Titlebook: Orphan Lung Diseases; A Clinical Guide to Vincent Cottin,Luca Richeldi,Francis X. McCormack Textbook 2023Latest edition The Editor(s) (if

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Granulomatosis with Polyangiitisneys (glomerulonephritis). A biopsy of the affected organ can support the diagnosis, as can detection in the serum of ANCAs with a proteinase 3 specificity (PR3-ANCAs) by enzyme-linked immunosorbent assay (ELISA). Important therapeutic advances have been made in the past three decades, and several m

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Portopulmonary Hypertension and Hepatopulmonary Syndromederlying chronic liver disease, so it is imperative that clinicians maintain a high index of suspicion for these pulmonary vascular disorders when confronted with a dyspneic patient with hepatic disease (Table 11.1, Fig. 11.1). Both conditions significantly increase the morbidity and mortality of ch

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Systemic Sclerosis and the Lunguppressive therapy, based on two controlled trials of cyclophosphamide and a comparison between cyclophosphamide and mycophenolate mofetil. Based on these data, the current consensus is that cyclophosphamide therapy is appropriate when SSc-ILD is extensive or overtly progressive with mycophenolate a

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Vincent Cottin by buildings or other structures, or by vegetation, and the boundaries affect the attractiveness and the usefulness of the space, the extent to which it can be experienced, just as much as its dimensions, sub-divisions and content. Forming and defining units that can be perceived spatially in this