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Lecture 4

Pulmonary IV - Restrictive Lung Disease

QuestionAnswer
Symptoms of Chronic Interstitial Pneumonias (1) progressive dyspnea (2) tachypnea (3) non-productive cough for more than 6 months duration. Begins with a flu-like illness in 50-70% of cases.
(T or F) Wheezing is a prominent symptom in chronic interstitial pneumonias. False
The most common idiopathic interstitial pneumonias and has the poorest prognosis. 60% of patients have elevated rheumatoid factor and anti-nuclear antibody. Usual Interstitial Pneumonia
What idiopathic interstitial pneumonias is indication for lung transplantation? Usual Interstitial Pneumonia
Pathological findings of Diffuse Interstitial Pneumonia Lesion is temporally uniform. Alveoli are filled with “desquamated” cells, consisting of densely packed macrophages associated with uniform interstitial fibrosis and chronic inflammation
What idiopathic Interstitial Pneumonia strongly associated with smoking? Diffuse Interstitial Pneumonia
Pathological findings of chronic interstitial pneumonia/nonspecific interstitial pneumonia Lesions that are temporally uniform, but patchy UIP-like changes
An idiopathic interstitial pneumonia with findings notable for more intense interstitial lymphoid infiltrates and less prominent fibrosis. Seen in AIDS, immunocompromised states, and some cases of collagen vascular disease. An association with EBV. Lymphoid interstitial pneumonia
An idiopathic interstitial pneumonia that is very rare. Pathology similar to hypersensitivity pneumonias. Specific subset associated with cobalt inhalation. Giant Cell Interstitial Pneumonia
Hisotpathological findings of Sarcoidosis (1) non-caseating granulomata (2) enlarged hilar lymph nodes (3) pulmonary interstitial infiltrates (4) BAL with very high (~10:1) of Thelper/Tsuppressor ratio
Organs involved in sarcoidosis (1) Pulmonary hilar and mediastinal lymph nodes (100%) (2) lungs (90%) (3) Liver (80%) (4) Scalene lymph nodes (74%) (5) Gastrocnemius muscle (69%) (6) Bone Marrow (30%) (7) spleen (8) joint (9) eyes (10) skin
Stage I of sarcoidosis Hilar adenopathy, usually bilateral
Stage II of sarcoidosis Hilar adenopathy and parenchymal disease
Stage III of sarcoidosis Parenchymal disease dominant
Stage IV of sarcoidosis Irreversible fibrosis – mortality
Morphologic changes in eosinophilic granuloma of the lung Stellate nodule at low power. Patchy peribronchiolar areas consisting of localized proliferation of Langerhans histiocytes cells admixed with eosinophils and lymphocytes. These areas of inflammation lead to fibrosis.
What are Langerhan histiocytes? Antigen presenting cells with characteristic cytoplasmic inclusions called Birbeck granules. Immunophenotype: S100(+), CD1(+).
(T or F) Eosinophilic granuloma disease is limited to the lung. False
Diameter of pathogenic silica particles < 3 microns
Complications of Silicosis (1) Conglomeration of nodules (2) Silicotuberculosis (3) Increased risk for bacterial infections (4) Increased risk of autoimmune diseases (5) Pulmonary alveolar proteinosis
A reaction to heavy exposure to high levels of silica over 1-3 years. The alveolar spaces are filled with surfactant apoprotein due to the inability of macrophages to degrade it. This can also occur in unexposed individuals. Pulmonary alveolar proteinosis
Pulmonary alveolar proteinosis Two or more asbestos bodies in a 5 micron histologic section of lung associated with pulmonary fibrosis
What are Chrysotiles? Curved asbestos fibers. Less pathogenic than the straight fibers.
What are Amphiboles? Straight asbestos fibers. Associated with the development of malignant mesothelioma.
Lung Pathology of Asbestosis Interstitial fibrosis, carcinoma. The lower lobes are most commonly affected.
Pleural Pathology of Asbestosis Plaques, pleuritis with effusion, fibrosis, and malignant mesothelioma
Prognosis of Mesothelioma 90% patients die within 1-3 years. Median survivial is 8-15 months
(T or F) 95% of females with mesothelioma have no known asbestos exposure True
Lung disease caused by inhalation of coal dust, which contains fibrogenic particulates like silica. Occurs in patients with long-term histories of coal mining. There two subtypes: simple, complicated. Coal worker’s pneumoconiosis
A variant of Coal worker's penumoconiosis characterized by coal macules form along the lymphatic distribution around the bronchioles. Associated with slight fibrosis. Usually less than 1 cm in size. Effects are not clinically significant. Simple type of Coal worker’s pneumoconiosis
A variant of Coal worker’s pneumoconiosis characterized by nodules over 2 cm in size associated with functional abnormalities. Nodules over 2 cm in size associated with functional abnormalities.
What collagen vascular disease are associated with Caplan’s nodules in the lung ? Rheumatoid Arthritis
Created by: UVAPATH2
 

 



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