Interstitial Lung Disease

Front 1

What is the most important thing that you need to get from a person who is presenting with something that is looking like an interstitial lung disease?

Back 1

A good history!!!

A good history!!!

A good history!!!

Front 2

What is the characteristic finding in all of the restrictive lung diseases?

Back 2

Decreased lung volume: decreased TLC

Front 3

What are some of the different classes of restrictive lung diseases?

Back 3

Chest wall/pleural
Parenchymal
Neuromuscular weakness

Front 4

What are some of the chest wall/pleural diseases that can restrict the lungs?

Back 4

Kyphoscoliosis
Obesity

Front 5

What are some of the parenchymal lung diseases that can restrict the lungs?

Back 5

Interstitial lung diseases
Diffuse parenchymal lung diseases

Front 6

What are some of the neuromuscular weaknesses that can restrict the lungs?

Back 6

Inflammatory myopathies
ALS
Myasthenia gravis
Guillan Barre, CIPD
Paralysis of diaphragm

Front 7

In your differential diagnosis for diffuse parenchymal pulmonary disorders, what are the different classes?

Back 7

-Known cause
-Idiopathic interstitial pneumonia
-Granulomatous
-Other

Front 8

What does DPLD stand for?

Back 8

Diffuse parenchymal lung disease

Front 9

What are some of the DLPDs of known causes?

Back 9

Connective tissue
Drug-induced
Pneumoconiosis

Front 10

What are some of the DLPDs that fall under the class of idiopathic interstitial pneumonia?

Back 10

Idiopathic pulmonary fibrosis (IPF) - most common
Non-specific pulmonary fibrosis

Front 11

What are some of the causes of granulomatous DPLD?

Back 11

Sarcoidosis
HP

Front 12

What are some of the DLPDs in the class of "other"?`

Back 12

Lymphangiomyotosis
EG/LCH
Eosinophillic pneumonia
Pulmonary alveolar proteinosis

Front 13

What types of DPLDs have an acute presentation?

Back 13

Acute hypersensitivity pneumonitis
Acute interstitial pneumnia
Acute eosinophilic pneumonia

Front 14

What is the typical timecourse for interstitial lung disease?

Back 14

Chronic - they take a long time to develop (most of the time - there's a couple of exceptions)

Front 15

What are the common symptoms of DPLDs?

Back 15

Dry, chronic cough along with dyspnea.

Many of the chronic conditions causing cough aren't also associated with dyspnea.

Lack of constitutional symptoms

Front 16

What sort of imaging should you order if you suspect DPLDs?

Back 16

High-resolution CTs

Front 17

What are the DPLDs that have an acute presentation?

Back 17

Acute idiopathic interstitial pneumonia
Eosinophilic pneumonia
Hypersensitivity pneumonia
Cryptogenic organizing pneumonia

Front 18

What type of a person should you think of as sterotypic for eosinophilic pneumonia?

Back 18

A young person who chain smokes

Front 19

What DPLDs have a subacute presentation?

Back 19

Sarcoidosis
Drug induced ILDs
Alveolar hemorrhage syndromes
Connective tissue diseases

Front 20

What DPLDs have a chronic presentation?

Back 20

Idiopathic pulmonary fibrosis
Sarcoidosis

Front 21

What are the most common causes of ILD from a persons's occupation?

Back 21

Silicosis (miners, sandblasters, foundry)
Asbestosis (ship builders, miners, insulation)
Coal workers

Front 22

What are some common environmental exposures that can cause ILDs?

Back 22

Organic dusts
Chemicals
Pets!

Front 23

What is the presentation of familial IPF? Inheritance?

Back 23

Young age, associated with surfactant problems

Autosomal dominant

Front 24

What is hermansky-pudlack disorder? What is it associated with? What is the inheritance?

Back 24

An interstitial lung disease

Albinism, platelet disorders

Autosomal recessive

Front 25

What are some of the extrapulmonary signs of ILD?

Back 25

Rash
Arthritis
Weakness
Visual changes
Neurologic problems
Hematuria

Front 26

What are some pulmonary signs of ILD?

Back 26

Pleuritic chest pain

Hemoptys

Front 27

What sign on physical exam should tip you off for interstitial lung disease?

Back 27

Dry rales

(TEST QUESTION)

Front 28

If the person comes back with a negative chest xray, should you exclude a diagnosis of ILD?

Back 28

NO.

Front 29

What are the PFT findings with ILD?

Back 29

FVC: decreased
FEV1: decreased
FEV1/FVC: increased
TLC: decreased
DLCO: decreased

Front 30

What are the labs that you should get if you're thinking ILDs?

Back 30

Serology to evaluate for:
-CVD
-Environmental exposures
-Systemic vasculitis

Front 31

What changes do you see to the pulmonary physiology in ILD?

Back 31

Restrictive ventilatory defects (low TLC)

Impaired gas exchange

Front 32

What patterns are present on this CT scan?

Back 32

Top arrow: ground glass appearance - gray, not white.

Bottom two arrows: mosaic attenuation - white and black interspersed, due to focal air trapping

Front 33

What feature is indicated on this CT scan? What conditions is it common in?

Back 33

Honeycomb cysts

Interstitial lung disease

Front 34

What feature is indicated by the red arrow here? In what condition is it common? What is the cause?

Back 34

Traction bronchiectasis

ILDs

Damage to the bronchioles, causing them to not taper down when going to the periphery of the luns

Front 35

What changes occur to CD4/CD8 ratio in sarcoidosis?

Back 35

Elevated

Front 36

What changes happen to CD4/CD8 ratio in hypersensitivity pneumonitis?

Back 36

Decreased

Front 37

What are the indications for bronchoalveolar lavage in the diagnosis ?

Back 37

If you have a suspicion of:

Granulomatous diseases
Malignancies

Not to useful for diagnosis of most ILDs

Front 38

What is the way to definitively diagnose ILDs?

Back 38

Surgical biopsies

Front 39

What is a good imaging modality for distinguishing between IPF and NSIP?

Back 39

HRCT

Front 40

What findings on HRCT make you think of IPF vs. NSIP?

Back 40

Honeycomb changes in a basilar/peripheral distribution

No ground glass

Front 41

If there's no ground glass and honeycombing on HRCT, what's the diagnosis? Should you biopsy?

Back 41

IPF

No - they've already got a death sentence.

Front 42

On a surgical biopsy, what's the main histological difference between UIP and NSIP?

Back 42

In NSIP, there's more cellularity (which correlates to a ground glass type of appearance on HRCT)

In UIP, there's holes (which is consistent with the honeycombing)

Front 43

What, actually, is UIP?

Back 43

UIP stands for usual interstitial pneumonia

It is a type of HISTOPATHOLOGY that is found in different types of interstitial lung diseases.

It is NOT a diagnosis. It is just a pathologic feature of diseases

Front 44

What types of conditions, both idiopathic interstitial pneumonias (IIP) and secondary systemic diseases, have UIP pathology?

Back 44

IIPs: idiopathic interstitial pneumonia

Secondary systemic disorders: rheumatoid arthritis, asbestosis

Front 45

What kind of conditions, both IIPs and secondary systemic disorders, exhibit with NSIP pathology?

Back 45

IIP: NSIP

Secondary systemic: SLE, scleroderma, polymyositis

Front 46

What secondary systemic diseases show NSIP pathology?

Back 46

SLE
Scleroderma
Polymyositis

Front 47

What secondary disorders show UIP pathology?

Back 47

RA
Asbestosis

Front 48

What is the prognosis for UIP pathology?

Back 48

Very poor - a death sentence

Front 49

What is the prognosis for NSIP pathology?

Back 49

Farily good

Front 50

What is the cause of RB-ILD (respiratory bronchiolitis interstitial lung disease) and DIP (desquamitive interstitial pneumonia)?

Back 50

Smoking

Get people to stop!

Front 51

What is AIP? What is the prognosis? Who gets this?

Back 51

Acute interstitial pneumonia

Very poor - it's essentially ARDS

Young people

Front 52

What are the IIPs and secondary systemic diseases associated with lymphocitic pneumonia?

Back 52

IIPs: LIP (lymphocitic inerstitial pneumonia)

Secondary: Sjoren's syndrome, HIV

Front 53

Is UIP normally homo or heterogenous?

Back 53

Heterogenous

Front 54

What is the best treatment for a person with IPF?

Back 54

Lung transplant