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Flashcards in Restrictive Lung Disease Deck (18):

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


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,


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


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

Must have reduced lung volumes AND FEV1/FVC


Approximate PFT values/trends for Restrictive lung diseases



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


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


Acute restrictive diseases

Pulmonary edema
Pleural effusion


Chronic restrictive diseases

ILD -interstitial lung diseases
Pleural fibrosis / plaques
Pleural effusion


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)


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


Treatment of ILD

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


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


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


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


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



*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


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