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2. HDS Pulmonary > Parenchymal Lung Diseases and Sarcoidosis > Flashcards

Parenchymal Lung Diseases and Sarcoidosis Flashcards

1
Q

Pulmonary Interstitium

Components

A
  • BM of endothelial & epithelial cells
  • Collagen fibers
  • Elastic tissue
  • Proteoglycans
  • Fibroblasts
  • Mast cells
  • Few lymphocytes and monocytes
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2
Q

Diffuse Parenchymal Lung Diseases (DPLD)

Overview

A

Diffuse and predominantly chronic changes of the pulmonary CT.

  • Affects most peripheral and delicate interstitium in alveolar walls
  • Diseases share clinical signs, sx, radiographic ∆, and pathophysiologic ∆
    • ± Intra-alveolar component
    • Freq. overlap in histologic features
  • Many have unknown etiologies
  • Accounts for 15% of noninfectious pulmonary diseases
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3
Q

DPLD

Known Etiologies

A
  • Environmental
    • Asbestos
    • Silica
    • Fumes
    • Gases
  • Ionizing radiation
  • Sequela of ARDS
  • Drugs
    • Busulfan
    • Bleomycin
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4
Q

DPLD

Pathogenesis

A
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5
Q

DPLD

Clinical Features

A

See clinical and pulmonary functional changes of restrictive disease:

  • Dyspnea & tachypnea ⇒ eventual cyanosis without wheezing
  • ↓ Oxygen diffusing capacity
  • ↓ Lung volumes
  • ↓ Compliance
  • Pulmonary HTN
  • Cor pulmonale
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6
Q

DPLD

Radiographic Features

A

Diffuse infiltration by small nodules or shadows

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7
Q

DPLD

Morphology

A

Similar histologic appearance in late stages:

Honeycomb lung or end-stage lung

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8
Q

Diffuse Parenchymal Lung Diseases

Types

A
  1. Idiopathic Pulmonary Fibrosis
  2. Non-specific Interstitial PNA
  3. Desquamative Interstitial PNA
  4. Acute Interstitial PNA
  5. Cryptogenic Organizing PNA
    • aka Bronchiolitis Obliterans with Organizing PNA (BOOP)
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9
Q

Idiopathic Pulmonary Fibrosis

Epidemiology

A
  • Age > 60 years
  • 7-16 cases/100k
  • ♂ > ♀
  • 75% former or current smokers
  • Rare familial cases
  • Poor prognosis ⇒ 5 year survival rate 50%
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10
Q

Idiopathic Pulmonary Fibrosis

Possible Causes

A
  • Alveolitis ⇒ Fibrosis
    • Repeated cycles of alveolitis provoked by unknown agent
  • Direct fibrosis
  • Aberrant wound healing with fibroblastic proliferation
  • Peripheral location ⇒ effect of gravity or aspiration
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11
Q

IPF

Histology

A

Termed “Usual Interstitial Pneumonia” (UIP)

Pathological definition

Patchy fibrosis most prominent in subpleural region

Temporal heterogeneity ⇒ alternating zones of inflammation, active fibrosis, scarring, honeycomb and normal lung

Usually affects lower lobes.

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12
Q

IPF

Radiology

A

Generally lower lobe and peripheral interstitial changes.

Central lung and upper lobe sparing typical.

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13
Q

IPF

Treatment

A
  • No proven therapy
  • Poor response to corticosteroids & combo therapy
  • Clinical trials ongoing
    • Anti-fibrotic drugs ⇒ some slowing of progression
  • Supportive care
    • Pulmonary rehab
    • Vaccines
    • Nutrition
    • Cough control
  • Only definitive therapy is lung transplant
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14
Q

Non-specific Interstitial Pneumonia (NSIP)

Epidemiology

A
  • Associations ⇒ HIV, CT diseases, drugs
  • Onset 5th and 6th decades
    • Earlier than IPF
  • Insidious onset
  • No male/female predominance
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15
Q

NSIP

Histology

A

Cellular and fibrosing patterns

Temporal uniformity ⇒ suggests that NSIP is not due to recurrent sequential bouts of alveolitis

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16
Q

NSIP

Radiology

A

CXR not necessarily able to differentiate NSIP from IPF.

CT can show a ground glass appearance (alveolar pattern)

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17
Q

NSIP

Treatment

A
  • Steroids and other immune modulators may be helpful
  • Treat underlying rheumatologic disease if present
  • Outcomes extremely variable
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18
Q

Diffuse Interstitial Pneumonia (DIP)

Epidemiology

A

“Desquamative Interstitial Pneumonia”

  • Exclusively in smokers
    • Usually heavy 30+ pack years
  • Onset in 4th and 5th decade
  • More common in men
  • 8% of total IPF cases
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19
Q

DIP

Histology

A
  • Airspaces ⇒ many Mφ with brown pigment (yellow star)
  • Alveolar septae thickened with lymphocytic infiltrate
  • Prominent pneumocytes (arrow)
  • Mild interstitial fibrosis
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20
Q

DIP

Radiology

A

More lower lobe involvement.

Ground glass appearance on CT

Milder changes compared to others.

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21
Q

DIP

Treatment

A
  • Quit smoking
  • Steroids if needed
  • Generally a good prognosis
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22
Q

Cryptogenic Organizing Pneumonia (COP)

Overview

A

“Bronchiolitis Obliterans Organizing Pneumonia” (BOOP)

  • Etiology unknown
    • Often linked to viral, drug, or cardiovascular disease
    • Idiopathic
  • Pt presents with subacute cough and SOB
  • Onset 5th decade most commonly
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23
Q

COP

Histology

A
  • Polypoid plugs of loose organizing CT within airspaces
  • Do not see interstitial fibrosis or honeycomb lung
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24
Q

COP

Radiology

A

Subpleural or peribronchial patchy areas of airspace consolidation.

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25
Q

COP

Treatment

A

Responds well to several months of steroids.

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26
Q

Acute Interstitial Pneumonia (AIP)

Pathophysiology

A

“Hamman-Rich Syndrome”

  • Pathologic features of ARDS appears acutely in pt without known triggers
  • Fulminant presentation
  • Rapid progressive clinical course
  • Average age 50-55 y/o
  • Not smoking related
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27
Q

AIP

Histology / Pathology

A

Termed diffuse alveolar damage (DAD)

  • ↑ Pulmonary vascular permeability
  • Interstitial inflammation
  • Epithelial cell death
  • See hyaline membranes lining alveolar spaces
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28
Q

AIP

Radiology

A

Diffuse, bilateral alveolar infiltrates.

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29
Q

Interstitial Lung Disease

Clinical Approach

A
  • History
    • Age
    • Fhx
    • Drugs, CT diseases, dust exposure, Beryllium, World Trade Center, etc.
  • Physical exam
  • CXR
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30
Q

DPLD

Summary Table

A
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31
Q

Sarcoidosis

Definition

A

Need all 3 of the following:

  • Compatible clinical symptoms
  • Evidence of non-caseating granulomas
  • Rule out other causes of granuloma
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32
Q

Sarcoidosis

Epidemiology

A
  • Affects all races and ethnic groups
  • ♀ > ♂
  • Peak incidence age 20-39 y/o
  • Most affected populations:
    • Northern Europeans
    • Japan
    • African Americans
33
Q

ACCESS Study

A

No single predominant cause of sarcoidosis found.

34
Q

Sarcoidosis

Environmental Associations

A
  • Beryllium
  • Inhalation of inorganic particles
    • World Trade Center
  • Wood burning stoves
  • Moldy environments
  • Occupational exposures
    • US Navy, firefighters, metal workers, buliding supplies, agricultural employment
  • Insecticides
35
Q

Sarcoidosis

Infectious Associations

A
  • Microbial bioaerosols
  • Nontuberculous mycobacterial DNA
  • Ab to Mycobacterium tubeculosis Ag and proteins
  • Propionibacterium RNA
36
Q

Sarcoidosis

Pathophysiology

A
  • Inciting Ag unknown
  • Mφ stimulation w/ CD4+ T-cell activation
  • Predominant CD4+ response w/ variable Th1/Th2 paths ⇒ stimulates fibroblast proliferation ⇒ collagen production ⇒ progressive fibrosis
  • Pulmonary Sarcoid granulomas form
    • Epithelioid giant cells
  • Granulomas ⇒ persist, resolve, fibrosis
37
Q

Sarcoidosis

Histology

A

Non-caseating granulomas which eventual hyalinize.

  • Giant cells
  • Schaumann bodies ⇒ concentric laminations of calcium or proteins
  • Asteroid bodies ⇒ stellate inclusions
38
Q

Sarcoidosis

Clinical Features

A
  • May be asymptomatic ⇒ incidental finding on CXR
  • Systemic sx ⇒ fever, fatigue, night sweats
  • Other specific sx related to organ involvment
  • General course:
    • ⅔ resolve spontaneously
    • ⅓ develop fibrosis w/ varying organ dysfunction
39
Q

Sarcoidosis

Organ Involvement

A

Any organ or tissue may be involved.

  • Common sites
    • Lung
    • Skin
    • Eye
  • Less common
    • Brain and nerves
    • Heart
    • Liver and spleen
    • Bone and joints
    • Kidney
40
Q

Pulmonary Sarcoidosis

Radiographic Stages

A

Stages do not correlate w/ sx, chronicity, or pulmonary function.

41
Q

Pulmonary Sarcoidosis

Symptoms

A

Asymptomatic

Dyspnea

Cough

42
Q

Pulmonary Sarcoidosis

PFTs

A
  • 50% ⇒ restrictive pattern
  • 40% ⇒ combined restrictive and obstructive pattern
  • DLCO ⇒ most sensitive indicator of disease
  • Most have a bronchodilator response
43
Q

Cutaneous Sarcoidosis

Manifestations

A
  • Erythema nodosum
  • Macules, plaques, and papules
  • Lupus pernio
  • Löfgren’s syndrome
44
Q

Erythema Nodosum

A
  • SubQ erythematous nodules
  • Lymphocytic panniculitis of SubQ fat
  • Usually pretibial
  • Painful
  • Usually self-limiting and benign
  • Seen in younger women
45
Q

Sarcoid

Macules, Plaques, and Papules

A
  • Often contain granulomas
  • Locations:
    • Neck, upper back and trunk, extremities
    • Areas of previous scars
    • Tattoos
  • Associated with chronic, poorly responsive disease
46
Q

Lupus Pernio

A
  • Violaceous nodules on nose, cheeks, and lips
  • Associated w/ chronic disease & extrapulmonary involvement
  • Contains granulomas
47
Q

Löfgren’s Syndrome

A
  • Triad of findings
    1. Polyarticular large joint arthritis
    2. Erythema nodosum
    3. Bilateral hilar adenopathy on CXR
  • Acute presentation
  • Often resolves spontaneously or w/ short course of steroids
48
Q

Hepatosplenic

Sarcoidosis

A
  • Occurs in 10% of sarcoid pts
  • Associated with systemic sx
    • Fever, night weats, anorexia, weight loss
  • Labs:
    • ↑ Bilirubin, Alkaline phosphatase
    • Normal transaminases
  • Severe hepatic manifestations ⇒ 1%
    • Portal HTN w/ varices
    • Hepatopulmonary syndrome
    • Cirrhosis and liver failure
49
Q

Cardiac Sarcoid

A
  • Granulomatous inflammation and scarring in LV free wall & septum
    • Septal affects conducting system
  • Presentation:
    • Restrictive cardiomyopathy
      • Dyspnea on exertion, orthopnea, PND
    • Conduction abnormalities
      • Tachyarrhythmias, bradyarrhythmias, LOC
  • Diagnosis:
    • Biopsies often unhelpful
      • Biopsy site RV, involvement is LV
    • Cardiac MRI or PET scan
    • EP study
  • Found in ~ 25% of cases at autopsy
  • Clinically apparent in 5%
50
Q

Renal Sarcoidosis

A
  • Sarcoid Mφ have high levels of 25-OH-Vit D hydroxylase
    • Converts 25-OH Vit D → 1,25-OH Vit D
    • ↑ calcium absorption ⇒ hypercalcemia and hypercalciuria
    • May cause renal calculi and obstruction
  • Renal failure d/t granulomatous nephritis rare
  • Renal disease management
    • Monitor calcium levels yearly w/ active sarcoidosis
    • Check 24 hr urinary calcium excretion
    • ↑ serum or urinary calcium excretion may warrant systemic sarcoid treatment
51
Q

Neurologic Sarcoidosis

A
  • 25% @ autopsy, 10% clinical involvement
  • Manifestations:
    • Cranial-nerve palsies
    • Headache
    • Ataxia
    • Cognitive dysfunction
    • Weakness
    • Seizures
  • Diagnosis:
    • MRI ⇒ periventricular white matter ∆
    • Lumbar puncture ⇒ nonspecific
      • Lymphocytes, Oligoclonal bands, ↑ ACE levels
52
Q

Ocular Sarcoidosis

A
  • Seen in 25% of cases
  • 75% have anterior uveitis (iritis)
    • Acute or chronic
    • Sx: blurred vision, tearing, photophobia
    • May lead to glucoma and vision loss
  • 30% have posterior uveitis
    • Ass. w/ CNS involvement
    • Conjunctiva may show yellow nodules or cysts
    • Lacrimal glands involvement ⇒ dry eyes
  • All sarcoid pts should have yearly eye exams
53
Q

Sarcoidosis

Clinical Evaluation

A
  • History w/ attention to environmental/occupational exposures, fhx
  • CXR
  • PFTs
  • EKG
  • Slit lamp eye exam
  • Biopsy of affected organ
54
Q

Sarcoidosis

Differentials

A
  • Infectious
    • Typical and atypical TB
    • Fungal
    • Brucellosis
  • Rhematologic
    • Juvenile RA
    • Polyangiitis w/ granulomatosis
    • Rheumatoid nodules
  • Lymphoma
  • Hypersensitivity pneumonitis
  • Berylliosis
  • Inflammatory bowel disease
  • Granulomatous hepatitis
  • FB aspiration or IV injection
  • Common variable immunodeficiency
  • Lung CA
55
Q

Sarcoidosis

Treatment Principals

A
  • Dx is not an indication for treatment
  • Most manifestations are asymptomatic and not disabling
  • Many manifestations are self-limiting
  • Consider instituting therapy when organ function is threatened
    • Brain, heart, hypercalcemia
56
Q

Sarcoidosis

Therapy

A
  • Initial therapy
    • Prednisone PO
    • Re-evaluate response at 1-3 months
    • Decrease to lowest effective dose for 9-12 months
  • Failure of steroids
    • Consider immunosuppressive or cytotoxic agents
      • Methotrexate
      • Azathioprine
      • Hydroxychloroquine
      • Infliximab or other anti-TNF agent
57
Q

Hypersensitivity Pneumonitis

Characteristics

A

A granulomatous disease:

Spectrum of predominantly interstitial lung disorders caused by intense and prolonged exposure to inhaled organic dusts:

⇒ Thermophilic bacteria

⇒ True fungi

⇒ Animal protein

⇒ Bacterial products

58
Q

Farmer’s Lung

A

Hypersensitivity pneumonitis caused by:

Harvested, humid hay which grows thermophilic actinomyces

59
Q

Pigeon-Breeder’s Lung

A

Hypersensitivity pneumonitis caused by:

Proteins from bird excreta and feathers

60
Q

Humidifier or Air Conditioner Lung

A

Hypersensitivity pneumonitis caused by:

Thermophilic bacteria in heated water reservoirs

61
Q

Hypersensitivity Pneumonitis Types

Summary

A
62
Q

Hypersensitivity Pneumonitis

Clinical Features

A
  • Acute form
    • Sx appear 4-6 hours after exposure
    • Recurrent attacks of fever, SOB, cough, and leukocytosis
    • Diffuse and nodular infiltrates on CXR
    • PFT w/ restrictive pattern
  • Early in disease course, progression prevented by removal of agent
  • Continued exposure ⇒ chronic form
    • Progressive respiratory failure
    • SOB
    • Cyanosis
    • ↓ TLC and compliance
63
Q

Hypersensitivity Pneumonitis

Histology

A
  • Left ⇒ Interstitial pneumonitis
    • Lymphocytes, Mφ, and plasma cells expand width of alveolar septa
  • Middle ⇒ Interstitial fibrosis w/ inflammatory cells in thickened speta
  • Right ⇒ Granuloma formation
64
Q

Pulmonary Eosinophilia

A

Several diseases w/ eosinophlic infiltration:

  • Acute eosinophilic PNA w/ respiratory failure
  • Simple pulmonary eosinophilia (Loffler syndrome)
  • Tropical eosinophilia 2/2 microfilarial infection
  • Secondary eosinophlia
    • D/t various infections, hypersensitivity pneumonitis, drug allergies, asthma
  • Chronic eosinophilic PNA
65
Q

Pneumoconioses

Characteristics

A

Non-neoplastic lung reaction to inhalation of mineral dusts or other particles or vapors.

  • Agents:
    • Coal dust
    • Silica
    • Asbestos
    • Beryllium
  • Development depends on:
    • Size, shape, and bouyancy of particles
    • Concentration in air
    • Duration of exposure
    • Particle solubility and physiochemical reactivity
    • Additional effects of other irritants
66
Q

Pneumoconioses

Types

A
  • Coal Workers Pneumoconiosis (CWP)
    • Anthracosis
    • Simple CWP
    • Complicated CWP
  • Silicosis
  • Abestosis
  • Berylliosis
67
Q

Anthracosis

A
  • Asymptomatic
  • Carbon pigment in Mφ, CT, lymphoid tissue
68
Q

Simple CWP

A
  • Accumulation of Mφ
  • Little or no pulmonary dysfunction
  • Coal macules & coal nodules
  • Upper lobes and upper zones of lower lobes
69
Q

Complicated CWP

A

“Progressive Massive Fibrosis (PMF) or Black Lung”

  • Extensive fibrosis
  • Pulmonary function compromised
  • Takes many years to develop
  • Multiple large blackened scars: 2-10 cm
  • Pigment and dense collagen
70
Q

Silicosis

Characteristics

A
  • Most prevalent occupational disease in the world
  • Presents after decades of exposure to sandblasting, mining, stone cutting
  • Silica occurs in amorphous and crystalline forms
    • Crystallin form more fibrogenic
71
Q

Silicosis

Histology

A
  • Tiny, barely palpable nodules initially
    • Pale to black depending on ± coal dust
  • Coalesce ⇒ hard collagenous scars containing silica particles
  • May undergo central softening ⇒ superimposed TB or ischemia
  • Eggshell calcification
  • Intervening parenchyma compressed or over-expanded ⇒ honeycomb pattern
  • Can end as Progressive Massive Fibrosis
72
Q

Abestos

A

Two distinct geometric forms:

  • Serpentine ⇒ curly and flexible fibers
    • Chrysolite ⇒ used in industry
  • Amphibole ⇒ straight, stiff, brittle fibers
    • Crocidolite, amosite, tremolite, anthophyllite, actinolyte
    • Less prevalent but more pathogenic
      • Esp. mesotheliomas
73
Q

Asbestosis

Clinical Manifestations

A

Can show many different clinical pictures:

  • Pleural plaques
  • Pleural effusions
  • Parenchymal intersitial fibrosis (aka asbestosis)
  • Mesothelioma
  • Bronchogenic carcinoma
  • Laryngeal and other extrapulmonary neoplasms
74
Q

Asbestos

Gross Effects

A
  • Affects lower lobes and subpleural areas first
  • Middle & upper lobes affected as fibrosis progresses
  • Visceral pleura ⇒ fibrous thickening
  • Parietal pleura ⇒ pleural plaques
    • Made of dense collagen and calcium
75
Q

Asbestosis

Pathogenesis

A

“Parenchymal Interstitial Fibrosis”

  1. Fibrosis of respiratory bronchioles & alveolar ducts
  2. Fibrosis of alveolar sacs and alveoli
  3. Diffuse pulmonary interstitial fibrosis
  4. Honeycomb lung
76
Q

Abestosis

Histology

A

Asbestos bodies ⇒ fusiform beaded rods w/ translucent center

Ferruginous body ⇒ asbestos body coated with iron-containing proteinaceous material

77
Q

Coal vs Silica vs Asbestos

A
78
Q

Berylliosis

A
  • Aerospace and nuclear industries
  • Acute exposure ⇒ almost non-existent, regulated
  • Chronic exposure ⇒ non-caseating pulmonary granulomas with hilar lymphadenopathy
    • Less common in spleen and liver
  • Caused by induction of cell-mediated immunity
  • Heavy exposure linked to lung cancer

2. HDS Pulmonary (28 decks)

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