Baughman / Carbone / Bottino Pulmonary Arterial Hypertension and Interstitial Lung Diseases

A Clinical Guide
2009
ISBN: 978-1-60327-074-8
Verlag: Humana Press
Format: PDF
Kopierschutz: Wasserzeichen (»Systemvoraussetzungen)

E-Book, Englisch, 248 Seiten, eBook

Interstitial lung disease (ILD) is a broad category of lung diseases that includes more than 150 disorders characterized by scarring or fibrosis of the lungs. In Pulmonary Arterial Hypertension and Interstitial Lung Disease: A Clinical Guide, renowned experts provide a state-of-the-art overview of the problems seen by physicians in the clinical management of ILDs. Divided into two sections, the first part provides and update on general issues and introduces both interstitial lung disease and associated pulmonary hypertension. A detailed analysis of the pathology of the various interstitial lung diseases is also provided. The second part addresses specific categories of disease. Bronchiolitis, hypersensitivity pneumonitis, and other conditions are covered, and the use of inspiratory and expiratory high resolution CT scan is discussed as well. This important new text is an invaluable resource for the practicing physician who must be aware of the broad and troubling manifestations of interstitial lung disease.

Zielgruppe

Professional/practitioner

Autoren/Hrsg.

Bottino, Giovanni

Baughman, Robert P.

Carbone, Roberto G.

Inhaltsverzeichnis

General Principles.- Interstitial Lung Disease: Introduction.- Pulmonary Hypertension in Interstitial Lung Disease.- Idiopathic Pulmonary Fibrosis and Associated Pulmonary Hypertension: Genetics, Pathobiology, Diagnosis, and Management.- Lung Pathology.- Primary Pulmonary Hypertension.- Specific Lung Disorders.- Bronchiolar Disorders.- Hypersensitivity Pneumonitis.- Connective Tissue Disease and Vasculitis-Associated Interstitial Lung Disease.- Pulmonary Hypertension in Idiopathic Pulmonary Fibrosis.- Occupational Interstitial Lung Disease Update.- Sarcoidosis.

Leseproben

Severe or Very Severe PH (S. 20-21)

In patients with severe or very severe PH, a mean PAP of 30–45 mmHg at rest correlates with decompesation and lung diseases. A mean PAP of 40–70 mmHg at rest correlates with decompensation and chronic pulmonary embolism. Finally, a mean PAP of between 65 and 120 mmHg at rest correlates with Eisenmenger’s Syndrome. When patients are at rest, there is reduced cardiac output.

Right ventricular adaptation is one of the most important prognostic factors in pulmonary hypertension. Initially, right ventricular hypertrophy compensates for the increased after load. The chamber decompensates when the pressure increases more rapidly than the adaptation mechanism. A moderate increase of pulmonary resistances and a slow progression of disease are good conditions for the development of right ventricular adaptation. On the whole, the factors that influence right ventricular remodeling are largely unknown. There is evidence that the local ACE system of the heart might be activated during PH [38].

Pulmonary arteries develop structural changes in parallel with the chronic vasoconstriction. In situ thrombosis develops, accelerating the remodeling of small cells. As a consequence, there is a reduction of the vascular cross-sectional area and a loss of compliance. Structural changes in pulmonary arteries differ between small and large vessels: the latter assume aneurismal forms, whereas the lumen of the former is progressively diminished as the result of the remodeling process.

The term remodelling is used to describe changes in small diameter pulmonary vessels [39,40]. Normal-appearing pulmonary arterial vessels have a continuous media down to a diameter of approximately 80 µm. Distally to this point, vessels are partially muscular and contain so-called intermediate cells, which range in their properties somewhere between pericytes and smooth muscle cells. In the most peripheral vascular areas, there are only few pericytes, and the remaining cell population consists of endothelial cells. The process of vessel remodeling results in (1) intimal fibrosis, (2) hypertrophy of the media, and (3) the formation of myocytes ex novo.

The smooth muscles of the media grow distally, initially in a longitudinal direction, providing a complete muscular layer to smaller pulmonary arterial vessels having a diameter down to 15 µm. The smooth muscle cells produce extracellular matrix proteins such as glycoprotein and elastin. At same time, there are changes in other vascular layers, such as the adventitia and intima. The adventitia shows a proliferation of fibroblasts with migration of these cells in the vessel wall.

The intimal cells responsible for fibrosis are poorly characterised and are described as my fibroblasts secreting extracellular matrix proteins such as collagen type I and III. As for the intima, changes involve the glycocalyx layer of the endothelial cells, with a reduction of heparan sulphate, which can trigger smooth muscle proliferation. It seems that endothelial cell mediators shift from an anticoagulatory to protrombotic profile. The internal elastic lamina is fragmented and allows cells to migrate into the intimal layer.