an awareness of the spectrum of comorbidities are important in optimizing outcomes in this group of patients.