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Interstitial Lung Diseases Flashcards

1
Q

what is interstitial lung disease?

A

– Lung parenchymal disorders with common clinical, radiologic, physiologic and pathologic features; hallmark – Involvement of interstitium

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2
Q

synonyms of interstitial lung disease?

A

• Infiltrative lung disease -Infiltration of cellular and non-cellular
elements within alveolar septa and alveoli.
• Diffuse parenchymal lung disease (DPLD)
• Restrictive lung disease – Characterized by reduced total lung capacity in presence of a normal or reduced expiratory flow rate.

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3
Q

types of interstitial lung disease

A

• Acute interstitial lung disease – Acute interstitial pneumonia (ARDS)
• Chronic interstitial lung disease
– Fibrosing lung disorders (pneumoconioses)
– Granulomatous disorders (sarcoidosis)
– Idiopathic interstitial pneumonias (IIPs)

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4
Q

pathogenesis of “alveolitis”?

A

– Damage to pneumocytes and endothelial cells
• Leads to leukocytes releasing cytokines which mediate and stimulate interstitial fibrosis
• Interstitial fibrosis –
– ↓es lung compliance and elasticity
• ↓ed lung expansion during inspiration

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5
Q

clinical laboratory findings of interstitial lung disease

A
  • Dry cough and dyspnea
  • Late inspiratory crackles, bibasilar (Velcro crackles)
  • Cor pulmonale
  • Chest radiography – bilateral reticulonodular infiltrates
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6
Q

Pneumoconioses definition

A

Non- neoplastic lung diseases in response to inhalation of mineral dusts inhaled in the workplace.
• Now expanded to include diseases induced by organic and inorganic particulate matter/chemical fumes /vapors.
• Coal dust, silica, asbestos, beryllium

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7
Q

what percent of interstitial lung disease does pneumoconiosis cause?

A

• 25% cases of chronic interstitial lung disease

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8
Q

what does the development of pneumoconiosis depend upon?

A

• The amount of dust retained in the lung parenchyma and airways
• The size, shape and buoyancy of particles
– 1 – 5 μm – reach bifurcation of respiratory bronchioles and alveolar ducts
– < 0.5 μm – reach alveoli and are phagocytosed by alveolar macrophages (much worse!!)
• Particle solubility and physicochemical reactivity
• Possible additional effects of other irritants (e.g., tobacco smoking)

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9
Q

causes of fibrogenic pneumoconiosis

A
  • coal worker’s pneumoconiosis (carbonaceous dust)
  • silicosis (crystalline silica)
  • silicatosis (talc, kaolin, fedspar, mica, muscovite)
  • asbestosis (asbestos fibers)
  • rare forms:
    • metalloconiosis
      • berylliosis (beryllium dust)
      • hard metal lung disease (tungsten carbide)
      • aluminosis (aluminum)
    • thesaurosis (hair spray)
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10
Q

types of Coal workers’ pneumoconiosis (CWP)

A
  • Anthracotic pigment – coal mines, urban centers, tobacco smoke
  • Pulmonary anthracosis
  • Simple CWP
  • Complicated CWP (progressive massive fibrosis)
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11
Q

Anthracosis findings

A
  • Asymptomatic

* Anthracotic pigment in interstitial compartment and lymph nodes

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12
Q

findings of Simple coal workers’ pneumoconiosis

A
  • Fibrous opacities < 1 cm
  • Upper lobes and upper portions of lower lobes
  • Characterized by coal dust deposits adjacent to respiratory bronchioles
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13
Q

findings of Complicated coal workers’ pneumoconiosis (progressive massive fibrosis)

A 
• Fibrous opacities > 1 cm
• With or without central necrosis
• Massive fibrosis – crippling lung disease (Black lung disease)
• Caplan syndrome – CWP with rheumatoid
nodules in lung
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14
Q

complication of Complicated coal workers’ pneumoconiosis (progressive massive fibrosis)

A

• Complication – Cor pulmonale

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15
Q

does Complicated coal workers’ pneumoconiosis (progressive massive fibrosis) increase the risk of a patient developing TB or cancer?

A

• No increased incidence of TB or cancer.

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16
Q

prevalence of silicosis

A

• Most common occupational disease worldwide

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17
Q

cause of silicosis

A

• Crystalline silicon dioxide (quartz)
– Foundries (metal casting), sandblasting, silica mines
• Quartz activates alveolar macrophages after engulfment → cytokine release → fibrogenesis

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18
Q

what do you get from chronic exposure to silicosis?

A

– Nodular opacities with concentric layers of collagen
– Polarizable quartz particles can be seen
– “Egg-shell” calcification in hilar lymph nodes

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19
Q

complications of silicosis?

A

• Complications – cor pulmonale; association with Caplan syndrome

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20
Q

does silicosis increase the risk of a patient developing TB or cancer?

A

yes,

• Increased risk for TB (silicotuberculosis) and cancer

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21
Q

forms of asbestos

A

– Serpentine (e.g.–chrysotile)–curlyandflexible

– Amphibole (e.g. – crocidolite) – straight and rigid

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22
Q

deposition sites of asbestos-related disease

A

• Deposition sites – respiratory bronchioles, alveolar ducts and alveoli

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23
Q

sources of asbestos-related disease

A

• Sources –
– Insulation around pipes in old naval ships
– Roofing material used over 20 years ago
– Demolition of old buildings

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24
Q

tissue appearance of asbestos-related disease

A

• Tissue appearance –

– Ferruginousbodies–macrophages phagocytose asbestos fibers and coat them with ferritin (iron and protein)

25
asbestos-related disease clinical variations
• Benign pleural plaques (KEY findings!!) – Not a precursor of mesothelioma • Diffuse interstitial fibrosis (asbestosis) • Bronchogenic carcinoma – Additional risk with smoking – 20 years after first exposure • Mesothelioma – No relationship to smoking – Arises from lining mesothelial cells of pleura – 25-40 years after first exposure
26
does asbestos-related disease increase the risk of a patient developing TB?
• No increased risk for TB
27
complications of asbestos-related disease
• Complications: – Cor pulmonale – Caplan syndrome
28
causes of Berylliosis
• Beryllium – Nuclear and airspace industry
29
findings of Berylliosis
• Granulomatous inflammation – TB and sarcoidosis (differential diagnosis)
30
complications of Berylliosis
• Complications – cor pulmonale and lung cancer
31
what is Sarcoidosis?
• Multisystem granulomatous disease of unknown etiology
32
how prevalence is sarcoidosis?
* 25% cases of chronic interstitial lung disease | * World wide occurrence; highest Scandinavia
33
who does sarcoidosis effect most often?
* AA: Whites – 10:1 * M: F – 1:2 * 70% < 40years * Non-smokers
34
cause of Sarcoidosis
* Disorder of immune regulation * Unknown antigen → Interaction with CD4 TH cells → cytokine release → recruitment of monocytes/histiocytes → non-necrotizing granuloma formation * Diagnosis of exclusion
35
tissue involved in patients with sarcoidosis
lung, lymph nodes, spleen, liver, skin, eyes, joints, nervous system, myocardium
36
sarcoidosis manifestations in the skin
* Nodular granulomatous lesions * Lupus pernio * Erythema nodosum
37
sarcoidosis manifestations in the eye
• Uveitis
38
sarcoidosis manifestations in the liver
• Granulomatous hepatitis
39
other manifestations of sarcoidosis
* Enlarged salivary and lacrimal glands * Diabetes insipidus * Bone marrow and splenic involvement
40
Lab and radiologic findings of sarcoidosis
– ↑ed angiotensin converting enzyme (ACE) levels • Marker of disease activity and response to steroids – Hypercalcemia (5% cases) – Polyclonal gammopathy – Cutaneous anergy (lack of response to common skin antigens (candida) due to consumption of CD4 TH cells
41
CXR findings of sarcoidosis
* Bilateral hilar adenopathy | * Reticulonodular shadows in lungs
42
prognosis of sarcoidosis
– Variable – spontaneous remissions and relapses | – Progressive interstitial fibrosis with cor pulmonale and death in 10 – 15% of cases
43
causes of Hypersensitivity pneumonitis
• Inhaled antigen (known or unknown) producing granulomatous interstitial pneumonitis (extrinsic allergic alveolitis)
44
what type hypersensitivity reaction is hypersensitivity pneumonitis?
• Type III hypersensitivity reaction
45
how does the hypersensitivity pneumonitis reaction occur?
– First exposure – • IgG antibodies in serum – Second exposure – • Antibodies combine with inhaled antigens to form immune complexes → inflammatory response in lung (interstitial) – Chronic exposure – • Granuloma formation (Type IV hypersensitivity response)
46
important types of hypersensitivity pneumonitis
– Farmers’ lung – moldy hay – thermophilic actinomycetes bacteria – saccharopolyspora rectivirgula – Silo fillers’ disease • Inhalation of gases from plant material (oxides of nitrogen) – Byssinosis • Cotton, linen, hemp • Textile factory workers • “Monday morning blues”
47
clinical syndromes of hypersensitivity pneumonitis?
Acute, subacute, chronic | • Interstitial and alveolar infiltrates of inflammatory cells, peri-bronchiolar accentuation, ill-defined granulomas
48
Clinico-pathologic diagnosis od hypersensitivity pneumonitis
symptoms and physical findings, x-ray abnormalities, PFTs, immunologic features (Abs to suspected Ags); lung biopsy may be needed
49
prevalence of Idiopathic pulmonary fibrosis
• 15% of cases of chronic interstitial lung disease
50
what is Idiopathic pulmonary fibrosis
* Broad and all encompassing term including a number of types of Idiopathic interstitial pneumonias * Usual interstitial pneumonia ~ Idiopathic interstitial fibrosis
51
who is affected most often by idiopathic pulmonary fibrosis?
* M> F | * 40 – 70 years
52
what are the clinical symptoms of idiopathic pulmonary fibrosis?
• Average duration of symptoms – 18 – 24 months • Clinical manifestations – Dyspnea; non-specific constitutional symptoms such as fever, weight loss, fatigue, arthralgias; cough
53
pathogenesis of idiopathic pulmonary fibrosis
• Repeated injury to the lung → alveolitis → cytokine release → interstitial fibrosis • Interstitial fibrosis → irregular dilatation of adjacent airways → honeycomb lung (end stage interstitial fibrosis) (KEY finding!!) -normal and "abnormal" areas give rise to a "patchwork" appearance
54
course of idiopathic pulmonary fibrosis
• Course – Progressive disease – end-stage lung; cor pulmonale.
55
prevalence of Collagen vascular disease
• 10% cases of interstitial lung disease
56
SLE and interstitial lung disease
• Systemic lupus erythematosus (SLE) – Interstitial lung disease in 50% of patients – Wide spectrum of pulmonary changes; common manifestation – pleural effusion (unexplained pleural effusion in a young woman likely to be SLE).
57
RA and interstitial lung disease
• Rheumatoid arthritis (RA) – Wide spectrum of pulmonary changes • Rheumatoid nodules (when associated with pneumoconiosis – Caplan syndrome) • Interstitial fibrosis • Pleural effusions
58
Systemic sclerosis (scleroderma) and interstitial lung disease
• Systemic sclerosis (scleroderma) – Interstitial fibrosis with pulmonary vascular hypertrophy – Commonest cause of death

Renal/Respiratory (8 decks)

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