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Pathology > Restrictive pulmonary diseases > Flashcards

Restrictive pulmonary diseases Flashcards

1
Q

What are the two general conditions causing restrictive lung defects?

A

Chest wall disorders: Bony abnormalities or neuromuscular disease that restrict lung expansion.
Chronic interstitial and infiltrative diseases: Inflammation and fibrosis of pulmonary connective tissue.

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2
Q

What are the key features of restrictive lung defects?

A

Reduced expansion of lung parenchyma.
Reduction in total lung capacity.

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3
Q

What are the clinical features of restrictive lung diseases?

A

Dyspnoea.
Tachypnoea.
End-inspiratory crackles.

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4
Q

What are the late-stage manifestations of restrictive lung defects?

A

Cyanosis.
Secondary lung hypertension.
Right-sided heart failure (Cor pulmonale).

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5
Q

What findings are commonly seen on chest radiographs in restrictive lung diseases?

A

Bilateral infiltrative lesions, including:
Small nodules.
Irregular lines.
Ground-glass shadows.

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6
Q

What is the epidemiology of UIP(Usual Interstitial Pneumonia)?

A

Most common interstitial pneumonia.
Associated with the worst prognosis.
Corresponds clinically to Idiopathic Pulmonary Fibrosis (IPF).

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7
Q

What is the hypothesized cause of UIP?

A

Immunologic factors are suspected.

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8
Q

What is the pathogenesis of UIP?

A

Unknown agent triggers repeated cycles of epithelial activation/injury.
Cytokines released → Abnormal epithelial repair.
Exuberant myo-/fibroblastic proliferation → Formation of “fibroblastic foci”.
TGF-β1 is a key driver:
Released by type I alveolar epithelial cells.
Promotes fibroblast transformation into myofibroblasts.
Collagen and ECM molecule deposition.

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9
Q

What are the macroscopic features of UIP?

A

Pleural surfaces: Cobblestone appearance due to scar retraction along interlobular septa.
Cut surface:
Firm, rubbery white fibrotic areas.
Lower-lobe predominance.
Sub-pleural and interlobular septal distribution

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10
Q

What are the microscopic findings in UIP?

A

Patchy interstitial fibrosis:
Varies in intensity and age of the lesions.
Early lesions: Exuberant fibroblastic proliferation.
Late lesions: Collagenous, less cellular.
Honeycomb fibrosis:
Dense fibrosis → Destruction of alveolar architecture.
Cystic spaces lined by hyperplastic type II pneumocytes or bronchiolar epithelium.
Mild to moderate mixed inflammatory infiltrates.
Vascular changes:
Intimal fibrosis and medial thickening → Lung hypertension.

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11
Q

What are the causes of Non-Specific Interstitial Pneumonia (NSIP)?

A

NSIP occurs secondary to various etiologic factors, including:

Infections.
Collagen vascular diseases.
Hypersensitivity pneumonitis.
Drug reactions.

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12
Q

What are the two pathological patterns in NSIP?

A

Cellular Pattern:

Mild to moderate chronic interstitial inflammation.
Mainly lymphocytes with a few plasma cells.
Uniform or patchy distribution.
Fibrosing Pattern:
Diffuse or patchy interstitial fibrosis.

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13
Q

What are the clinical features of NSIP?

A

Dyspnoea.
Cough.

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14
Q

How does the prognosis of NSIP compare to UIP?

A

Better than UIP, with a 5-year survival rate of > 80%.

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15
Q

Which pattern has a better outcome in NSIP?

A

Cellular pattern (younger patients) has a better outcome than the fibrosing pattern (older patients).

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16
Q

What is the synonym for Cryptogenic Organizing Pneumonia (COP)?

A

Bronchiolitis Obliterans Organizing Pneumonia (BOOP).

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17
Q

What are the characteristic microscopic findings in COP?

A

Polypoid plugs of loose organizing connective tissue (“Masson bodies”) found in:
Alveolar ducts.
Alveoli.
Bronchioles.
The connective tissue is all of the same age.
Normal underlying lung architecture.
No interstitial fibrosis or honeycomb lung.

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18
Q

What are the chest X-ray findings in COP?

A

Sub-pleural or peri-bronchial patchy areas of airspace consolidation.

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19
Q

What are the clinical features of COP?

A

Cough.
Dyspnoea.

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20
Q

How is COP treated?

A

Oral administration of steroids for ≥6 months.

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21
Q

What is pneumoconiosis?

A

A lung disease caused by the inhalation of inorganic dust particles, leading to interstitial fibrosis.

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22
Q

What are the common inorganic dust particles associated with pneumoconiosis?

A

Coal dust (least fibrogenic).
Silica (very fibrogenic).
Asbestos (very fibrogenic).
Beryllium (very fibrogenic).

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23
Q

What factors influence the development of pneumoconiosis?

A

Amount of dust retained in the lungs and airways.
Size and shape of the particles, influencing resilience (elastic capacity).
Solubility and physicochemical reactivity of the particles.
Additional effects of other irritants, e.g., tobacco smoking.

24
Q

What is anthracosis, and how is it characterized?

A

Cause: Inhalation of carbon dust.
Symptoms: Asymptomatic.
Macro/microscopic features:
Irregular black patches within lung tissue.
Carbon-carrying macrophages (“dust cells”).

25
Q

What are the types of coal workers pneumoconiosis?

A

Simple Coal Workers’ Pneumoconiosis:

Inhalation of coal dust (carbon and silica).
Coal macules (<1cm) around bronchioles in upper lobes.
Complicated Coal Workers’ Pneumoconiosis (Progressive Massive Fibrosis):

Fibrotic nodules filled with necrotic black fluid.
Complications:
Bronchiectasis, pulmonary hypertension, Cor pulmonale, Caplan Syndrome (RA + pneumoconiosis), death from respiratory or right-sided heart failure.

26
Q

What is silicosis and what are its features?

A

Cause: Exposure to free silica dust (miners, glass manufacturers, stone cutters).
Pathogenesis: Silica dust ingested by alveolar macrophages → Macrophage damage → Release of lysosomal enzymes & cytokines → Fibrogenesis.
Macro/microscopic findings:
Silicotic nodules: Concentric collagen layers, with or without central cavitation.
Complication: SilicoTuberculosis (concurrent with tuberculosis).

27
Q

What is asbestosis and its histopathological features?

A

Cause: Inhalation of asbestos fibres.
Pathogenesis: Asbestos fibres ingested by macrophages → Release of fibroblast-stimulating growth factors → Diffuse interstitial fibrosis (mainly in lower lobes).
Predispositions: Bronchogenic carcinoma, malignant mesothelioma (pleura and peritoneum).
Histopathological features:
Ferruginous bodies: Iron-coated asbestos fibres, yellow-brown, rod-shaped with clubbed ends (Prussian blue [+]).
Hyalinised fibro-calcific plaques on the parietal pleura.

28
Q

What are the lung manifestations associated with connective tissue diseases?

A

Rheumatoid Arthritis:

Chronic pleuritis, with or without effusion.
Diffuse interstitial pneumonitis and fibrosis.
Intrapulmonary rheumatoid nodules and pulmonary hypertension.
Scleroderma:

Diffuse interstitial fibrosis (more common NSIP than UIP pattern).
Systemic Lupus Erythematosus:

Patchy, transient parenchymal infiltrates.
Occasionally, severe lupus pneumonitis.

29
Q

Multisystem, non-infectious granulomatous disease that produces chronic interstitial fibrosis

A

Sarcoidosis

30
Q

Epidemiology of Sarcoidosis

A

African people. Clinically apparent during teenage or young adult years

31
Q

Pathogenesis of sarcoidosis

A

CD4Th cells interact with unknown airborne Ags (e.g. mold or mildew, pesticides) → Release of cytokines → Formation of non-caseating granulomas

32
Q

What are the macroscopic/microscopic findings in sarcoidosis?

A

Granulomas located in the interstitium, mediastinal, and hilar nodes.
Granulomas contain multinucleated giant cells.
Presence of Schaumann bodies (calcium and protein inclusions) and asteroid bodies (star-shaped inclusions).

33
Q

What are the clinical features of sarcoidosis?

A

Interstitial lung disease.
Enlarged hilar lymph nodes.
Anterior uveitis (inflammation of the front part of the eye).
Erythema nodosum (painful red nodules on the skin).
Polyarthritis (inflammation of multiple joints).

34
Q

What are the typical radiologic findings in sarcoidosis?

A

Bilateral hilar lymphadenopathy.
Interstitial lung disease with diffuse reticular densities.

35
Q

What are the key immunologic phenomena in sarcoidosis?

A

Reduced sensitivity and often anergy to skin test antigens (e.g., Tuberculin test [-]).
Polyclonal hyperglobulinemia.
Laboratory findings:
Hypercalcemia and hypercalciuria.
Hypergammaglobulinemia.
Increased activity of serum Angiotensin-Converting Enzyme (ACE).

36
Q

An immunologically mediated disease associated with exposure to a known inhaled antigen.

A

Hypersensitivity Pneumonitis

37
Q

No involvement of IgE antibodies (Type I hypersensitivity) and no eosinophilia.

A

Hypersensitivity Pneumonitis

38
Q

What is the immune mechanism behind Hypersensitivity Pneumonitis?

A

Type III hypersensitivity reaction: Formation of immune complexes due to binding of specific precipitating antibodies to inhaled allergens.
Type IV hypersensitivity reaction: Presence of non-caseating granulomas.
Bronchial-alveolar lavage specimens show an increase in T lymphocytes (both CD4+ and CD8+).
Complement and immunoglobulins are found in the vessel walls.

39
Q

What are the histopathologic features of Hypersensitivity Pneumonitis?

A

Patchy mononuclear cell infiltrates in the pulmonary interstitium, typically with peribronchial accentuation.
Predominantly lymphocytes, with plasma cells and epithelioid cells.
In acute forms, there are also neutrophils.
Non-caseating granulomas in peribronchiolar locations.
Chronic cases show diffuse interstitial fibrosis.

40
Q

What is Farmer’s Lung, a common form of Hypersensitivity Pneumonitis?

A

Caused by moldy hay.
First exposure: Development of IgG antibodies.
Second exposure: Binding of IgG antibodies to inhaled allergens leads to the formation of immune complexes (Type III hypersensitivity).
Chronic exposure: Leads to granulomatous inflammation (Type IV hypersensitivity).

41
Q

Group of clinical/pathologic pulmonary conditions characterized by infiltration and activation of eosinophils

A

Pulmonary eosinophilia

42
Q

Pulmonary eosinophilia is divided into

A

Acute Eosinophilic Pneumonia with respiratory failure
Simple Pulmonary Eosinophilia
Tropical Eosinophilia
Secondary Eosinophilia
Idiopathic Chronic Eosinophilic Pneumonia

43
Q

Rapid onset of fever, dyspnoea, hypoxia and diffuse pulmonary infiltrates on Chest x-rays

A

Acute Eosinophilic Pneumonia with respiratory failure

44
Q

Transient pulmonary lesions
- Blood eosinophilia
- Benign clinical course
- Thickening of the alveolar septa by an infiltrate of eosinophils and giant cells

A

Simple Pulmonary Eosinophilia

45
Q

Caused by infection with microfilariae and helminthic parasites

A

Tropical eosinophilia

46
Q

Associated with:
❖ Asthma ❖ Drug allergies ❖ Certain forms of vasculitis

A

Secondary eosinophilia

47
Q

Aggregates of lymphocytes & eosinophils within the septal walls & alveolar spaces in the lung periphery; Clinical features: High fever, night sweats and dyspnoea

A

Idiopathic Chronic Eosinophilic Pneumonia

48
Q

A form of eosinophilic pulmonary disease, characterized by absent or mild respiratory symptoms (most often dry cough), fleeting migratory pulmonary opacities, and peripheral blood eosinophilia

A

Loeffler syndrome

49
Q

Causes of Loeffler syndrome

A
  • Unidentifiable etiologic agent in up to 1/3 of patients
  • Parasitic infections (especially Ascaris lumbricoides)
  • Drugs (aspirin, penicillin)
  • Self-limiting condition; Resolution of the lung opacities within a month
50
Q

What is Eosinophilic Granuloma?

A

Synonym: Unisystem Langerhans Cell Histiocytosis.
Epidemiology: Occurs mainly in adolescents and young adults.

51
Q

What are the clinical features of Eosinophilic Granuloma?

A

Bone pain.
Pathologic fractures.

52
Q

What are the radiologic findings of Eosinophilic Granuloma?

A

Unifocal lytic lesions commonly in the skull, ribs, and femur.

53
Q

What are the histopathologic features of Eosinophilic Granuloma?

A

Localized proliferation of histiocytic cells, similar to Langerhans cells in the skin.
Presence of Birbeck granules (tennis racket appearance) in the cytoplasm.
Monocytes-macrophages, lymphocytes, and eosinophils are also present.

54
Q

What is Desquamative Interstitial Pneumonia (DIP)?

A

A form of restrictive pulmonary disease often seen in smokers in their 30s or 40s.
Associated with respiratory bronchiolitis-interstitial lung disease.

55
Q

What are the histopathologic features of Desquamative Interstitial Pneumonia (DIP)?

A

Alveolar spaces filled with macrophages.
Preservation of alveolar architecture.
Minimal fibrosis.

56
Q

What is the prognosis for Desquamative Interstitial Pneumonia (DIP)?

A

Much better than Usual Interstitial Pneumonia (UIP).
10-year survival rate is 70-100%.

Pathology (26 decks)

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