Restrictive Disease Flashcards
What process is disrupted in restrictive diseases? What parameters are affected and what usually causes these disorders?
Characterized by restrictive filling of the lung. Low TLC, low FEV1 and much lower FVC. FEV1:FVC ratio is INCREASED. Most commonly due to interstitial diseases of the lung, may also arise with chest wall abnormalities due to massive obesity.
Patient presents with dyspnea and cough. Lung CT shows fibrosis in the subpleural patches. What disease causes this? What happens at the end stage of this disease? How is it treated?
Idiopathic pulmonary fibrosis. Initially results in diffuse fibrosis with end-stage ‘honeycomb’ lung. Treatment is lung transplantation.
What are possible causes of idiopathic pulmonary fibrosis? What induces fibrosis? What are some secondary causes?
Cyclical lung injury. TGF-beta from injured pneumocytes induces fibrosis. Secondary causes of interstitial fibrosis such as drugs (bleomycin and amiodarone) and radiation therapy must be excluded.
What is the pathogenesis of pneumoconioses?
Interstitial fibrosis due to occupational exposure. Requires chronic exposure to small particles that are fibrogenic. Alveolar macrophages engulf foreign particles and induce fibrosis.
What is the pathogenesis of pneumoconioses?
Interstitial fibrosis due to occupational exposure. Requires chronic exposure to small particles that are fibrogenic. Alveolar macrophages engulf foreign particles and induce fibrosis.
A patient presents with dyspnea. Lab values are notable for elevated serum ACE, Hypercalemia. He also has uveitis. What does this patient have and how is she treated? Which population does this disease target?
Sarcoidosis which is asystemic disease characterized by NON caseating granulomas in multiple organs. Classically seen in African American females. Treatment is steroids. Often resolves without treatment.
What is the likely etiology of sarcoidosis? What tissues are commonly involved in sarcoidosis?
Due to CD4+ helper T cell response. Uvea, Skin, salivary and lacrimal glands. Mimics Sjogren syndrome.
Where do you find granulomas in sarcoidosis? What do they lead to? What is seen on histology?
Most commonly involve the hilar lymph nodes and lung leading to restrictive lung disease. Characteristic stellate inclusions (‘asteroid bodies’) are often seen within giant cells of the granulomas.
Where do you find granulomas in sarcoidosis? What do they lead to? What is seen on histology?
Most commonly involve the hilar lymph nodes and lung leading to restrictive lung disease. Characteristic stellate inclusions (‘asteroid bodies’) are often seen within giant cells of the granulomas.
What is hypersensitivity pneumonitis? How does it present? What causes interstitial fibrosis?
Granulomatous reaction to inhaled organic antigens (pigeon breeder’s lung). Presents with fever, cough and dyspnea hours after exposure. Resolves with removal of the exposure. Chronic exposure.
What is the exposure, pathologic findings and increased risk of Coal Worker’s Pneumoconiosis?
Carbon dust. Massive exposure leads to diffuse fibrosis (‘black lung’) associated with rheumatoid arthritis (Caplan syndrome). Mild exposure to carbon results in anthracosis (collection of carbon-laden macrophages). Not clinically significant.
What is the exposure, pathologic findings and increased risk of Silicosis?
Silica seen in sandblasters and silica miners. Fibrotic nodules in UPPER lobes of the lung. Increased risk for TB; silica impairs phagolysosome formation by marcophages.
What is the exposure, pathologic findings and increased risk of Berylliosis?
Beryllium seen in beryllium miners and workers in the aerospace industry. Noncaseating granulomas in the lung, hilar lymph nodes, and systemic organs. Increased risk for lung cancer.
What is the exposure, pathologic findings and increased risk of Asbestosis?
Asbestos fibers seen in construction workers, plumbers and shipyard workers. Fibrosis of lung and pleura with increased risk for lung carcinoma and mesothelioma. Lung carcinoma is more common than mesothelioma in exposed individuals. Lesions may contain long, golden brown fibers with associated iron (asbestos bodies).
