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
Q

asbestos-related disease clinical variations

A

• 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
Q

does asbestos-related disease increase the risk of a patient developing TB?

A

• No increased risk for TB

27
Q

complications of asbestos-related disease

A

• Complications:
– Cor pulmonale
– Caplan syndrome

28
Q

causes of Berylliosis

A

• Beryllium – Nuclear and airspace industry

29
Q

findings of Berylliosis

A

• Granulomatous inflammation – TB and sarcoidosis (differential diagnosis)

30
Q

complications of Berylliosis

A

• Complications – cor pulmonale and lung cancer

31
Q

what is Sarcoidosis?

A

• Multisystem granulomatous disease of unknown etiology

32
Q

how prevalence is sarcoidosis?

A
  • 25% cases of chronic interstitial lung disease

* World wide occurrence; highest Scandinavia

33
Q

who does sarcoidosis effect most often?

A
  • AA: Whites – 10:1
  • M: F – 1:2
  • 70% < 40years
  • Non-smokers
34
Q

cause of Sarcoidosis

A
  • 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
Q

tissue involved in patients with sarcoidosis

A

lung, lymph nodes, spleen, liver, skin, eyes, joints, nervous system, myocardium

36
Q

sarcoidosis manifestations in the skin

A
  • Nodular granulomatous lesions
  • Lupus pernio
  • Erythema nodosum
37
Q

sarcoidosis manifestations in the eye

A

• Uveitis

38
Q

sarcoidosis manifestations in the liver

A

• Granulomatous hepatitis

39
Q

other manifestations of sarcoidosis

A
  • Enlarged salivary and lacrimal glands
  • Diabetes insipidus
  • Bone marrow and splenic involvement
40
Q

Lab and radiologic findings of sarcoidosis

A

– ↑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
Q

CXR findings of sarcoidosis

A
  • Bilateral hilar adenopathy

* Reticulonodular shadows in lungs

42
Q

prognosis of sarcoidosis

A

– Variable – spontaneous remissions and relapses

– Progressive interstitial fibrosis with cor pulmonale and death in 10 – 15% of cases

43
Q

causes of Hypersensitivity pneumonitis

A

• Inhaled antigen (known or unknown) producing granulomatous interstitial pneumonitis (extrinsic allergic alveolitis)

44
Q

what type hypersensitivity reaction is hypersensitivity pneumonitis?

A

• Type III hypersensitivity reaction

45
Q

how does the hypersensitivity pneumonitis reaction occur?

A

– 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
Q

important types of hypersensitivity pneumonitis

A

– 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
Q

clinical syndromes of hypersensitivity pneumonitis?

A

Acute, subacute, chronic

• Interstitial and alveolar infiltrates of inflammatory cells, peri-bronchiolar accentuation, ill-defined granulomas

48
Q

Clinico-pathologic diagnosis od hypersensitivity pneumonitis

A

symptoms and physical findings, x-ray abnormalities, PFTs, immunologic features (Abs to suspected Ags); lung biopsy may be needed

49
Q

prevalence of Idiopathic pulmonary fibrosis

A

• 15% of cases of chronic interstitial lung disease

50
Q

what is Idiopathic pulmonary fibrosis

A
  • Broad and all encompassing term including a number of types of Idiopathic interstitial pneumonias
  • Usual interstitial pneumonia ~ Idiopathic interstitial fibrosis
51
Q

who is affected most often by idiopathic pulmonary fibrosis?

A
  • M> F

* 40 – 70 years

52
Q

what are the clinical symptoms of idiopathic pulmonary fibrosis?

A

• Average duration of symptoms – 18 – 24 months
• Clinical manifestations – Dyspnea; non-specific constitutional symptoms
such as fever, weight loss, fatigue, arthralgias; cough

53
Q

pathogenesis of idiopathic pulmonary fibrosis

A

• 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
Q

course of idiopathic pulmonary fibrosis

A

• Course – Progressive disease – end-stage lung; cor pulmonale.

55
Q

prevalence of Collagen vascular disease

A

• 10% cases of interstitial lung disease

56
Q

SLE and interstitial lung disease

A

• 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
Q

RA and interstitial lung disease

A

• Rheumatoid arthritis (RA)
– Wide spectrum of pulmonary changes
• Rheumatoid nodules (when associated with pneumoconiosis – Caplan syndrome)
• Interstitial fibrosis
• Pleural effusions

58
Q

Systemic sclerosis (scleroderma) and interstitial lung disease

A

• Systemic sclerosis (scleroderma)
– Interstitial fibrosis with pulmonary vascular hypertrophy
– Commonest cause of death