Interstitial Lung Disease Flashcards
What is lymphocytic interstitial pneumonia (LIP)?
It is the most common interstitial lung disease in children. Pathogenesis is unknown, but it appears to have two general causes: an exaggerated response to inhaled antigens in a child with another autoimmune dysfunction, or the result of a primary infection with a virus such as HIV or EBV.
Which interstitial lung disease is considered to be an AIDS-defining illness in children?
Lymphocytic interstitial pneumonia (LIP). It is most commonly seen in children with perinatally acquired HIV, who usually develop LIP at 2-3 years of age.
What is sarcoidosis?
A chronic multisystem disorder of unknown origin that affects young adults and, rarely, children. It is characterized by noncaseating epithelioid cell granulomas, which have a predilection for thoracic lymph nodes and lung tissue.
What does the CXR characteristically show in sarcoidosis?
Usually, there is bilateral hilar and/or mediastinal adenopathy +/- reticulonodular or alveolar infiltrates. Interestingly, the hilar adenopathy disappears as the disease progresses.
What does the biopsy of an affected bronchial wall in sarcoidosis show?
Noncaseating granulomas
How does the presence of erythema nodosum affect the prognosis in patients with sarcoidosis?
Erythema nodosum is an associated skin lesion that denotes a good prognosis in patients with sarcoidosis.
What ocular complication should one screen for in all patients with sarcoidosis?
Perform a slit-lamp exam on all patients with sarcoidosis to look for asymptomatic uveitis, which can lead to blindness.
List six indications for the use of systemic corticosteroids in patients with sarcoidosis.
Involvement of the eyes, heart conduction abnormalities, CNS involvement, severe pulmonary symptoms, severe skin lesions, and persistent hypercalcemia.
List three laboratory abnormalities commonly seen in patients with sarcoidosis.
Hypercalcemia, hypercalciuria, and hypergammaglobulinemia.
What is pulmonary alveolar proteinosis?
In children, most cases result from a genetic or acquired defect in surfactant metabolism, leading to a buildup of proteinaceous material in the airways.
In a patient with pulmonary alveolar proteinosis, hypoxemia is often due to what?
Often, patients are hypoxemic from a large R > L shunt because gas exchange is impaired secondary to clogged alveoli.
How do interstitial lung diseases injure the lungs, and how does it affect the lung function?
Interstitial lung diseases injure the alveoli and surrounding tissues, resulting in interference of gas exchange. This injury is followed by wound healing that results in fibrosis. Many types of ILD also involve inflammation. ILD causes a restrictive pattern of lung physiology.
