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MHD > Restrictive Lung Disease > Flashcards

Restrictive Lung Disease Flashcards

1
Q

What is the primary issue in restrictive lung diseases?

A

Decreased TLC
Normal FEV1/FVC ratio

Could be due to fibrosis of the lung or a chest wall disorder preventing expansion

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2
Q

What is seen on histology of restrictive lung diseases that is not seen in obstructive diseases?

A

Fibrosis

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3
Q

Interstitial Lung Disease

Symptoms

A

Reduced compliance of lungs
Dyspnea
End inspiratory crackles
Hypoxia

Could eventually cause pulmonary HTN and cor pulmonale

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4
Q

Interstitial Lung Disease

What is the end stage pathology appearance?

A

Honeycomb lung

Fibrosis causing an appearance of cystic spaces (but not like emphysema)

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5
Q

Describe the pathogenesis of Idiopathic Pulmonary Fibrosis

A

Repeated epithelial activation/injury by some unidentified agent causes release of TGF-B. Leads to abnormal epithelial repair.

Eventually, this causes fibroblastic proliferation and collagen deposition in the lungs, leading to pulmonary fibrosis

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6
Q

What is the morphology of Idiopathic Pulmonary Fibrosis?

A

Cobblestone lung
(Scarring of the pleural surface)

Patchy interstitial fibrosis
Non-uniform, varying in intensity and age

Get Honeycomb fibrosis and cystic spaces lined by bronchiolar epithelium

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7
Q

Idiopathic Pulmonary Fibrosis

Where do the cystically dilated airspaces tend to appear?

A

Lower lobe and subpleural areas

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8
Q

Idiopathic Pulmonary Fibrosis

What stain might you use to identify collagen deposition?

A

Trichrome stain

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9
Q

Idiopathic Pulmonary Fibrosis

Symptoms

A

Dyspnea on exertion
Dry cough
Velcro crackles
Deterioration of lung

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10
Q

Idiopathic Pulmonary Fibrosis

What is the only definitive treatment?

A

Lung transplant

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11
Q

Idiopathic Pulmonary Fibrosis

Prognosis

A

Mean survival time is less than 3 years

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12
Q

What is Pneumoconioses?

A

Accumulation of dust in the lungs and tissue reaction to its presence

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13
Q

What is the most prevalent occupational disease?

A

Silicosis

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14
Q

What is the size of the most dangerous particles for Pneumoconioses? Why?

A

1-5 microns
They can reach the terminal small airways, air sacs, and settle in the linings, but are too big to be ingested by alveolar macrophages

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15
Q

Describe the basic pathogenesis of Pneumoconioses

A

Macrophages endocytose trapped particles in the small airways. Reactive particles tigger release of inflammatory mediators from macrophages, leading to fibrogenesis and collagen deposition

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16
Q

Anthracosis

Who is most likely to get it?

A

Smokers
Urban dwellers
Coal miners

17
Q

Describe the pathogenesis of Anthracosis

A

Inhaled carbon pigment is engulfed by alveolar and interstitial macrophages

Deposits in the lungs and lymph nodes (get black nodes on lungs and the lymph nodes are black)

18
Q

Anthracosis

Prognosis

A

No sequelae associated with Anthracosis

If it develops over time, it may cause Simple Coal Workers Pneumoconiosis

19
Q

Simple Coal Workers Pneumoconiosis

What is the appearance?

A

Accumulation of dust-laden macrophages with some patchy fibrosis

20
Q

Progressive Massive Fibrosis

What is the appearance?

A

Coal nodules coalesce in the lungs

Get fibrotic heavy black tissue masses

21
Q

Caplan Syndrome

What are the two diseases associated with it? What happens?

A

Form of Coal Worker Pneumoconiosis associated with Rheumatoid Arthritis

Fibrotic lesions form and they get fibrotic nodules with a tendency to cavitate

22
Q

Silicosis

What is the characteristic appearance of the nodules?

A

Whorled hyalinized collagen (whirls of concentric collagen appear)

23
Q

Silicosis

Where may the fibrotic lesions appear?

A

They tend to affect the upper lobe

The fibrotic lesions may spread to hilar lymph nodes. The lymph node capsule tends to get calcified (“eggshell” calcification)

24
Q

Silicosis

What sequelae does it cause?

A

Increased susceptibility to tuberculosis (suppressed cell mediated immunity)

Dyspnea
Pulm HTN
Cor pulmonale

25
Q

Asbestos is a strong carcinogen. What cancers may it cause?

A

Bronchogenic carcinoma
Laryngeal carcinoma
Malignant Meseltheliomas

26
Q

Asbestos

What may been seen characteristically on H&E?

A 
Asbestos body (ferrogenous body)
Beaded structure with a knobby head (Intracellular iron deposits on the asbestos body coats it)
27
Q

How much higher is risk for lung cancer with asbestos exposure? With both asbestos and tobacco?

A

5x with asbestos alone

55x with both

28
Q

Sarcoidosis

What is seen on histology?

A

Non-caseating granulomas (with lymphocytes, epithelioid histiocytes, and giant cells)

You may see the granulomas in the bronchiole epithelium, alveolar airspace, lymph nodes

29
Q

Sarcoidosis

What is seen on CXR?

A

Bilateral hilar lymphadenopathy

30
Q

Describe the pathogenesis of Sarcoidosis

A

Some antigen in a patient with genetic susceptibility results in an abnormally large IFN-y and TNF-a response

Results in formation of granulomas, which may resolve or progress to chronicity and fibrosis

31
Q

Sarcoidosis

What is a Schaumann body?

A

Concentration of calcium and proteins within giant cells

32
Q

Sarcoidosis

What is an asteroid body?

A

Stellate inclusions in the granuloma

33
Q

Sarcoidosis

What patients are more likely to get it? Where?

A

Young adults, mostly in upper lobe

Young asymptomatic African American woman is a common boards prompt

34
Q

Sarcoidosis

What tests are done to confirm the diagnosis?

A

It is a diagnosis of exclusion (test for infection and other granuloma causes first)

Do a transbronchial biopsy with 4 samples submitted

35
Q

Sarcoidosis

Symptoms

A 
Often asymptomatic
SOB
Dry cough
Fever
Fatigue
Night sweats
Weight loss
Anorexia
Diffuse intersititial fibrosis
36
Q

What are some systemic involvements of Sarcoidosis?

A

Eye- Uveitis

Skin - Erythema nodosum

Heart- Restrictive cardiomyopathy

Bell’s Palsy- CN VII

37
Q

Sarcoidosis

List some lab/diagnostic abnormalities

A

Elevated serum ACE
Hypercalcemia
Anergy to common skin test antigens

38
Q

Why does Sarcoidosis cause hypercalcemia?

A

Granulomas can make the hydroxylase enzyme to make 1,25 (OH)2 Vit D3, which increases Ca2+ absorption

MHD (57 decks)

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