Jeopardy

Question 1

Characteristics of Rare Disease

Answer 1

Chronic, Progressive, Degenerative and life threatening

Question 2

Challenges of rare diseases for the patients include:

Answer 2

limited scientific understanding of the disease
inequities and difficulties in accessing treatment and care
substantial economic impact for patients and their families

Question 3

Challenges of rare diseases for the HCP

Answer 3

healthcare provider’s unawareness of rare diseases and their inability to provide optimal care
• treatments not available, or when available, may be costly
lack of clinical guidelines for vast majority of rare diseases

Question 4

Orphan Drug Act 1983

Answer 4

7 year patent and tax credits

Question 5

In the United States, a disease or disorder is considered rare when it

Answer 5

affects fewer than 200,000 people in the country at any given time

Question 6

Patients with rare diseases comprise 1 out of every _______ Americans and _________ of the European Union (EU) population.

Answer 6

10; 6% to 8%

Question 7

ILD can be caused by HP (Hypersensitivity pneumonitis)

Answer 7

True

Question 8

Primary function of Resp System

Answer 8

Obtain O2 remove Co2

Question 9

Cell type responsible for gas exchange

Answer 9

Type 1 alveolar epithelial cells

Question 10

Healthy lungs

Answer 10

High lung capacity

Question 11

Obstructive Lung Dx

Answer 11

Asthma, COPD, Chronic Bronchitis, Cystic Fibrosis

Question 12

What % of IIP (Idiopathic Interstitial Pneumonia) have IPF (Idiopathic Pulmonary Fibrosis)

Answer 12

50%

Question 13

forced vital capacity (FVC)

Answer 13

which is the volume of air that can be forcefully exhaled from the lungs after taking the deepest breath possible

Question 14

The pathogenesis of interstitial lung disease

Answer 14

believed to initiate with an injury to structural cells in the lung that is not properly repaired.

Question 15

The pathogenesis of interstitial lung disease

Answer 15

results in repetitive injury to alveolar epithelial cells

Question 16

Which cells produce ECM

Answer 16

myofibroblasts

Question 17

Simplest PFT

Answer 17

Spirometry

Question 18

Low DLCO can indicate

Answer 18

poor gas exchange

Question 19

HRCT can be interpreted by

Answer 19

Radiologist

Question 20

According to the 2018 ATS/ERS/JRS/ALAT guidelines, the HRCT features most commonly seen in UIP (Usual Interstitial Pneumonia) are:

Answer 20

honeycombing, reticulation, traction bronchiectasis, and traction bronchiolectasis.

Question 21

IPF (Idiopathic Pulmonary Fibrosis) Radiologic you will see

Answer 21

UIP Pattern

Question 22

IPF (Idiopathic Pulmonary Fibrosis) Symptoms

Answer 22

Dyspnea, cough, bilateral crackles

Question 23

As the name interstitial lung disease implies, the microscopic changes that occur in the lungs of patients with ILD typically begin within

Answer 23

pulmonary interstitium

Question 24

a type of transbronchial biopsy that uses a frozen probe to extract lung tissue and has the advantage of removing a larger piece of lung tissue with a higher percentage of alveolar tissue

Answer 24

Cryobiopsy

Question 25

Probable UIP (Usual Interstitial Pneumonia) Distribution

Answer 25

Subpleural and basal predominant

Question 26

Bronchoalveolar Lavage (BAL)

Answer 26

BAL cell analysis may be useful in excluding other diagnoses, such as hypersensitivity pneumonitis

Question 27

Rheumatologist

Answer 27

a physician, specifically an internist or pediatrician, with additional training in the diagnosis and treatment of musculoskeletal diseases and systemic autoimmune conditions, including CTDs (connective tissue diseases) which are referred to as rheumatic diseases.

Question 28

the 2013 ATS/ERS classification system groups idiopathic interstitial pneumonias into 3 main categories

Answer 28

* major IIPs * rare IIPs * unclassifiable IIPs

Question 29

Underlying Conditions are INDEPENDENT of progressive fibrosis in PF-ILD

Answer 29

True

Question 30

The following three factors impact how well pulmonary gas exchange by diffusion can occur

Answer 30

* partial pressure gradients of O2 and CO2 * physical properties of the respiratory membrane * ventilation-perfusion coupling

Question 31

The classic example of Fibrosing ILD

Answer 31

IPF (Idiopathic Pulmonary Fibrosis)

Question 32

exposure to an HP( Hypersensitivity Pneumonitis)-inducing environmental agent, such as one of many different types of bacteria, fungi, animal proteins, and chemicals. How to treat?

Answer 32

Remove the agent

Question 33

TGF-β, PDGF, and FGF are

Answer 33

profibrotic cytokines whose signaling cascades are believed to play an important role in the pathogenesis of ILD.

Question 34

myofibroblasts lay down ECM that is primarily composed of

Answer 34

dense collagen fibers