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Flashcards in Restrictive Lung Disease Deck (18):
1

Define the primary physiologic abnormalities in restrictive lung disease

Restrictive physiology is defined by reduced lung volumes
Inhalation is affected
At a given pressure, volume is lower in restrictive disease

2

Describe general mechanisms that lead to restrictive physiology (i.e. diseases and/or processes)

May be caused by increase in lung elastic recoil (stiffness) or Chest wall or pleural disease
Diseases: Pleural Effusion, Asbestos-Related Pleural Plaque, Kyphoscoliosis,

3

Determine how PFTs can distinguish between increased lung elastic recoil vs. increased chest wall resistance
(i.e. differentiate between restrictive physiology and
restrictive lung disease)

*Restrictive lung disease is a subset of restrictive pathology
*Slope of P-V curve is reduced in restrictive disease.
*DLCO/VA is reduced in restrictive disease
*Both P-V curve and DLCO/VA are preserved in restrictive physiology without restrictive disease

4

Review how PFTs are interpreted in patients with mixed obstructive /restrictive lung disease

Must have reduced lung volumes AND FEV1/FVC

5

Approximate PFT values/trends for Restrictive lung diseases

TLC

6

What is lung compliance affected by?

tissue content of lung
lung water
surface tension
Either inflammation or scarring may expand the interstitium and reduce compliance

7

Causes of Increased Elastic Recoil

1)Pulmonary edema: Interstitial edema increases mass of and stiffens interstitium. Alveolar edema depletes surfactant; increases mass and stiffness of parenchyma

2)Surfactant depletion –increases alveolar surface
tension, leading to alveolar collapse

3)Any alveolar filling process

8

Acute restrictive diseases

Pulmonary edema
ARDS/DAD
Pneumonia
Pleural effusion

9

Chronic restrictive diseases

ILD -interstitial lung diseases
Pleural fibrosis / plaques
Pleural effusion

10

Interstitial lung disease (ILD)

Heterogeneous group of disorders characterized by a
combination of inflammation or scarring
Can be drug induced, genetic/inherited, Idiopathic (ie: sarcoidosis), exposure to environmental pathogens, or systemic diseases (ie: collagen vascular disease, immunodeficiency disorder)

11

Presentation of ILD

Dyspnea with exertion, cough
Subacute to insidious timing
Restrictive PFTs with decreased DLCO
Abnormal lung imaging
Auscultation: end-inspiratory “velcro-like” crackles
Digital clubbing in advanced cases

12

Treatment of ILD

Remove offending exposure if possible
Immunosuppressive therapy
IPF: nintedanib, pirfenidone
Oxygen
Transplantation

13

Idiopathic Pulmonary Fibrosis

*Scarring lung disease with a pattern of injury of usual interstitial pneumonia (UIP)
*Etiology is idiopathic
*Disease of older patients
*Associated with tobacco use
*“velcro-crackles” on auscultation
*Peripheral and basilar predominant reticulation, traction
bronchiectasis, honeycombing, and a paucity of ground glass infiltrate
*Treat with Pirfenidone and nintedanib

14

Nonspecific Interstitial Pneumonia

*presents similarly to other ILD
*May be idiopathic, collagen vascular disease-related (RA, Sjögren’s, scleroderma, myositis), fibrotic, or drug-related
*More likely to be female; younger
*Responds to anti-inflammatory therapy
*basilar predominant reticular abnormality with volume loss, traction bronchiectasis. Frequent ground glass opacity. Rare honeycombing

15

Smoking-related ILD

*Respiratory bronchiolitis
*Desquamative interstitial pneumonia
*Pulmonary Langerhans Cell Histiocytosis: Young smokers, Imaging: cysts and nodules, Upper lobe predominant, Mixed PFTs
*Treatment is smoking cessation

16

Lymphangioleiomyomatosis (LAM)

*Cystic lung disease exclusively of young women
*Mutation in tuberous sclerosis gene 1 or 2
*Spontaneous pneumothorax and chylothorax common
*Obstructive pattern on PFTs
*Renal tumors

17

Sarcoidosis

*Systemic granulomatous disorder of unknown etiology
*Noncaseating granulomas on path
*Commonly affects: Lungs, Eyes, Skin
*May have restrictive, obstructive or mixed PFTs
*Presents before 40 y.o
*More common in African Americans than white Americans

18

Eosinophilic Pneumonia

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, treat with steriods