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Pulmonology > Restrictive Lung Disease > Flashcards

Restrictive Lung Disease Flashcards

1
Q

Physiologic abnormalities in restrictive lung disease

A

TLC AND FRC < 80

Decreased compliance (stiffer lungs OR restricted motion of the chest wall)

P-V curve is flatter and shifted downward (reflecting lower compliance & decreased lung volumes)

FEV1/FVC often normal or slightly elevated

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2
Q

3 mechanisms that lead to restrictive physiology

A
  1. Increased thickness of lung interstitium (ILD)
  2. Increased lung water (interstitial/alveolar edema)
  3. Increased alveolar surface tension (RDSI, ARDS, pulmonary edema)
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3
Q

Differentiation of restrictive disease vs. physiology

A

P-V curve slope is reduced in restrictive lung disease but preserved in restrictive physiology (with lower TLC)

DLCO/VA is reduced in restrictive disease but preserved in restrictive physiology

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4
Q

PFT pattern of mixed lung disease

A

Reduced lung volumes (decreased TLC, FRC)

Reduced air flow (decreased FEV1/FVC)

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5
Q

Diagnostic approach to interstitial lung disease

A

Clinical

Radiographic (high resolution CT)

Pathologic (surgical lung biopsy)

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6
Q

Known causes of ILD

A

Autoimmune (RA, lupus, scleroderma)
Exposure to inorganic dusts (silica, asbestos)
Exposure to organic molecules (birds, mold)
Drug effects (chemo, radiation)
Familial

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7
Q

Sarcoidosis & Chronic Beryllium Disease - Pathology

A

Systemic, granulomatous inflammation disorders of unknown (Sarcoid) or known (CBD) etiology

Characterized by the presence of well-formed, non-necrotizing granulomas surrounded by collagen deposition; often follow lymphatic distribution

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8
Q

Sarcoidosis - Treatment

A

Usually none

Sometimes corticosteroids or methotrexate to treat/prevent progressive organ involvement

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9
Q

Idiopathic pulmonary fibrosis (IPF) - Definition

A

Idiopathic, scarring lung disease with a pattern of lung injury consistent with usual interstitial pneumonia (UIP)

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10
Q

Usual Interstitial Pneumonia (UIP)

A

Most common pattern if ILD

Patchy, heterogenous fibrosis of the septa by collagen; usually worse in lower lobes

Fibroblastic foci - compact collections of fibroblasts within the alveolar septae, bulging out into the airspaces

Honeycomb cystic change - formation of mucus-filled cysts surrounded by thick, fibrotic walls

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11
Q

IPF - Radiographic findings

A

Peripheral and basilar predominant reticulation and bronchiectasis

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12
Q

Idiopathic Nonspecific Interstitial Pneumonia (NSIP)

A

Uniform, homogenous inflammation (cellular type) or fibrosis (fibrotic type); cellular type is steroid-responsive

Lack of fibroblastic foci and honeycombing

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13
Q

Clinical characteristics of IPF vs. NSIP

A

NSIP is more likely to affect younger females, is more responsive to anti-inflammatory treatment, and has a better prognosis

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14
Q

Cryptogenic organizing pneumonia

A

Noninfectious pneumonia - may be idiopathic, secondary to drugs HP, or aspiration

Path shows organizing pneumonia with plugs of granulation tissue

Steroid responsive

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15
Q

Pulmonary manifestatiosn of ALS

A

Dysphagia - risk of aspiration pneumonia
Restrictive physiology due to respiratory muscle weakness
Hypoventilation - elevated PCO2

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16
Q

Pulmonary manifestations of Rheumatoid Arthritis

A

Rheumatoid effusion - exudative with increased protein and > 3,000 WBCs

Pleuritis

Lung nodules

Pulmonary hypertension (2/2 vasculitis)

17
Q

Goodpasture Syndrome

A

Caused by auto-antibodies directed against pulmonary and glomerular basement membrane proteins

Associated with diffuse alveolar hemorrhage, resulting in restrictive disease (due to alveolar destruction)

18
Q

Pulmonary manifestations of Sickle Cell Disease

A 
Acute Chest Syndrome
Infection (2/2 spleen auto-infarct)
Bone marrow emboli (2/2 bone marrow auto-infarct)
Lung infarction (2/2 in-situ thrombosis)
Pulmonary edema
Pulmonary HTN
19
Q

Acute pneumonia - Pathology

A

Characterized by an accumulation of neutrophils, macrophages, and fibrin within air spaces

20
Q

Eosinophilic pneumonia - Pathology

A

Characterized by an accumulation of eosinophils, macrophages, and fibrin within air spaces

21
Q

Organizing pneumonia (AKA BOOP, COP) - pathology

A

Fibroblast plugs filling airspaces - usually in a “patchy” pattern; some fibrin deposition within air spaces

Reversible, steroid responsive

22
Q

Respiratory bronchiolitis

A

Characterized by the presence of brown pigmented macrophages inside airspaces surrounding the respiratory bronchiole; air spaces farther away from the airway are usually spared

Smokig-related disease; macrophages are trying to clear the cigarette smoke particles but end up leading to air space destruction

23
Q

Diffuse alveolar damage (DAD) - pathology

A

Presence of fibrin ribbons (“hyaline membranes”) caked over the alveolar membranes; alveolar space are sealed off from gas exchange

This pattern is characteristic of ARDS and Respiratory Distress Syndrome of the Infant

24
Q

Diffuse alveolar hemorrhage - Pathology

A

Presence of blood and iron-containing macrophages within the air spaces; may be associated with capillaritis (neutrophils attacking the capillaries of the alveolar septa)

25
Q

Hypersensitivity Pneumonia (HP) - Pathology

A

Response to foreign antigens (birds, mold) characterized by:

Airway-centered chronic inflammation (lymphocytes and activated macrophages)

Non-necrotizing granulomas

Organizing pneumonia

26
Q

Causes of acute consolidation on CXR

A

HEAP

Hemorrhage
Edema
Alveolar Protein
Pneumonia

27
Q

Hypersensitivity Pneumonitis

A

Presents acutely with flu-like respiratory illness occurring after acute, high dose antigen exposure

28
Q

Chronic Beryllium Disease (CBD)

A

A chronic, granulomatous lung disease pathologically indistinguishable from sarcoid

Treatment: Inhaled or oral steroids

29
Q

Coal Workers’ Pneumoconiosis (Black Lung) - Diagnosis

A

Imaging shows small, rounded, nodular opacities present in the upper lobes

30
Q

Silicosis

A

Upper lobe predominant nodular interstitial lung disease; may progress to involve architectural distortion and volume loss (complicated silicosis)

31
Q

Asbestosis

A

Bilateral fibrosis affecting the lower lung zones preferentially

32
Q

Non-malignant asbestos-related lung diseases

A

Asbestos pleural effusion
Pleural thickening/calcification
Rounded atelectasis
Asbestosis

33
Q

Malignant asbestos-related lung diseases (2)

A

Lung cancer - all histologic types

Mesothelioma

34
Q

5 ILDs associated with environmental exposure

A 
Asbestos-related lung disease
Silicosis 
Coal Miner's Pneumoconiosis (Black Lung) 
Chronic Beryllium Disease 
Hypersensitivity Pneumonitis
35
Q

Risk groups for asbestos exposure

A

Construction workers
Boilermakers
Shipyard and dock workers
Automechanics who do brake work

36
Q

Risk groups for silicosis

A

Workers who blast, cut, or grind hard rock
Sandblasters
Foundry workers
Stone-washed jean manufacturers

37
Q

Risk groups for Chronic Beryllium Disease

A

Beryllium is a lightweight metal used in aerospace engineering and high tech electronics

Aerospace workers, airplane mechanics, air traffic controllers, etc. are all at higher risk

38
Q

Triggers of Hypersensitivity Pneumonitis

A 
Animal proteins (birds)
Microbial aerosols (fungi, hot tubs and indoor pools, humidifiers)

Pulmonology (15 decks)

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