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MED234 Respiratory > Resp Path 3 - RESTRICTIVE DZ (Fibrosing Dz, Granulomatous Dz), Smoking related interstital dz, Other interstitial dz - Galbraith > Flashcards

Resp Path 3 - RESTRICTIVE DZ (Fibrosing Dz, Granulomatous Dz), Smoking related interstital dz, Other interstitial dz - Galbraith Flashcards

1
Q

What characterizes chronic diffuse interstitial diseases?

A

Inflammation and pulmonary interstitial tissue fibrosis, particularly involving the alveolar walls.

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2
Q

What clinical and functional changes constitute restrictive lung disease due to interstitial fibrosis?

A

Decreased of diffusion lung capacity, volumes, and compliance without evidence of airway obstruction.

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3
Q

Long term sequelae of restrictive lung disease.

End stage:

A

Pulmonary HTN and cor pulmonale.

End stage: parenchymal destruction and scarring

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4
Q

What is honey-comb lung?

A

Indicative of end stage restrictive lung disease. Parenchymal destruction and scarring.

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5
Q

Histological and clinical features of chronic diffuse interstitial diseases.

A

(1) Fibrosing
(2) Granulomatous
Esoinophilic
(3) Smoking-related

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6
Q

What fibrosing disease is a disorder of unknown cause, but characterized by PROGRESSIVE PULMONARY INTERSTITIAL FIBROSIS?

A

Idiopathic Pulmonary Fibrosis

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7
Q

Postulated pathogenesis of Idiopathic Pulmonary Fibrosis

A

REPEATED CYCLES of epithelial activation/injury, causing abnormal “wound healing” resulting in EXCESSIVE FIBROBLAST PROLIFERATION (driven by TGF-B1).

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8
Q

Sources of injury to epithelium in Idiopathic Pulmonary Fibrosis (5)

A 
Tobacco smoke
Occupational irritants
Viruses
Persistent gastric reflux
Genetic: telomerase mutations (TERT, TERC)
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9
Q

What three disorders have a pathologic morphologic pattern of fibrosis denoted “Unusual Interstitial Pneumonia” (UIP)?

A

Connective tissue disorders
Chronic hypersensitivity pneumonia
Asbestosis

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10
Q

Describe UIP morphology

A
  • SUBPLEURAL AND INTERLOBULAR septal fibrotic distribution (around alveolar edges, works inward), as well as lower lobe predominance.
  • PATCHY INTERSTITIAL FIBROSIS with VARIABLE AGE
  • Honeycomb fibrosis/lung = due to destruction of alveolar architecture.
  • NEW FIBROBLASTIC FOCI mixed with more densely fibrotic areas
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11
Q

Characteristics of honeycomb fibrosis

A

dense fibrosis and cystic spaces lined by hyperplastic type II pneumocytes (tall cells) or bronchiolar epithelium

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12
Q

Fibroblastic Foci are indicative of what stage of what lesion?

A

EARLY lesion of idiopathic pulmonary fibrosis

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13
Q

Clinical course of Idiopathic Pulmonary Fibrosis.

A

> 50yo initially presents with DOE, dry cough. Progresses to hypoxemia, cyanosis, clubbing.

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14
Q

Median survival of Idiopathic Pulmonary Fibrosis.

Therapy.

A

3 years

Lung transplant only definitive tx.

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15
Q

What develops as EITHER interstitial chronic inflammation OR interstitial fibrosis, NO heterogeneity.

A

Nonspecific interstitial Pneumonia

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16
Q

The 2 common histologic patterns seen in Nonspecific Interstitial Pneumonia (NSIP)

A
  1. CELLULAR&raquo_space; Moderate chronic interstitial inflammation.

2. FIBROSING&raquo_space; Interstitial fibrosis - more homogenous in age than UIP.

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17
Q

Demographic and clinical course associated with Nonspecific interstitial Pneumonia.

A
  • Females in 50s.
  • No smoking association
  • Presents with dyspnea and cough.
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18
Q

Loose fibrous tissue balls that fill terminal bronchioles, alveolar ducts, and alveolar spaces.
Think what?

A

Cryptogenic Organizing Pneumonia (COP).

Masson bodies

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19
Q

COP presentation and treatment/recovery.

A
  • Present with cough/dyspnea.

- Most require steroid therapy, but can recover spontaneously.

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20
Q

Interstitial fibrosis in COP?

A

No

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21
Q

Pulmonary involvement patterns in CT diseases (i.e. SLE, RA, scleroderma)

A

NSIP, UIP, vascular sclerosis, organizing pneumonia, bronchiolitis.
Better prognosis with idiopathic UIP.

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22
Q

What is a group of non-tumor lung diseases in a setting of INHALATION exposure to mineral dusts, inorganic and organic particles, and chemical fumes?

A

Pneumoconiosis

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23
Q

What four factors determent particle pathogenicity in pneumoconiosis?

A
  • Particle size (**1-5 micrometers most dangerous&raquo_space; can reach terminal alveoli)
  • Particle solubility
  • Level and duration of exposure/effectiveness of clearance.
  • Intensity of immune response (can cause fibroses)
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24
Q

Two types of pneumoconiosis.

A
  1. Coal Workers’ Pneumoconiosis (CWP)
  2. Silicosis
  3. Asbestosis
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25
Q

Carbon dust causes…

A
  • Anthracosis to
  • CWP (w/o significant pulmonary dysfunction) to
  • Complicated-CWP to
  • Progressive Massive Fibrosis (PMF)
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26
Q

Describe anthracosis. Histo and demographic.

A

BLACK pigmented lesions formed by inhaled carbon that’s taken up by alveolar and interstitial MACROPHAGES. Accumulate in LYMPHATICS an lymphoid tissues.
- Seen in CWP, smokers, urban dwellers.

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27
Q

Describe simple Coal Workers’ Pneumoconiosis

A
  • Little functional decrement
  • 1-2mm COAL MACULES composed of dust-filled macrophages; slightly larger coal nodules also contain collagenous networks.
  • Located primarily adjacent to RESPIRATORYBRONCHIOLES
28
Q

Describe Progressive Massive Fibrosis (PMF)

A

(aka - complicated CWP)
- Develops in less than 10%.
Multiple anthracotic scars, which may lead to:
- Respiratory failure, pulmonary HTN, cor pulmonale

29
Q

Describe silicosis

A
  • Due to prolonged inhalation of silica crystals. Ingested by macrophages activates MACROPHAGES&raquo_space; causing fibrosis and COLLAGEN deposition.
  • Results in chronic, progressive nodular fibrosis in HILAR LN and APEX
30
Q

Calcified hilar LN on xray, think what?

31
Q
  • Polarized light show BIREFRINGENT silica particles.

- See hyalinized PINK WHORLS OF COLLAGEN balls with scant inflammation, surrounded by macrophages

32
Q

What part of the lung does silicosis typically start?

33
Q

At what stage of silicosis is dyspnea typically present? Describe the disease’s progression.

A
  • Progressive massive fibrosis occurs and then dyspnea.

- Disease can progress after exposure ends

34
Q

What two potential silicosis sequelae diseases that have increased risk.

A

Increased risk for TB, lung cancer

35
Q

Describe “asbestos”

A

Group of fibrous hydrated silicate crystals that cause interstitial and pleural fibrosis.

36
Q

What two cancers are linked with asbestos?

A

Lung carcinoma and malignant mesothelioma.

especially with smoking

37
Q

Name the two forms of asbestos and their pathogenicity.

A

1) Amphiboles - more pathogentic, reach deep lung more readily than serpentine
2) Serpentine fibers - curved, less pathogenic, more soluble

38
Q

Pathogenesis of asbestos-related lung disease. Histo and what part of lung affected first?

A
  • Macrophages ingest particles&raquo_space; activation of mediators. SEE ASBESTOS BODIES (or ferruginous) WITHIN MACROPHAGES.
  • BASE affected first.
39
Q

Two morphologic features of asbestos

A
  1. Pleural plaques - well circumscribed (collagen) that don’t contain asbestos bodies. Asymptomatic.
  2. Asbestosis - diffuse interstitial fibrosis, indistinguishable form UIP, EXCEPT for PRESENCE OF ASBESTOS BODIES.
40
Q

Clinical course of asbestos-related lung disease.

A
  • Decades to appear. s/s first dyspnea, then productive cough.
  • Can result in pleural effusion
  • POOR prognosis for asbestosis and lung/pleural malignancy
41
Q

Name complications of therapies that can lead to restrictive diseases.

A
  1. Drug induced lung diseases: ASPIRIN=acute bronchospasm; AMIODARONE=pneumonitis; BLEOMYCIN=fibrosis
  2. Radiation induced lung diseases - with acute radiation pneumonitis 1-6mo post-therapy&raquo_space; can progress to&raquo_space; chronic radiation pneumonitis (pulmonary fibrosis)
42
Q

What disease is characterized by:

  • noncaseating granulomas in all tissues with multinucleated giant cells.
  • 90% having hilar LN or lung involvement.
  • unknown cause
A

Sarcoidosis

A dx of exclusion

43
Q

Sarcoidosis, think what race/sex?

A

African Americans! Females.

44
Q

Sarcoidosis caused by what possible disordered immune responses in genetically predisposed peoples?

A
  • HLA-A1 and HLA-B8 associated genotypes
  • Accumulation of CD4+ T-cells
  • Increased Th1 cytokine production that causes macrophage activation and T-cell expansion
  • Inc macrophage TNF production, leading ot granuloma formation.
  • Polyclonal hypergammaglobulinemia
45
Q

Sarcoidosis morphology in the:

  • Lungs
  • LN
  • Spleen, liver
  • Bone marrow
  • Skin
  • Eyes
A
  • Lungs - scattered granulomas that may for a grossly apparent reticulonodular pattern on x-ray
  • LN - hilar and mediastinal regions
  • Spleen, liver - HSM
  • Bone marrow - often phalangeal predilection in 20% of sarcoidosis cases
  • Skin - up to 50% have subcutaneous nodules or erythematous scaling plaques
  • Eyes - up to 50% have iritis, iridocyclitis, or choroid retinits. Possible concurrent salivary gland involvement
46
Q

What is mukilcz syndrome?

A

Concurrent salivary gland involvement with eye involvement in sarcodisosis

47
Q

Where are grossly apparent (2cm) granulomatous nodules found in Sarcoidosis?

A

lungs, distributed along pulmonary lymphatics.

48
Q

Constitutional and pulmonary s/s in sarcoidosis.

A

Variable, but most with insidious onset of respiratory difficulty (dyspnea, hemoptysis, fatigue, night sweats) or constitutional symptoms (fever, night sweat, weight loss).

49
Q

While sarcoidosis may spontaneously resolve, what are possible residua?

A
  • 20% have residual pulmonary impairment
  • 10% develop pulmonary fibrosis, P-HTN, and cor pulmonale
    (70% have no residua)
50
Q

How is sarcoidosis dx?, explain

A
  • DIAGNOSIS OF EXCLUSION.
  • Must establish biopsy of NONCASEATING “naked” GRANULOMAS
  • Rule out TB, fungal infections - anything with the same histology
51
Q

What is this:
A spectrum of immunologically mediated interstitial disorders caused by inhaled dusts or antigens. Primarily affects the alveoli.

A

Hypersensitivity Pneumonitis, “allergic alveolitis”

52
Q

Name three possible sources of antigen in Hypersensitivity Pneumonitis.

A
  1. Farmer’s Lung - actinomycete spore in hay (spore forming fungi/bacteria)
  2. Pigeon breeder’s lung - proteins form bird feathers or exretions
  3. Air-conditioner lung or humidifier lung - thermophilic bacteria (heated water reservoirs)
53
Q

In Hypersensitivity Pneumonitis, what three things does the immunologic reaction to inhaled organic antigens cause?

A

1) Interstitial pneumonitis (WBCs - lymphocytes, plasma cells (macrophages) in bronchioles
2) Noncaseating granulomas in bronchioles
3) Interstitial fibrosis

54
Q

Clinical manifestation of Hypersensitivity Pneumonitis.

A
  • s/s - cough, dyspnea, fever, diffuse and nodular radiographic densities.
  • cessation of exposure may prevent progression
55
Q

Name five different types of pulmonary eosinophilias.

A

All characterized by interstitial or alveolar eosinophil infiltrates.

  1. Acute eosinophilic penumonia with respiratory failrue
  2. Simple pulmonary eosinophilia (Loeffler syndrome)
  3. Tropical eosinophilia (cause: microfilarieae)
  4. Secondary eosinophilia (induced by infection)
  5. Idiopathic chronic eosinophilic pneumonial
56
Q

What is this?

A 30s-40s yo (M=F) presents with insidious onset of dry cough, dyspnea, and clubbing, often with EMPHYSEMA.

A

Desquamative Interstitial Pneumonia - SMOKING RELATED INTERSTITIAL DISEASE

57
Q

Abundant DUSTY BROWN (smokers’) MACROPHAGES in alveolar spaces.

  • MINIMAL Fibrosis
  • Smoker
A

Histology of Desquamative Interstitial Pneumonia

58
Q

Tx of Desquamative Interstitial Pneumonia

A

steroids, stop smoking

59
Q

What is this:

Peribronchiolar inflammation and mild fibrosis with bronchiolar accumulations of smokers’ macrophages.

A

Respiratory bronchiolitis-associated interstitial lung disease - SMOKING RELATED INTERSTITIAL DISEASE

60
Q

S/s and Tx for Respiratory bronchiolitis-associated interstitial lung disease

A

s/s - cough and dyspnea in a long term smoker

tx - smoking cessation

61
Q

Accumulations of dendritic cells (Langerhans cells) into NODULES, w/ fibroblasts, macrophages, and many eosinophils
- Lesions are due to reactive processes or BRAF mutations

A

Pulmonary Langerhans Cell Histiocytosis

62
Q

Demographic of Pulmonary Langerhans Cell Histiocytosis.

A

young smokers

63
Q

What physically happens in Pulmonary Alveolar Proteinosis?

A

Accumulation of surfactant in alveoli and bronchioles, secondary to diminished pulmonary macrophage activity.

64
Q

People with PAP present how - clinically and histologically?

A
  • Clinically - coughing up sputum and gelatinous material

- Histo - alveoli filled with granular pink precipitate (lipid-laden, PAS-positive Schiff)

65
Q

Progression and tx for PAP

A
  • May have progressive respiratory failure.

- Tx - whole lung lavage, GM-CSF

66
Q

Three different causes of PAP

A
  1. Acquired PAP - 90%. Autoantibodies CM-CSF
  2. Congenital PAP - in newborns and rapidly fatal.
  3. Secondary PAP - follows exposure to dusts or chemicals OR in immunocompromised.

MED234 Respiratory (15 decks)

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