Pulmonary Pathology III Flashcards by Kevin N

What is the general feature of restrictive lung disease?

Decreased total lung capacity

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What are the two categories of restrictive lung disease?

Interstitial lung disease

- Chest wall disease

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What are the characteristics of interstitial lung disease?

Group of disorders which result in inflammation and fibrosis of alveolar walls (septa) with similar features:
-Dyspnea
-Tachypnea
-Decreased lung volume
and compliance
-Diffuse infiltration by small nodules
-"Honeycomb lung" at the end stage

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What are the categories of interstitial lung disease?

-􏰀 Fibrosing
􏰀- Granulomatous
􏰀- Eosinophilic
􏰀- Smoking related

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What is Idiopathic Pulmonary Fibrosis AKA?

Usual interstitial pneumonia

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What is the pathogenesis of IPF?

Repeated cycles of epithelial activation/injury by some unidentified agent lead to abnormal epithelial repair and fibroblastic proliferation resulting in fibrosis with increased collagen from wound healing

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What are the gross findings in IPF?

 Scarring of pleural surfaces - cobble stoned
 Fibrosis of lung parenchyma which appear as firm, rubbery, white areas
 Findings most prominent subpleural and along interlobular septa

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What is the histology of IPF?

Honeycomb fibrosis represents the destruction of alveolar architecture and formation of cystic spaces lined by pneumocytes type II and bronchial epithelium

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What will stain areas of fibrosis in IPF blue?

Trichrome Stain

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What is the typical patient of IPF?

Male between 40-70 years

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What is the prognosis of IPF and definitive treatment for it?

 Definitive treatment is lung transplant

 Mean survival is 3 years after diagnosis

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Pneumoconiosis

The accumulation of dust in the lungs and the tissue reaction to its presence

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Agent in Siderosis

Iron - welding

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Agent in Anthracosis

Coal - mining

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Agent in Silicosis

Silica - sand blasting

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Agent in Asbestosis

Asbestos - ship building

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What size particles are the most dangerous when inhaled and why?

1-5 􏰃m particles most dangerous - they can reach terminal small airways, air sacs, settle in linings

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Pathogenesis of Pneumoconiosis

Macrophages endocytose and trap particles. Reactive particles trigger release of inflammatory mediators from macrophages which initiate fibroblast proliferation and activation. End result – collagen deposition and fibrosis.

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What are the 3 states of Coal Worker’s Pneumoconiosis?

􏰀Anthracosis
Simple coal worker’s pneumoconiosis 􏰀
Progressive massive fibrosis

What is the pathogenesis of anthracosis?

Inhaled carbon pigment is engulfed by alveolar/interstitial macrophages. Accumulates in connective tissue, pleural lymphatics, lymph nodes - no sequelae

What is the patient population of anthracosis?

Seen in urban dwellers, tobacco smokers and coal miners

What is seen with simple coal worker’s pneumoconiosis?

􏰀Coal macules, nodules with accumulations of dust-laden macrophages and fibrosis - not many other symptoms though

Pathogenesis of Progressive Massive Fibrosis

Results from coalescence of coal nodules leading to haphazard fibrosis, large scars (2-10cm) with dense collagen and pigment leading to: progressive dyspnea, pulmonary dysfunction, pulmonary hypertension, cor pulmonale

What is the finding in progressive massive fibrosis - anthracosis?

“Black Lung”

What is Caplan Syndrome and what is it AKA?

AKA rheumatoid pneumoconiosis Form of Coal Worker Pneumoconiosis associated with rheumatoid arthritis

What is the most prevalent occupational disease?

Silicosis

Inhalation of crystalline silica causes macrophage activation and the release of cytokines that are ultimately fibrogenic

Simple Nodular Silicosis

 Upper zones of lungs with concentric hyalinized collagen with a whorled appearance  Fibrotic lesions may be present in hilar lymph nodes

Progressive Massive Fibrosis - Silicosis

Coalescence of nodules that can lead to: progressive dyspnea, pulmonary dysfunction, pulmonary hypertension, cor pulmonale

What disease is silicosis associated with?

Silicosis is associated with increased susceptibility to tuberculosis. It is postulated that silicosis depresses cell mediated immunity and silica may inhibit ability of pulmonary macrophages to kill phagocytosed myocbacteria

What are the effects of asbestos?

- Acts as tumor initiator and promoter (mediated by reactive free radicals) - Adsorbs toxic chemicals (tobacco smoke) - Fibrinogenic effect

What are the plethora of asbestos effects?

``` 􏰀 Interstitial fibrosis (asbestosis) 􏰀 Localized fibrous plaques - pleural plaques 􏰁 Diffuse fibrosis of pleura 􏰀 Pleural effusions 􏰀 Bronchogenic carcinoma 􏰀 Malignant mesothelioma 􏰀 Largyngeal carcinoma ```

What is the interaction of asbestos with smoking?

Synergistically increased risk of bronchogenic cancer with smoking - 55x more likely chance of cancer

What is the most common manifestation of asbestos exposure?

Localized fibrous pleural plaques - well circumscribed plaques of dense collagen usually on parietal pleura and domes of diaphragm.

What cancer is highly related to asbestos but NOT smoking?

Mesothelioma

What is the pathogenesis of malignant mesothelioma?

 Asbestos fibers settle near mesothelium |  Reactive oxygen free radicals induce DNA damage

What stain will mesothelioma be positive for?

Positive staining for calretinin

Sarcoidosis

 Multisystem disease of unknown etiology |  Characterized by non-caseating granulomas

How is sarcoidosis diagnosed?

Diagnosis of exclusion because other diseases may present as non-caseating granulomas: TB, fungal infection, berylliosis

Pathogenesis of Sarcoidosis

Unknown, however evidence suggests disordered immune regulation in genetically predisposed individuals exposed to certain environmental agents - hypothesized to be CD4 T-cell driven

What are some findings with sarcoidosis?

- Uveitis - Bell’s Palsy - CN VII - Pulmonary interstitial fibrosis - Restrictive cardiomyopathy - Hypercalcemia - Asteroid and Schaumann bodies

What are Asteroid and Schaumann bodies?

- Schauman bodies – laminated concretions composed of calcium and proteins - Asteroid bodies – stellate inclusions

What is the typical patient of sarcoidosis?

Young adult - under the age of 40 African Americans, Danish and Swedish

Why is there hypercalcemia in sarcoidosis?

Granulomas autonomously convert 25-hydroxyvitamin D to 1,25-dihydroxyvitamin D which increases calcium absorption

A 66-year-old man has had increasing dyspnea for the past year. He is a smoker. He is retired from the construction business. There are some rales auscultated in both lungs on physical examination. A chest radiograph reveals bilateral diaphragmatic pleural plaques with focal calcification as well as diffuse interstitial lung disease. A sputum cytology shows no atypical cells. Pulmonary function studies reveal a low FVC and a normal FEV1/FVC ratio. These findings are most likely to suggest prior exposure to which of the following environmental agents? ``` A Cotton fibers B Silica dust C Fumes with iron particles D Asbestos crystals E Beryllium ```

D Asbestos crystals

Hypersensitivity Pneumonitis

Abnormal hypersensitivity response from spores of thermophilic bacteria, fungi, animal proteins, bacterial products - Immunologically mediated response to an extrinsic antigen that involves both immune complex and delayed type hypersensitivity reactions (Type III and IV)