Unit 3 Day 6 (Tue 4/28)

Question 1

Primary Physiologic Abnormalities in Restrictive Lung Disease

Answer 1

TLC

Question 2

Restriction

Answer 2

• hard to inhale

- affects inhalation work only

Question 3

Obstruction

Answer 3

• hard to exhale

- inc. resistive work- both inspiration and expiration

Question 4

Diseases/Processes That Lead to Restrictive Physiology

Answer 4

Restrictive Physiology Due to inc. in Elastic Recoil:

• inflammation or scarring may expand the interstitium and reduce compliance
• pulmonary edema
• surfactant depletion
• any alveolar filling process
• ARDS (acute respiratory distress syndrome)
• pneumonia
• cardiac pulmonary edema

Restrictive Physiology Due to Chest Wall Disease:

• pleural effusion
• asbestos-related pleural plaque
• kyphoscoliosis

Question 5

Two features distinguish restrictive lung disease from restrictive physiology (chest wall restriction):

Answer 5

 Slope of P-V curve is reduced in restrictive disease.
 DLCO/VA is reduced in restrictive disease.
 Both are preserved in restrictive physiology without restrictive disease.

Question 6

Gas Exchange is _______ in Restrictive Lung Disease.

Answer 6

-Impaired
 Loss of alveolar surface area
 due to decreased lung volumes and
decreased alveolar volume
 Diffusion limitation
 Restrictive chest wall diseases generally do not cause gas exchange problems

Question 7

Acute Restrictive Processes

Answer 7

 Pulmonary edema
 ARDS/DAD
 Pneumonia
 Pleural effusion

Question 8

Chronic Restrictive Processes

Answer 8

 ILD - interstitial lung
diseases
 Pleural fibrosis / plaques
 Pleural effusion

Question 9

Mixed Obstructive an Restrictive Disease

Answer 9

Must have reduced lung volumes AND reduced airflow (FEV1/FVC

Question 10

Interstitial Lung Disease (ILD)

Answer 10

 Heterogeneous group of disorders characterized by a combination of inflammation or scarring
 Classified by etiology and pattern

Question 11

ILD Presentation

Answer 11

• Dyspnea with exertion, cough
• Subacute to insidious timing
• Restrictive PFTs with decreased DLCO
• Abnormal lung imaging
• Variable prognosis; specific diagnoses matter!

Question 12

ILD Diagnosis

Answer 12

-clinical-radiographic-pathologic diagnosis:
• Clinical info: associated diseases, exposures, drugs, lab testing
• Radiographic pattern on HRCT
• Pathologic pattern on surgical lung biopsy

Question 13

ILD Physical Exam

Answer 13

 Auscultation: end- inspiratory “velcro-like” crackles
 Digital clubbing in advanced cases.
 Joint and skin exam is important in identifying coexistent (causative?) collagen vascular disease.

Question 14

ILD Treatment

Answer 14

• Remove offending exposure if possible
• Immunosuppressive therapy – Depends on type and etiology
• IPF: nintedanib, pirfenidone • Oxygen
• Transplantation

Question 15

Idiopathic Pulmonary Fibrosis

Answer 15

 IPF is a scarring lung disease with a pattern of injury of usual interstitial pneumonia (UIP).
 Etiology is idiopathic.
(There are known causes for the same UIP pattern of injury).
 Disease of older patients ( > 6th decade)
 Associated with tobacco use
 Cough, DOE, fatigue
 Physical exam: Basilar predominant “velcro-crackles.” Digital clubbing in advanced cases.
 Median survival from diagnosis is 2-3 years
 Imaging: Peripheral and basilar predominant reticulation, traction bronchiectasis, honeycombing, and a paucity of ground glass infiltrate.
 Path: Spatially and temporally heterogeneous fibrosis with fibroblast foci and juxtaposition of fibrotic and normal lung.
 Pirfenidone and nintedanib were approved to treat IPF in Fall 2014.

Question 16

Nonspecific Interstitial Pneumonia (NSIP)

Answer 16

NSIP presents similarly to other ILD
May be idiopathic, collagen vascular disease- related (RA, Sjögren’s, scleroderma, myositis), fibrotic HP, or drug-related.
More likely to be female; younger.
 Prognosis is better than UIP/IPF, and collagen vascular-related may have better prognosis than idiopathic.
Responds to anti-inflammatory therapy
 Radiographic – basilar predominant reticular abnormality with volume loss, traction bronchiectasis. Frequent ground glass opacity. Rare honeycombing.
 Path: temporally homogeneous fibrosis with varying degrees of inflammation and fibrosis.

Question 17

Smoking Related ILD

Answer 17

Respiratory bronchiolitis-ILD
Desquamative interstitial pneumonia (DIP)
 These two diagnoses exist on a spectrum
 Relatively rare compared to other IIP
 Smoking cessation is key
Pulmonary Langerhans Cell Histiocytosis

Question 18

Smoking Related ILD- Pulmonary Langerhans Cell Histiocytosis (PLCH)

Answer 18

 Young smokers
 Imaging: cysts and nodules
 Upper lobe predominant
 Mixed PFTs
 PTX common
 15% extrapulm dz—including bone lesions, pituitary involvement
 Treatment is smoking cessation

Question 19

Organizing Pneumonia

Answer 19

 Subacute to chronic presentation
 Noninfectious pneumonia
 Imaging shows ground glass and consolidation that may be migratory
 Path: organizing pneumonia
 Plugs of granulation tissue and
fibrosis distal to bronchioles
 Very steroid responsive, but requires 6-12 months.
 Relapses with rapid steroid withdrawal.

Question 20

Eosinophilic Pneumonia

Answer 20

 Acute eosinophilic pneumonia

• Mimics ARDS
• Often results in acute respiratory failure
• Diagnosed by BAL showing eosinophils
• Resolves with steroids

 Chronic eosinophilic pneumonia
-Subacute onset
-Associated with asthma
-Peripheral eosinophilia common
-“Photographic negative of pulmonary
edema”
-Also diagnosed by BAL 
-Treated with steroids

Question 21

Lymphangioleiomyomatosis (LAM)

Answer 21

 Cystic lung disease exclusively of young women
 Mutation in tuberous sclerosis gene 1 or 2
 Peribronchovascular proliferation of smooth muscle cells
 Cysts and nodules
 Spontaneous pneumothorax and
chylothorax common
 Obstructive pattern on PFTs
 Renal tumors (angiomyolipomas)
 Potential treatment with mTOR inhibitors (sirolimus)

Question 22

Sarcoidosis

Answer 22

 Abnormal CXR in up to 90%
 Lymphadenopathy is common
 Fibrosis may develop in up to 20%
 Airway involvement in up to half
 May have restrictive, obstructive or mixed PFTs
 Cough, chest pain or wheezing
 Presents before 40 y.o.
 Commonly affects northern European descent; can affect
any race/ethnicity
 More common in African Americans than white Americans
 more likely to be severe and progressive
• Diagnosis is based on clinical findings and confirmed with tissue demonstrating granulomas
• Treatment is often not necessary
• Progressive disease is treated with steroids and cytotoxic drugs