Interstitial Lung Dz

Front 1

What are some physical exam findings (5) and symptoms (3) for individuals with interstitial lung dz?

Back 1

PE: rales, clubbing, pulmonary HTN, R ventric failure, cyanosis

Symptoms: dyspnea, dry cough, fatigue

Front 2

Why is pulmonary HTN a physical exam finding in interstitial lung dz? What does it result from? (3)

Back 2

Pulmonary HTN results from:
- chronic hypoxemia
- obliteration of many capillaries
- reduced distensibility of the pulmonary blood vessels

As a result of pulmonary HTN, R ventricular failure may occur

Front 3

Someone with interstitial lung dz will have an xray that shows... (what about the types of markings, lung volume, and something special with sarcoidosis, berylloisis and hypersensitivity pneumonitis)

Back 3

Lung markings: reticular (linear), nodular (dots) or reticulonodular

Lung volume: small lung volume

With sarcoidosis, hypersensitivity pneumonitis or berylliosis, we see enlarged hilar and mediastinal lymph nodes.

Front 4

True or False:
CT scan shows interstitial dz bettwer than plain chest xrays

Back 4

True

Front 5

Interstitial lung dz is usually classified as [ obstructive / restrictive ] respiratory condition.

Back 5

restrictive

exception is sarcoidosis in which obstruction is seen ALON with restriction in some patients (due to granulomatous inflammation in walls)

Front 6

Compare interstitial versus obstructive
A. pO2
B. pCO2
C. pH

Back 6

Interstitial:
A. pO2 : N (normal) or decreased
B. pCO2 : slightly decreased
C. pH : N or increased

Obstructive:
A. pO2: N or decreased
B. pCO2: N or increased
C. pH: N or decreased

Front 7

Hypoxemia in [ obstructive / restrictive ] lung dz usually worsens during exercise, reflecting worse diffusion and V/Q mismatch than at rest.

Back 7

restrictive (as with interstitial lung dz) . Obstructive dz (like asthma) usually does not exhibit worsening hypoxemia with exercise.

Front 8

Blood tests are generally NOT helpful with interstitial lung dz diagnosis EXCEPT for one, sarcoidosis, in which 80% of newly diagnosed individuals with sarcoidosis will have what finding on their routine blood test?

Back 8

elevated angiotensin converting enzyme

Front 9

This is often adequate to diagnose sarcoidosis or hypersensitivity pneumonia but can only provide supportive, not definitive evidence for the other kinds of fibrosis.
A. blood test
B. bronchoalveolar lavage scrapings
C. transbronchial biopsy
D. surgical lung biopsy

Back 9

C. transbronchial biopsy

For other non-granulomatous forms of pulmonary fibrosis, need to do surgical lung biopsy (bigger piece of tissue)

Front 10

Which respiratory granulomatous diseases are common? Uncommon?

A. Berylliosis
B. Eosinophilic granuloma
C. Hypersensitivity pneumonia
D. Lymphomatoid granulomatosis
E. Sarcoidosis
F. Wegner's granulomatosis

Back 10

Common: E. Sarcoidosis and C. Hypersensitivity pneumonia

All else, uncommon

Front 11

Which granulomatous respiratory dz is described?
- enlarged hilar and mediastinal lymph nodes
- hepatosplenomegaly (since granulomas can form anywhere)
- uveitis
- erythema nodosum
- Angiotensin Converting Enzyme (ACE) level elevated in 80% new cases
- Obstruction in pulmonary function tests in 20% of ppl
- prognosis varies: 1/3 have full remission , 1/2 have some non-life threatening permanent organ damage, 15% have serious recurrent/persistent illness, 5-10% eventually die of this
A. Berylliosis
B. Eosinophilic granuloma
C. Hypersensitivity pneumonia
D. Lymphomatoid granulomatosis
E. Sarcoidosis
F. Wegner's granulomatosis

Back 11

E. Sarcoidosis

Front 12

Which granulomatous respiratory dz is described?
- this is essentially a broad collection of conditions that essentially boil down to an allergy to inhaled organic antigens
- acute and chronic forms
- key tocontrolling dz is avoiding further exposure to the antigen
- examples are: farmer's lung, humidifier lung, maple bark stripper's lung, cheese worker's lung, sauna taker's dz

A. Berylliosis
B. Eosinophilic granuloma
C. Hypersensitivity pneumonia
D. Lymphomatoid granulomatosis
E. Sarcoidosis
F. Wegner's granulomatosis

Back 12

Hypersensitivity pneumonitis

Acute: begins 4-8 hours after exposure, sudden fever and dyspnea symptoms, hypoxemia, interstitial lung xray pattern

Chronic: insidious onset, acute episodes may not occur, looks like pulmonary fibrosis

Front 13

Hypersensitivity pneumonia is a disease category in which all conditions basically boil down to an allergy to inhaled organic antigens. Name the condition that each of the following are antigens of.
1. Aspergillus
2. Cryptostroma
3. Micropolyspora in hay
4. Thermophilic actinomyces
5. Pullularia

Cheese worker's lung
Farmer's lung
Humidifier lung
Maple bark stripper's lung
Sauna taker's dz

Back 13

1. Aspergillus - Cheese Worker's lung
2. Cryptostroma - Maple bark stripper's lung
3. Micropolyspora in hay - Farmer's lung (hay)
4. Thermophilic actinomyces - humidifier lung
5. Pullularia - Sauna taker's dz

Front 14

Inhalation of inorganic dusts is usually an occupational dz that can cause interstitial lung dz. What are some of those dusts?

Back 14

- Silica (MOST fibrogenic dust!), sandblasters
- asbestos
- coal dust

usually need years of daily exposure but for silica, several weeks of intense exposure can be fatal

Front 15

What other diseases besides the granulomatous ones and those caused by inhalation of inorganic dusts also have pulmonary fibrosis as a feature? (3)

Back 15

20% of RA patients
40% of scleroderma
5% of patients with polymyositis, dermatomyositis and/or SLE

Front 16

Idiopathic pulmonary fibrosis is classified based on examination of surgical biopsy samples. Which is described below?

This shows fibrosis of the intrestititum along with infilration with mostly PMNs and macrophages. Lesions are temporally heterogeneous. Average survival is 3 yrs from diagnosis. There is no effective treatment and patients are advised to seek lung transplantations.

A. Bronchiolitis oblieterans / organizing pneumonia (BOOP)
B. Desquamative interstitial pneumonia (DIP)
C. Nonspecific interstitial pneumonia (NSIP)
D. Respiratory bronchiolitis wiht interstitial lung dz (RBILD)
E. Usual interstitial pneumonia (UIP)

Back 16

E. Usual interstitial pneumonia (UIP)

This is also the most common of the idiopathic pulmonary fibrosises.

Front 17

Idiopathic pulmonary fibrosis is classified based on examination of surgical biopsy samples. Which is described below?

This shows mostly macrophages in the alveolar lumen. Responds to steroid treatment. Most of the patients are cigarette smokers, and treatment requires smoking cessation.

A. Bronchiolitis oblieterans / organizing pneumonia (BOOP)
B. Desquamative interstitial pneumonia (DIP)
C. Nonspecific interstitial pneumonia (NSIP)
D. Respiratory bronchiolitis wiht interstitial lung dz (RBILD)
E. Usual interstitial pneumonia (UIP)

Back 17

B. Desquamative interstitial pneumonia (DIP)

Front 18

Idiopathic pulmonary fibrosis is classified based on examination of surgical biopsy samples. Which is described below?

This is sometimes idiopathic, sometimes associated with collagen vascular dz and sometimes a result of inhalation caused by inhaled agents. This is a distinct pathologic entity.

A. Bronchiolitis oblieterans / organizing pneumonia (BOOP)
B. Desquamative interstitial pneumonia (DIP)
C. Nonspecific interstitial pneumonia (NSIP)
D. Respiratory bronchiolitis wiht interstitial lung dz (RBILD)
E. Usual interstitial pneumonia (UIP)

Back 18

C. Nonspecific interstitial pneumonia (NSIP)

The cellular type behaves more like DIP, the fibrotic type behaves more like UIP.

Front 19

Idiopathic pulmonary fibrosis is classified based on examination of surgical biopsy samples. Which is described below?

This dz is mild in severity and found exclusively in smokers. Nobody has ever died from it. Treatment means smoking cessation.

A. Bronchiolitis oblieterans / organizing pneumonia (BOOP)
B. Desquamative interstitial pneumonia (DIP)
C. Nonspecific interstitial pneumonia (NSIP)
D. Respiratory bronchiolitis wiht interstitial lung dz (RBILD)
E. Usual interstitial pneumonia (UIP)

Back 19

D. Respiratory bronchiolitis wiht interstitial lung dz (RBILD)

Front 20

Idiopathic pulmonary fibrosis is classified based on examination of surgical biopsy samples. Which is described below?

This is seen inassociation with drug reactions, infectious pneumonias, toxic inhalations, and connective tissue dz. Response to steroids is usually good.

A. Bronchiolitis oblieterans / organizing pneumonia (BOOP)
B. Desquamative interstitial pneumonia (DIP)
C. Nonspecific interstitial pneumonia (NSIP)
D. Respiratory bronchiolitis wiht interstitial lung dz (RBILD)
E. Usual interstitial pneumonia (UIP)

Back 20

A. Bronchiolitis oblieterans / organizing pneumonia (BOOP)

Front 21

Generally the more [ cellular / fibrotic ] the pattern of all the interstitial lung diseases, the better the response to treatment; the more [ cellular / fibrotic ] the pattern, the worse the response.

Back 21

CELLLAR = better response to treatment

FIBROTIC = worse the response to treatment

Front 22

What does treatment entail with interstitial lung dz? (4)

Back 22

1. Id cause if possible and prevent further exposure (applies to occupational dz and hypersensitivity)
2. suppress alveolitis if inflammation is present using anti-inflammatory drugs like prednisone or immunosuppressive drugs (more useful if histologic abnormalities are more cellular than fibrotic)
3. avoid hypoxemia (prevents pulmonary HTN from getting worse or at least slows down progression)
4. Prevent infection by vaccinating and avoiding sick contacts. Respiratory infections often cause major illness or death!

Front 23

The air-blood barrier in the lungs consists only of the cytoplasm of Type I alveolar linign cells, capillary endothelium, and their fused basal laminas. The alveolar septa do not contain lymphatics. Lymphatic vessels appear at the level of _________.

Back 23

respiratory bronchioles and are found in the interstitium of the bronchovascular bundles.

Front 24

Sarcoidosis is a granulomatous dz of unknown etiology that can affect multiple organs. Pulmonary involvement is common and can dominate the clinical picture or can be asymptomtic. Other common sites of involvement with sarcoidosis includes... (7)

Back 24

lymph nodes, eyes, skin, liver, spleen, kidney and heart

Front 25

With this interstitial lung dz, increased numbers of activated T helper cells are in lungs, depressed cellular immune reactivity is present in skin, and patients also often have hypergammaglobulinemia.

A. Hypersensitivity pneumonitis
B. Sarcoidosis
C. Usual interstitial pneumonia (UIP)

Back 25

B. Sarcoidosis

Front 26

Early or very active cases of this ILD can yield alveolar septa may be infiltrated by activated T lymphocytes.

A. Hypersensitivity pneumonitis
B. Sarcoidosis
C. Usual interstitial pneumonia (UIP)

Back 26

B. Sarcoidosis

Other microscopic pathological signs include granulomas, fibrosis (in minority of patients),

Front 27

What are microscopic pathological changes seen in sarcoidosis? (3)

Back 27

- alveolitis
- granulomas (characteristic histologic change of sarcoidosis)
- fibrosis (in minority of patients)

Front 28

True or False:
Well-formed non-necrotizing granulomas are typical of sarcoidosis.

Back 28

TRUE

Extensive necrosis (caseous necrosis) is typical of tuberculous granulomas, and are NOT features of sarcoidosis.

Front 29

Describe the granulomas in sarcoidosis with regards to:
A. number
B. placement
C. necrosis present?

Back 29

In sarcoidosis, granulomas are:
A. mulitple inn number
B. usually found throughout both lungs as well as scattered individually in the interstititium though in some cases they can coalesce into large aggregates --> nodules
C. no, necrosis (at least the caseating necrosis) is NOT present. some light scant fibrinoid (coagulative type) necrosis can sometimes be found though- VERY SCANT THOUGH!

Front 30

True or False:
The majority of patients with pulmonary sarcoidosis recover completely with disappearance of the granulomas.

Back 30

True.

Some patients, though, are left with focal residula disease or develop extensive pulmonary fibrosis to which the microscopic architecture becomes known as "honeycombing". Honeycombing is a nonspecific change that results from fibrosis though it can be fibrosis of other diseases including sarcoidosis.

Front 31

Gross pathology if lungs with sarcoidosis is highly variable depending on the extent of involvement. In many patients, granulomas are ........ whereas in patients with end-stage fibrosis, lungs look.......

Back 31

In many patients, granulomas are microscopic and no nodularity is evident grossly.

In end stage fibrosed lungs, there is increased firmness, usually with abnormally enlarged air spaces (honeycombing).

Front 32

Microscopic pathology of hypersensitivity pneumonitis includes what four things?

Back 32

1. Diffuse or patchy chronic insterstitial inflammation: principally composed of lymphocytes, and is centered on respiratory bronchioles
2. Small, poorly-formed , non-necrotizing granulomas: around bronchioles
3. Bronchiolitis obliterans-organizing pneumonia: usually patchy in distribution and consists of plugs of inflammatory cells, fibroblasts and loose fibrous tissue within the lumens of respiratory bronchioles and alveolar ducts.
4. Interstitial fibrosis (in chronic form): fibrosis is variable, and usualy minimal in early stages. granulomas are usually poorly formed and peribornchiolar with inflammation and bronchial obliteration

Front 33

In [ hypersensitivity pneumonia / sarcoidosis ], granulomas are poorly formed and peribronchiolar; in [ hypersensitivity pneumonia / sarcoidosis ], granulomas are well-formed and perilymphatic.

Back 33

hypersensitivity pneumonia = poorly formed and peribronch

sarcoidosis = well-formed granulomas and perilymphatic

Front 34

What are the histopathalogic changes seen in UIP (usual interstitial pneumonia)? (5)

Back 34

- interstitial fibrosis (heterogeneous)
- subpleural dominance
- fibroblastic foci
- interstitial inflammation
- ends stage: honeycombed lung

Front 35

A honeycombed lung refers to the gross adn microscopic appearance of end-stage pulmonary fibrosis of any etiology. It is characterized by what 4 things according to Grimes?

Back 35

- dilated airspaces with fibrotic walls
- smooth muscle hyperplasia
- metaplastic epithelium
- hyperplasia of Type II pneumocytes