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Transcript
Pathology Laboratory
Air exchange Diseases:
Interstitial diseases
Air Exchange Diseases
• Infectious Pneumonias
– Bacterial, Mycobacterial, Viral and Fungal
• Interstitial Lung Diseases
– Pneumoconiosis, Sarcoidosis, Fibrosis etc.
• Pulmonary “Vascular” Diseases
– Pulmonary Embolism, pulmonary edema,
ARDS
OBJECTIVES
• Describe the normal interstitium and the
changes in cells and lung architecture that may
occur in interstitial lung disease.
• Describe the key pathologic features of the
common idiopathic interstitial lung diseases.
• Describe the pathologic features of common
secondary interstitial lung diseases: sarcoidosis,
pneumoconiosis, and collagen vascular disease
and compare and contrast their pathological
features.
• Describe the key pathologic features of
pulmonary vascular diseases.
Interstitial Lung Diseases
• The clinician, radiologist and pathologist all
approach ILD differently
• Each approach provides unique insight
– But this can be confusing as each specialty has its
own terminology:
• Clinician: Disease causing breathlessness and
restriction on lung function
• Radiologist: Disease producing extra lines/dots
on radiographs
• Pathologist: Disease involving the interstitium
Pulmonary Interstitium
Structure
Axial Interstitium
Peribronchial
Perivascular
Peripheral Interstitium
Interlobular
Interstitium
Pleural Interstitium
Septal Interstitium
Pathophysiology
• Why is having extra stuff in your
pulmonary interstitium bad?
– Impairment of Lung Mechanics
• Compliance of the lungs is reduced
• Increases work of breathing
• Reduces Lung volumes
– Impairment of Gas Exchange
• Smaller lung volumes mean less surface area for
gas exchange
• Extra stuff in interstitium impedes transfer of gas
oxygen from alveoli to pulmonary capillaries
Interstitial lung diseases
• Acute vs chronic
• Known vs Unknown etiology
• Primary vs secondary
• Pathologic patterns
10
Interstitial Lung Diseases
• Primary
– Sarcoidosis (20%)
– Idiopathic Interstitial Pneumonia - IPF (15%)
• Secondary
–
–
–
–
–
–
–
Congestive Heart Failure / Pulmonary Edema
Infection (Pneumonia)
Malignancy (Lymphangitic Carcinomatosis)
Pneumoconiosis (25%)
Connective Tissue Disease (10%)
Hypersensitivity Pneumonitis (5%)
Drugs / Radiation (5%)
Primary Interstitial Lung
Diseases
12
Sarcoidosis
• Systemic disease of unknown etiology
• Often Involves lung and lymph nodes
• Etiopathogenesis: type IV hypersensitivity
reaction with granulomatous inflammation
along the lymphatic routes
• Progressive disease presenting symptoms
only late in its course
• Unpredictable outcome
Sarcoidosis
Granulomas
Granulomas
Artery
Bronchiole
Interlobular septum
Sarcoidal Granuloma
Histiocytes
Granuloma
Giant cell
Idiopathic Pulmonary Fibrosis
• Unknown etiology
• Middle-age to elderly individuals
• Chronic interstitial pneumonia evolving to
lung fibrosis
Idiopathic Pulmonary Fibrosis
End-Stage Lung
(Honeycombing)
Fibrous septa
Honeycombing
Idiopathic Pulmonary Fibrosis
Cystic spaces
Septal fibrosis
Idiopathic Pulmonary Fibrosis
Inflammatory cells
Septal fibrosis
Normal septum
20
Secondary Interstitial Lung
Diseases
21
Pneumoconioses
• Known etiology: Inorganic particulate matter
• Usually fibrogenic:
– Silica
– Asbestos
• Usually non-fibrogenic
– Coal
– Berylium (granulomatous)
Silicosis
• Subacute or chronic presentation
• Intra-histiocytic short needle-shaped
particles
• Histiocytic aggregates along lymphatic
routes and within lymph nodes
• Fibrous silicotic nodules, predominantly
upper lobes
23
Pulmonary Silicosis
Silicotic Nodule
Asbestosis
• Chronic presentation
• Long slender fibres coated by iron
(ferruginous body/asbestos body)
• Peribronchiolar fibrosis
• Fibrous thickening of the alveolar septa
• Predominantly lung bases and subpleural
26
Asbestos Body
Pulmonary Fibrosis
Septal thickening
Pleural Plaques
Collagen Vascular Diseases
• Collagen diseases frequently involve the
lung and cause interstitial pneumonia :
– Rheumatoid Arthritis
– Systemic Sclerosis
– Systemic Lupus Erythematosis
– Sjögren Syndrome
– Mixed Connective Tissue Disease
• The histological features are
indistinguishable from idiopathic forms
Hypersensitivity Pneumonitis
•
•
•
•
Known etiology: organic matter (e.g. fungal)
Acute, subacute or chronic presentation
Centrilobular inflammation (peribronchiolar)
Poorly-formed granulomas in the alveoli
31
Hypersensitivity Pneumonitis
Septal thickening
32
Hypersensitivity Pneumonitis
Giant cell
33
Pulmonary Vascular Diseases
Types of Diseases
• Obstruction to the venous outflow (pulmonary
congestion )
– Congestive heart failure → pulmonary edema
• Increase in the pulmonary artery resistance
(pulmonary hypertension)
– Primary or secondary (e.g. systemic sclerosis)
• Pulmonary artery obstruction (pulmonary
thromboembolism)
– Emboli (common) or thrombosis (uncommon)
• Vascular inflammation (vasculitis)
– Wegener’s granulomatosis, Churg-Strauss
Pulmonary Thromboembolism
• Pulmonary artery embolism (common)
– More than 95% associated to deep venous
thrombosis of the lower limbs in either
hypercoagulability state or bed ridden
patients.
• Pulmonary artery thrombosis (uncommon)
– Large vessels: rare, associated to pulmonary
hypertension or atherosclerosis.
– Small vessels: vasculitis, inflammation,
diffuse intravascular coagulation (DIC)
Pulmonary Embolism
• Acute morphologic changes
– Luminal obstruction with endothelial damage
– Small vessels: Alveolar hemorrhage in the
correspondjng irrigated area (e.g. lobule) and
possible infarct
– Larger vessels: hemorrhagic infarction in the
center and hemorrhage in the periphery
• Chronic morphologic changes
– Infarcted areas become fibrotic
– Chronic micro pulmonary embolism: pulmonary
hypertension
Thrombosis
Pulmonary Embolism
Pulmonary
Embolism
Pulmonary Embolism
Embolus
Artery
Respiratory bronchiole
41
Hemorrhagic pulmonary infarct
Alveolar hemorrhage
Alveolar septa
Complete Obstruction
Organized thrombosis
Internal elastic layer (short arrows)
Recanalized Thrombus
New lumen
Organized thrombus
ARDS
• Associated with the systemic release of
inflammatory mediators :
– Systemic inflammatory response syndrome
(SIRS)*
– Sepsis
– Infectious Pneumonias
– Mechanic Trauma (shock)
– Extensive burns
– Pancreatitis
– Toxic fumes inhalation
ARDS
• Pathogenesis
– Diffuse alveolar damage (DAD) to the
epithelial and endothelial cells, increased
vascular permeability, interstitial and alveolar
oedema, septal inflammatory infiltrate,
fibrinous exudate and hyaline membranes
formation (diffuse alveolar damage)
– Neutrophils are the main cells involved
– Difficult repair, frequently evolving to death or
fibrosis
ARDS - Pathogenesis
Diffuse Alveolar Damage
Diffuse Alveolar Damage
Early septal fibrosis
Hyaline membranes
OBJECTIVES
• Describe the normal interstitium and the
changes in cells and lung architecture that may
occur in interstitial lung disease.
• Describe the key pathologic features of the
common idiopathic interstitial lung diseases.
• Describe the pathologic features of common
secondary interstitial lung diseases: sarcoidosis,
pneumoconiosis, and collagen vascular disease
and compare and contrast their pathological
features.
• Describe the key pathologic features of
pulmonary vascular diseases.