A Case of Diffuse Parenchymal Lung Disease (Nonspecific Interstitial Pneumonia) with Cor Pulmonale

Case Presentation: Diffuse parenchymal lung disease (Nonspecific Interstitial Pneumonia) with Cor Pulmonale

Diffuse parenchymal lung disease (DPLD) refers to a large and heterogeneous group of pulmonary diseases that are characterized by replacement of extensive portions of the normal lung parenchyma with inflammation and/or fibrosis.

Nonspecific interstitial pneumonia (NSIP) is one class of idiopathic interstitial pneumonia (IIP). NSIP is chronic interstitial pneumonia with the homogeneous appearance of interstitial fibrosis and inflammation. It is nonspecific as it lacks the histopathological features of the other subtypes of the IIP. NSIP typically has bilateral lung involvement and may have a predisposition for the lower lobes.

Cor pulmonale is a disease of the heart characterised by dilatation of the right ventricle and paradoxical movement of the interventricular septum. The diagnosis depends on echocardiography even if pulmonary artery catheterisation suggests it. It is secondary to pulmonary disease or a disorder of the pulmonary circulation. These two mechanisms, which are often connected, involve pulmonary hypertension as the origin of a systolic and diastolic overload of the right ventricle, which then leads to the alterations of its structure and performance. Acute cor pulmonale is usually secondary to an acute respiratory distress syndrome or to a pulmonary embolism but it can also be seen in primary lactic acidosis, a vaso-occlusive crisis in a patient with sickle cell anaemia, severe acute asthma, and entry of air or injected crushed tablets into the circulation. Chronic cor pulmonale is the terminal stage of pulmonary hypertension. Clinically these patients are dyspnoeic with signs of chronic right heart failure. They should have an echocardiogram confirming the cardiac involvement. Certain precipitating factors, such as infection of any origin, have been reported, leading to acute on chronic cor pulmonale that has a particularly high mortality.

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