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Pulmonology Exam > Pathology of Restrictive Lung Diseases > Flashcards

Pathology of Restrictive Lung Diseases Flashcards

1
Q

restrictive lung diseases - general principles

A

*disorders characterized by inflammation and fibrosis of lung interstitium
*stiff lungs → reduced diffusion capacity, lung volume, lung compliance
*damage to alveolar epithelium and interstitial vasculature
*PFTs: decreased TLC, normal FEV1/FVC

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2
Q

major restrictive lung diseases

A
  1. idiopathic pulmonary fibrosis (IPF)
  2. sarcoidosis
  3. hypersensitivity pneumonitis
  4. pneumoconioses
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3
Q

clinical features of restrictive lung diseases

A

*dyspnea, tachypnea, end-inspiratory crackles
*usually no wheezing or other evidence of airway obstruction
*eventual cyanosis
*eventually, secondary pulmonary hypertension → cor pulmonale

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4
Q

“honeycombing” of lungs on CT scan

A
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5
Q

idiopathic pulmonary fibrosis (IPF) - overview

A

*clinicopathologic syndrome of unknown etiology marked by PROGRESSIVE INTERSTITIAL FIBROSIS and respiratory failure

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6
Q

idiopathic pulmonary fibrosis (IPF) - pathogenesis

A

*? repeated injury and defective repair of alveolar epithelium, leading to interstitial fibrosis
*may involve multiple cycles of lung injury, inflammation, and fibrosis
*associated with tobacco smoking, environmental pollutants, and genetic defects

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7
Q

idiopathic pulmonary fibrosis (IPF) - genetic alterations

A

*telomerase loss-of-function mutations
*mucin (MUC5B) altered production
*surfactant germline mutations

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8
Q

idiopathic pulmonary fibrosis (IPF) - gross morphology

A

*“cobblestoned” pleural surface
*firm/rubbery white fibrosis

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9
Q

idiopathic pulmonary fibrosis (IPF) - microscopic morphology

A

*patchy fibrosis
*replacement of elastin with collagen
*collapse of alveolar walls and formation of cystic spaces:
-lined by hyperplastic epithelium
-secondary pulmonary hypertension vascular changes

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10
Q

idiopathic pulmonary fibrosis (IPF) - diagnostic criteria

A
  1. chronic, progressive fibrotic usual interstitial pneumonia
  2. limited to the lung
  3. occurring primarily in older (>50) adults (male)
  4. chronic exertional dyspnea
  5. unknown origin
    *diagnosis has substantial therapeutic/prognostic implications
    *requires a multidisciplinary approach to rule out many other diseases
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11
Q

which pathologic changes explain the functional differences between idiopathic pulmonary fibrosis (IPF) and emphysema

A

*IPF: increased fibrosis
-causes rigid, dilated airspaces restricting lung expansion

*emphysema: decreased elastic fibers
-alveolar hyperexpansion due to air trapping caused by collapse of small airways upon expiration, producing obstruction

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12
Q

sarcoidosis - overview

A

*systematic disease of unknown etiology characterized by NONCASEATING GRANULOMATOUS INFLAMMATION

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13
Q

sarcoidosis - epidemiology

A

*predilection for adults younger than 40 years of age
*familial and racial clustering suggest involvement of genetic factors (incidence relatively high in Danish, Swedish, African descent)

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14
Q

sarcoidosis - pathogenesis

A

*sustained CD4+ Th1 T-cell response to unknown antigen
*local recruitment/activation of macrophages, forming NONCASEATING GRANULOMAS

*pathogenesis of pulmonary fibrosis uncertain:
-? shift from Th1 to Th2 cell cytokines
-activation of pulmonary macrophages, leading to activation of lung fibroblasts

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15
Q

sarcoidosis - gross morphology

A

*HILAR ADENOPATHY
*parenchymal scarring and honeycomb

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16
Q

sarcoidosis - microscopic morphology

A

*NON-NECROTIZING GRANULOMA:
-at sites of active disease
-discrete, compact epitheloid macrophages
-rimmed by a zone rich in CD4+ T cells

*admixed mutlinucleated giant cells:
-SCHAUMANN BODIES - laminated calcifications
-ASTEROID BODIES - stellate inclusions

*surrounded by ring of fibroblasts:
-produce collagen replacing granuloma with hyalinized scar

17
Q

sarcoidosis - Schaumann bodies

A

*laminated calcifications

18
Q

sarcoidosis - asteroid bodies

A

*stellate inclusions
*pathognomonic for sarcoidosis

19
Q

sarcoidosis - clinical features

A

*may be asymptomatic (incidental discovery on routine chest films)
*gradual appearance of respiratory and constitutional symptoms: dyspnea, cough, fever, fatigue, weight loss, anorexia, night sweats
*multi-systemic: bilateral hilar lymphadenopathy and lung involvement; sometimes skin (lupus pernio, erythema nodosum) or bone marrow involvement

20
Q

sarcoidosis - diagnosis

A

*diagnosis of exclusion
*no definitive diagnostic test
*exclude other disorders with similar presentations (TB must be excluded)
*establish consistent clinical and radiologic findings

21
Q

hypersensitivity pneumonitis - overview

A

*immunologically mediated interstitial lung disease caused by intense, prolonged exposure to INHALED ORGANIC ANTIGENS
*occupational inhalation of organic dust containing antigens from thermophilic bacteria, fungi, animal proteins, or bacterial products
*examples include Farmer’s lung, Pigeon breeder’s lung

22
Q

hypersensitivity pneumonitis - pathogenesis

A

*abnormal sensitivity or heightened reactivity to causative antigen, leads to pathology involving alveolar walls
*immunologically mediated disease:
-T CELL-MEDIATED (type IV) HYPERSENSITIVITY REACTIONS
-increased numbers of both intra-alveolar CD4+ and CD8+ T lymphocytes
-most patients have specific IgG antibodies against causative agent

23
Q

hypersensitivity pneumonitis - morphology

A

*early phase = acute alveolar damage
*late phase = bronchiole/airway centered chronic inflammation:
-lymphocytes, plasma cells
-non-necrotizing granulomas
*chronic phase = INTERSTITIAL FIBROSIS:
-fibroblastic foci
-obliterative bronchiolitis
-honeycombing

24
Q

hypersensitivity pneumonitis - clinical features

A

*acute attacks following inhalation of antigenic dust in sensitized patients:
-usually appear 4-6 hours after exposure and may last for 12 hours-several days
-fever, dyspnea, cough, and leukocytosis
*PFTs show an acute restrictive disorder
*imaging: MICRONODULAR INTERSTITIAL INFILTRATES
*chronic/continuous protracted exposure leads to progressive fibrosis

25
Q

pneumoconioses - overview

A

*restrictive lung disorders due to inhalation of mineral dusts (INORGANIC AGENTS)
*causative agents include:
-coal dust
-silica
-asbestos
-berylliosis

26
Q

pneumoconioses - pathogenesis

A

*particle phagocytosis by pulmonary alveolar macrophages:
-reaction depends on size, shape, solubility, and reactivity of particles
-chronic inflammation leads to interstitial fibroblast proliferation and collagen deposition

note - tobacco smoking exacerbates effects of all inhaled mineral dusts

27
Q

Coal Worker’s Pneumoconiosis - overview

A

*restrictive lung disorder caused by inhalation of coal particles
*exposure = coal mining

28
Q

Coal Worker’s Pneumoconiosis - pathogenesis

A

*coal dust phagocytosed by macrophages stimulate release of proinflammatory fibrogenic mediators

29
Q

Coal Worker’s Pneumoconiosis - clinical features

A

*anthracosis (simple) = asymptomatic
*“Black lung” = progressive/massive fibrosis

30
Q

Coal Worker’s Pneumoconiosis - morphology

A

*ANTRACOSIS = black carbon deposits
*coal nodules = black collagenous scars
-adjacent to respiratory bronchioles, usually upper lobes
-usually multiple

31
Q

Silicosis - overview

A

*restrictive lung disorder caused by inhalation of silicon
*exposures: sandblasting, quarrying, mining, stone cutting, foundry work, ceramics

32
Q

Silicosis - pathogenesis

A

*crystalline silica particles phagocytosed by macrophages releasing inflammatory mediators, e.g. IL-1

33
Q

Silicosis - clinical features

A

*imaging: fine nodularity in upper lung/apices
*slowly progressive, leading to impairment of pulmonary function

34
Q

Silicosis - morphology

A

*SILICOTIC NODULES = concentric whorls of collagen fibers surrounding amorphous center
-polarized birefringent silica particles
*may occur in hilar lymph nodes and pleura
*disease progression leads to honeycomb fibrosis

35
Q

Silicosis - polarized silicon crystals

A
36
Q

Asbestosis - overview

A

*restrictive lung disorder caused by inhalation of asbestos
*exposures: mining, milling, and fabrication of ores and materials; installation and removal of insulation

37
Q

Asbestosis - pathogenesis

A
  1. fibrous crystalline hydrated silicates phagocytosed by macrophages stimulate FIBROGENIC EFFECT
  2. asbestos fibers generate free radicals as ONCOGENIC EFFECT (most common malignancy is mesothelioma)
38
Q

Asbestosis - clinical features

A

*progressive dyspnea 10-20 years after exposure
*increased risk of: lung carcinoma (5x); mesothelioma (1000x)

39
Q

Asbestosis - morphology

A

*interstitial pulmonary fibrosis
*ASBESTOS BODIES (ferruginous body) = golden brown, beaded rods with translucent center
-asbestos fibers coated with Fe
*pleural fibrosis/plaques in visceral pleura
-dense collagenous adhesions between lungs and chest wall

Pulmonology Exam (36 decks)

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