Gomez - Interstitial Diseases Flashcards by Kash Kaul

What type of appearance do infiltrative X-rays have?

Reticulonodular

Ground glass

How well did you know this?

Not at all

Perfectly

What is another name for Idiopathic Pulmonary Fibrosis?

Usual interstitial pneumonia

How well did you know this?

Not at all

Perfectly

Describe Idiopathic Pulmonary Fibrosis

Repeated bouts of inflammation lead to scarring of the lung

Cause of inflammation is largely unknown

Scarring causes increased thickness of diffusion barrier, inhibiting effectiveness of gas exchange

Very patchy

How well did you know this?

Not at all

Perfectly

What does caveolin do?

Where is it found?

What is it inhibited by?

Inhibits deposition of collagen and ECM

Found in fibroblasts

TGF-β1

How well did you know this?

Not at all

Perfectly

What are the typical sx of IPF?

Dyspnea

Cough

Hypoxemia with cyanosis

Clubbing of digits

How well did you know this?

Not at all

Perfectly

What is the typical affected age group by IPF?

What is the median survival?

Typically affects middle-aged individuals

Median survival 3 yrs

How well did you know this?

Not at all

Perfectly

What is the pathological progression of IPF?

Repeated bouts of inflammation causes scarring

Healing/scarring cycle causes patcy fibrosis

Over time, more and more areas of lung become involved

End result: honeycomb lung

How well did you know this?

Not at all

Perfectly

What part of the lung does IPF affect first?

Usually affects sub-pleural region first, then progresses to involve more and more of lung

How well did you know this?

Not at all

Perfectly

Describe Nonspecific Interstitial Pneumonia

Appears to be more limited than IPF

Not a result of repeated bouts of inflammation

Better prognosis than IPF

Will see inflammatory cells in interstitium

How well did you know this?

Not at all

Perfectly

What is organizing pneumonia?

Replacement of tissue in the alveolar space by fibrous connective tissue

How well did you know this?

Not at all

Perfectly

What are Masson bodies?

Balls of fibrous connective tissue that fill alveoli in cryptogenic organizing pneumonia

How well did you know this?

Not at all

Perfectly

Describe cryptogenic organizing pneumonia

A pneumonia of unknown etiology in which fibrous connective tissue fills the alveolar spaces

No interstitial fibrosis, so no progression to honeycomb lung

How well did you know this?

Not at all

Perfectly

How are systemic conditions related to lung diseases? What are some of them?

Autoimmune diseases can have lung involvement

Rheumatoid arthritis

Scleroderma

SLE

Sjögren synrome

Polymyositis/dermatomyositis

Mixed connective tissue disorder

How well did you know this?

Not at all

Perfectly

What process is pictured here? What can it be a sign of?

Follicular bronchiolitis

Can indicate rheumatoid arthritis

How well did you know this?

Not at all

Perfectly

What is Caplan syndrome?

Combination of rheumatoid arthritis and pneumoconiosis

Pneumoconiosis is disease caused by inhalation of something

How well did you know this?

Not at all

Perfectly

What sized particles are most dangerous to lungs?

1-5 microns

How well did you know this?

Not at all

Perfectly

What factors contribute to particulates entering lung tissue?

Amount/volume

Size, shape, and buoyancy of particles

Solubility/cytotoxicity

Concomitant irritants

How well did you know this?

Not at all

Perfectly

Is a pt exposed to asbestos more likely to develop mesothelioma or carcinoma of the lung?

Carcinoma of the lung

How well did you know this?

Not at all

Perfectly

What is Farmer’s lung?

Exposure to moldy hay

What are the three stages of Coal Worker’s Pneumoconiosis (CWP)?

Asymptomatic anthracosis (coal dust inhalation)

Simple CWP

Complicated CWP

What is anthracosis?

Deposition of black pigment in lung and adjacent lymph nodes

From coal dust

Describe simple coal worker’s pneumoconiosis

Usually benign

Will see some macules and nodules that appear on X-ray

Pt has no difficulty breathing

Describe complicated coal worker’s pneumoconiosis

Symptomatic form of CWP, often associated with progressive massive fibrosis (PMF)

Extensive scarring

Can result in pulmonary HTN and cor pulmonale

What is depicted in this image?

Anthracosis

What will make coal worker's pneumonia significantly worse?

Concomitant exposure to silica

Who is at greatest risk for developing silicosis?

Sandblasters Miners Demolition crews

Describe the process of silicosis

Slowly progressive disease that takes decades to develop Slica particle taken into lungs Particle ingested by MΦ MΦ killed by particle, causing it to be released along with inflammatory mediators Particle can be taken up by other MΦ and process repeats

What does silicosis look like on X-ray?

Egg shell calcification

What conditions are related to asbestos exposure?

Pleural effusion Pleural plaques or diffuse pleural fibrosis Asbestosis Lung CA Laryngeal CA Mesothelioma

What are the two types of asbestos?

Chrysotile (more common in industry); wavy and larger particle, not as carcinogenic Amphibole (more harmful form)

What is shown in this image? Why is it brown?

Asbestos body Brown because of iron content

Do pleural plaques contain asbestos bodies?

No asbestos bodies present in pleural plaques

What do pleural plaques look like on X-ray?

Pleural plaques appear as masses Need to do multiple images or use another modality (CT or MR) to confirm presence

What is Farmer's lung caused by?

Moldy hay carrying *Micropolysporia faeni*

What causes bagassosis?

Moldy pressed cane sugar colonized by thermophilic actinomycetes

What causes humidfier lung?

Cool mist humidifiers colonized by thermophilic actinomycetes or *Aureobasidium pullulans*

What causes bird fancier's lung/pigeon breeder's lung?

Serum proteins in pigeon droppings

What is desquamative interstitial pneumonia?

Associated with smoking A bunch of alveolar MΦs eating up all the junk that's been inhaled

What are Birbeck granules?

Found within Langerhans cells Trilaminar in appearance with dilation at the end Sometimes resemble tennis racquets, depending upon plane of section

Describe Respiratory Bronchiolitis-associated Interstitial Lung DIsease (RBILD)

Commonly found in smokers MΦ infiltrates in bronchioles Can find peribronchiolar fibrosis

What markers do Langerhans cells typically express?

CD1a, CD207 are the most common Can also see S-100

What are the characteristics of sarcoidosis?

Formation of numerous non-caseating granulomas Can occur almost anywhere in the body More common in women than men More common in African Americans than Whites

What testing can be abnormal in pts with sarcoidosis? Why does this happen?

Pts can have anergy to PPD or *Candida* antigens Due to immune system working so hard to address the sarcoidosis that it will not respond to other antigens

What happens to angiotensin converting enzyme in sarcoidosis?

ACE level will be elevated in pts with sarcoidosis Also elevated in pts with leprosy

What is Löeffler's syndrome?

Transient acute pulmonary eosinophilia from any cause

What will be seen histologically in pts with pulmonary alveolar proteinosis?

Accumulation of acellular surfactant in the alveolar and bronchiolar spaces

How would you treat autoimmune pulmonary alveolar proteinosis?

With granulocyte/macrophage colony stimulating factor (GM-CSF)