Chronic Interstitial Lung Diz - Pathology

Question 1

Define chronic interstitial pulmonary disease

Answer 1

inflammation & fibrosis of the interstitium

(many unknown causes & pathogenesis)

Question 2

chronic interstitial pulmonary disease accounts for ______% of noninfectious pulmonary diseases

Answer 2

15

Question 3

Clinical and pathologic features of chronic intersitial lung disease that is shared with restrictive lung disease (3).

Answer 3

1. decreased compliance
2. decreased TLC
3. decreased diffusing capacity

Question 4

“honeycomb lung”

Answer 4

advanced stages of chronic interstitial fibrosis

(cor pulmonale is a possible complication)

Question 5

General pathophysiology of idiopathic pulmonary fibrosis

Answer 5

injured/activated alveolar pneumocytes & activated immune cells release profibrogenic cytokines → fibrosis

Question 6

Genetic factors associated w/idiopathic interstitial lung disease (3)

Answer 6

1. telomerase mutation (25%)
2. increased MUC5B→increased mucin → cell injury (30%)
3. increased signaling in the PI3K/AKT pathway

Question 7

Answer 7

IFP

(note the fibrosis of the lower lobes, subpleural areas and interlobular septa)

Question 8

Answer 8

IFP: yellow arrows: dense fibrosis

(early lesions have fibroblasts, late have collagen)

Question 9

Answer 9

Usual Interstitial Pneumonia

(fibroblasts, infiltrates, “honeycombing” & dilated air spaces lined by bronchiolar epithelium)

Question 10

Answer 10

“honeycomb lung”: cystically dilated air spaces

(end-stage lung disease)

Question 11

IPF tx

Answer 11

lung transplant

Question 12

IPF: Prognosis

Answer 12

4 years

Question 13

IPF sx

Answer 13

• early: dyspnea on exertion, dry cough
• late: hypoxemia, cyanosis, clubbing

Question 14

Age of presentation: IPF

Answer 14

55-75 yo

Question 15

“cryptogenic”

Answer 15

cause unknown

(ex: cryptogenic organizing pneumonia)

Question 16

define cryptogenic organizing pneumonia

Answer 16

loose organizing CNT fills alveoli & lumen of bronchioles

Question 17

_______ (3) are conditions associated w/cryptogenic organizing pneumonia.

Answer 17

1. bacterial & viral pneumonia
2. inhaled toxins, drugs
3. CNT disorders

Question 18

Answer 18

loose organizing CNT tissue

(aka “masson bodies”; note: alveoli are relatively normal)

Question 19

Cryptogenic organizing pneumonia: clinical presentation (2)

Answer 19

1. cough
2. dyspnea

Question 20

Cryptogenic organizing pneumonia: diagnostic findings (2)

Answer 20

1. patchy subpleural or peribronchial areas of consolidation on CXR
2. pulmonary function studies = restrictive pattern

Question 21

define sarcoidosis

Answer 21

noncaseating granulomas in many tissues

(MC lungs & lymph nodes)

Question 22

Sarcoidosis usually occurs in _______ (pt population)

Answer 22

• black adults > 40 yo

Question 23

Sarcoidosis pathogenesis

Answer 23

exaggerated CD₄+ T cell response to environmental antigen

(no evidence that it is infectious)

Question 24

Sarcoidosis genetic factors (2)

Answer 24

1. HLA-A1
2. HLA-B8

Question 25

Organs mainly affected by sarcoidosis include ______.

Answer 25

1. hilar & mediastinal lymph nodes
2. lungs

Question 26

Answer 26

non-caseating granulomas w/scattered multinucleated giant cells (Langhans type at the arrow)

Question 27

Answer 27

Sarcoidosis: lung bx; numerous noncaseating granulomas, some of which involve the wall of a bronchus.

The noncaseating granulomas in sarcoidosis frequently involve the walls of bronchi (found with bronchoscopic biopsy)

Question 28

Answer 28

Sarcoidosis: noncaseating granuloma (no necrosis).

• Blue arrow: foreign body type giant cell w/overlapping nuclei in the central portion of the cell. Langhans giant cells MC.
• Yellow arrow: Asteroid bodies (star-shaped crystalline structures) are present within the foreign body type giant cell. (They are characteristically seen in sarcoidosis; however, they are not specific for sarcoidosis)

Question 29

Clinical outcomes of sarcoidosis

Answer 29

• 70% full recovery
• 20%: lung or eye dysfunction
• 10-15% die

Question 30

Sarcoidosis: general & lung specific sx

Answer 30

• General: Fever, Fatigue, Weight loss, Night sweats
• Lung: SOB, cough, chest pain, hemoptysis

Question 31

Sarcoidosis Lab Findings (3)

Answer 31

1. CXR: bilateral hilar lymphadenopathy (w/pulm infiltrates)
2. bronchoalveolar lavage: CD4+ T cells, ⇡ TNF
3. ⇡ ACE levels

(also: anergy to common skin test Ags & polyclonal hypergammaglobulinemia)