Diffuse Parenchymal Lung Disease

Question 1

What can effect the interstitium

Answer 1

• edema
• malignancy
• infections
• inflammation and fibrosis (INTERSTITIAL LUNG DISEASE)

Question 2

Mechanism of interstitial lung disease

Answer 2

1. lung injury: environment, autoimmune, unknown (can lead to 2 or 3)
2. inflammation: this can lead to possible resolve or 3
3. lung fibrosis

Question 3

Causes of primary interstitial lung disease (ILD)

Answer 3

• idiopathic interstitial pneumonias

- sarcoidosis

Question 4

Causes of secondary ILD

Answer 4

• drugs
• exposures/occupations
• radiation
• aspiration
• connective tissue disease related ILD (autoimmune)
• smoking-related

Question 5

Important aspect to diagnosing ILD

Answer 5

• taking a good history

Question 6

Types of diffuse parenchymal lung disease

Answer 6

• smoking related
• environmental exposure
• granulomatous
• autoimmune
• idiopathic interstitial pneumonias

Question 7

Signs of sarcoidosis

Answer 7

• uveitis
• erythema nodosum (bruises on shins)
• noncaseating granulomas

Question 8

Person to first diagnose someone with sarcoidosis & when

Answer 8

J. Hutchinson - 1878

Question 9

Systems effected by sarcoidosis

Answer 9

• multisystem disease: every system can be effected

- most common: lung (95%), eyes, lymph nodes, liver, skin

Question 10

Who gets sarcoidosis

Answer 10

• all ages, gender, races

- familial

Question 11

Incidence/Prevalence of sarcoidosis

Answer 11

• highest in african american women

- norther european african americans (sweden)

Question 12

Clinical manifestations of sarcoidosis by country

Answer 12

• US/Europe: respiratory disease

- Japanese: cardiac & eye disease

Question 13

What defines sarcoidosis

Answer 13

• granuloma with no identifiable bug

Question 14

What is a granuloma

Answer 14

• macrophages, epitheliod cells, multinucleated giant cells

- surrounded by CD4 T cells (Th1) w/ few CD8 and B cells

Question 15

How do patients present w/ sarcoidosis

Answer 15

• skin abnormality
• fevers, night sweats, fatigue, weight loss
• cough, shortness of breath
• chest pain, palpitations, arrhythmia
• NO symptoms at all**

Question 16

Aspects needed to diagnose sarcoidosis

Answer 16

• clinical symptoms
• radiograph
• biopsy w/ pathology
• MUST RULE OUT ANY OTHER CAUSE: infections, cancer, foreign bodies, inhalations*

Question 17

Meaning behind staging of sarcoidosis

Answer 17

• doesn’t mean you have a worse prognosis

- deals with how you will treat the patient

Question 18

Clinical course of sarcoidosis

Answer 18

• spontaneous remission: 50-70%
• wax and wane-chronic and persistent: 30-50%
• progressive: 1-5%

Question 19

Treatment of sarcoidosis

Answer 19

• lacking evidence
• vital organ involvement such as eye, cardiac, neuro: corticosteroids
• other immunosuppressants

Question 20

Clinical case of fibrosis

Answer 20

• old age

- chronic

Question 21

Physical exam findings of fibrosis

Answer 21

• low O2 when walking
• VELCRO crackles w/ NO wheezing
• mild clubbing

Question 22

Percentage of idiopathic pulmonary fibrosis (IPF) patients that have crackles

Answer 22

80%

Question 23

Percentage of IPF patients that have clubbing

Answer 23

50%

Question 24

Pulmonary function test results of IPF patients

Answer 24

• low FVC, FEV, total lung capacity, diffusing capacity

Question 25

Type of lung disease in IPF

Answer 25

- restrictive lung disease w/ severely reduced diffusing capacity

Question 26

Radiograph findings of IPF patient

Answer 26

- reduced lung volume - honeycombing - lower lobe predominance

Question 27

Diagnosing idiopathic pulmonary fibrosis (IPF)

Answer 27

- exclude other known causes of pulmonary fibrosis - abnormal pulmonary function tests - features of pulmonary fibrosis on CT scan - usual interstitial pneumonia an open lung biopsy

Question 28

Other causes of pulmonary fibrosis

Answer 28

- medications: amiodarone, nitrofurantoin, cocaine/heroin, mineral oil, chemo, anti TNF - medical history: autoimmune disease

Question 29

Social history showing possible causes of IPF

Answer 29

- smoking, occupation, hobbies, inhalations, drugs, mold exposure, pets (BIRDS ARE BAD), hot tubs (mycobateria)

Question 30

Mechanism of injury in IPF

Answer 30

insult to epithelium and basement membrane--aberrant wound healing or vascular injury--fibrotic foci (fibroblast proliferation, collagen deposition)--Fibrosis (circulating fibroblasts also contribute) ***INFLAMMATION does NOT play a roel***

Question 31

Does inflammation play a role in IPF

Answer 31

NO INFLAMMATION DOES NOT PLAY A ROLE

Question 32

Another name for idiopathic pulmonary fibrosis

Answer 32

usual interstitial pneumonia

Question 33

Median survival of patient w/ IPF

Answer 33

3-5 years

Question 34

Treatment for IPF

Answer 34

- anti fibrotics: prifenidone, nintedanib - pulmonary rehab - oxygen - lung transplant

Question 35

Diffuse parenchymal lung disease types and treatments

Answer 35

- smoking related: stop smoking - environmental exposure: remove exposure - granulomatous: steroids/immunosuppressants - idiopathic interstitial pneumonias: steroids/immuno/ANTIFIBROTIC - autoimmune/collagen vascular disease: steroids/immuno

Question 36

How is the type of interstitial lung disease determined

Answer 36

- by the type, amount, location of cells