Current opinion in pulmonary medicine
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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.
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Although routine use of Doppler echocardiography has led to an increased recognition of pulmonary hypertension, the role of Doppler echocardiography has largely remained as a screening tool with the primary emphasis on the presence or absence of an increased Doppler-estimated pulmonary artery systolic pressure (PASP). However, the utility of Doppler echocardiography in the workup of pulmonary hypertension extends far beyond that of a screening tool, with the integration of relevant Doppler echocardiography parameters providing a wealth of hemodynamic insight into not only if a patient has pulmonary hypertension, but why they have pulmonary hypertension. This review summarizes some of the recent advances in the use of Doppler echocardiography in evaluating the pathophysiology of pulmonary hypertension. ⋯ This review highlights the importance of integrating two-dimensional and Doppler parameters in order to inform the clinician as to the hemodynamic cause of pulmonary hypertension, thus enhancing the diagnostic accuracy of Doppler echocardiography, rapidly identifying those with PHPVD and right heart dysfunction and assisting in triage of patients to further invasive hemodynamic assessment.