Path: Restrictive Lung Disease Flashcards
What does a honeycomb lung appearance signify?
End Stage lung disease
Histologic pattern seen in Idiopathic Pulmonary Fibrosis (IPF).
Usual Interstitial Penumonia
What cell types undergo hyperplasia in IPF?
Type II pneumocytes
Fibroblasts
Tx for IPF
Lung Transplant
How do IPF and Nonspecific Interstitial Pneumonia appear different on biopsybased on fibrosing pattern?
IPF has temporal heterogeneity
-you will see both early and late signs of collagen and fibrosis deposition along with areas of normal lung tissue in the same biopsy.
Tx for nonspecific Interstitial Pneumonia
Steroids
Tx for Cryptogenic Organizing Pneumonia (Bronchiolitis obliterans organizing pneumonia: or BOOP)
Oral Steroids
Characteristic microscopic finding indicating BOOP.
Masson Bodies: polypoid plugs of loose organizing connective tissue
General cause of Pneumoconioses
Inhalation of mineral dust
What 2 conditions can result from chronic simple coal workers pneumoconiosis?
- Centrilobular Emphysema
- if dilation of adjacent alveoli occurs - Progressive Massive Fibrosis
Does coal workers pneumoconiosis (CWP) increase risk of lung cancer?
No
Who is at risk of developing Silicosis (type of pneumoconiosis)?
Foundry workers
Sandblasters
Stone Cutters
What is the pathogenesis of Silicosis?
Inhalation of the particles causes macrophages to release mediators
-TNF, IL, free radicals, cytokines, stimulate fibroblasts
How does silicosis progression differ from CWP?
Progression occurs even if the exposure is removed in Silicosis.
What is viewed on microscopy in lung tissue with silicosis?
Birefringent needle like crystals piercing the tissue under polarized light
Name the 2 types of asbestos that can cause asbestosis.
- Serpentine: curly, flexible
2. Amphiboles: straight, stiff
Why does smoking increase risk of asbestosis?
The silica crystals (asbestos) that cause asbestosis are removed by the mucociliary elevator which does not function in smokers
What gross finding can be seen on lung tissue in a patient that had asbestosis.
Pleural Plaques
What is Caplan Syndrome?
Rheumatoid Arthritis patients that are exposed to mining dusts that develop fibrosis in the lungs. Granulomatous inflammation occurs.
How is Sarcoidosis diagnosed?
Dx of exclusion. Non-Caseating granulomas are present and all other diseases are ruled out.
Treatment for sarcoidosis.
Steroids
-it’s thought to be an autoimmune rxn or a malfunction of the immune system
What are Schaumann Bodies?
Name for the non-Caseating granulomas in sarcoidosis.
same for asteroid bodies, the names just describe the pattern seen in microscopy
What is the cause for Hypersensitivity Pneumonitis?
Intense, prolonged exposure to inhaled organic dusts and occupational antigens, fungi, animal proteins etc.
Cause of Tropical Pulmonary Eosinophilia.
Wuchereria bancrofti
Best Tx for Desquamative Interstitial Pneumonia.
Stop Smoking
-disease process reverses
Best Tx for Respiratory Bronchiolitis-Associated Interstitial Lung Disease.
Stop Smoking
-disease process reverses
Pathogenesis of Acquired Alveolar Proteinosis
Autoimmune
-Anti-granulocyte-macrophage colony stimulating factor antibody
Tx for congenital Alveolar Proteinosis.
Lung transplant
-gene mutation doesn’t allow proper lung development
