VASCULAR
DISORDERS OF THE
LUNG
•PULMONARY OEDEMA
•PULMONARY EMBOLI / INFARCT
•PULMONARY HYPERTENSION
•PULMONARY HAEMORRHAGE &
VASCULITIS
PULMONARY EMBOLI / INFARCT
• PE – most common preventable cause of death in
hospitalized patients
• Embolus: A detached intravascular solid, liquid or
gaseous mass that is carried by the blood to a site
distant from its point of origin
• 99% of all emboli are thromboemboli
• Rarer – bone, marrow, atheroma, fat, tumour, FBs [
cotton, cardiac catheter, talc (ivdu)], parasites, amniotic
fluid
• 95% PE arise in thrombi in large deep veins of LL
• What happens depends on size:occlude main PA, lodge
at bifurcation = saddle embolus, shower of smaller
emboli may travel distally, passage thru ASD/VSD =
“paradoxical embolus”
PULMONARY EMBOLI / INFARCT
• Most PEs are small and silent
• Respiratory & haemodynamic compromise
• If > 60% of total pulmonary vasculature obstructed sudden death, acute RHF, EMD
• Middle size arteries – haemorrhage
• Obstruction of smaller Pul. Aa branches (end arteries) infarction
• PE leading to infarction uncommon in young – but is
seen where circulation already inadequate – heart &
lung disease
• Chronically - Pulmonary hypertension, R heart strain
PULMONARY EMBOLI / INFARCT
• Acute thromboemboli - blood, fibrin,
platelets, neutrophils arranged in
alternating linear zones – Lines of Zahn
• After 2-3 days – organization, ingrowth
of fibroblasts , capillaries from vessel wall
• Thrombus is replaced by fibrosis and
small vascular spaces – recanalization
PULMONARY EMBOLI / INFARCT
• ¾ infarcts affect the lower lobes
• Not usually excised – unless clinically
unsuspected e.g. unresolving infiltrate or
nodular opacity
• Classically a wedge shape with base on pleural
surface
• Central bland necrosis with ghosts of lung
architecture, haemorrhage, active fibroblasts at
edge, squamous metaplasia, reactive atypia
• Eventually a fibrous scar
• Consider causes other than simple TE
PULMONARY HYPERTENSION
•
Mean pulmonary artery pressure >25mmHg
at rest, > 30mmHg during exercise
•
Elevated pressure is related to high
pulmonary vascular resistance due to
obstruction of small arteries
3 factors contribute to small pulmonary
artery obstruction: vasoconstriction, cellular
proliferation & fibrosis and thrombosis
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•
Reclassification at Venice 2003
PULMONARY HYPERTENSION
Clinical Classification
• Pulmonary arterial hypertension
• Pulmonary hypertension with left heart
disease
• Pulmonary hypertension with lung
diseases / hypoxaemia
• Pulmonary hypertension due to chronic
TE disease
PRIMARY PULMONARY
HYPERTENSION
• Primary plexiform arteriopathy
• Young women (20-40 years)
• Dyspnoea & fatigue, some chest pain
• Progression to resp distress, RVH and cor
pulmonale
• Rx – vasodilators, anticoagulants, prostacyclins
SECONDARY PULMONARY
HYPERTENSION
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Cardiac disease
Obstruction of main Pulmonary Veins
Chronic embolic disease
Lung disease
Alveolar hypoxia
Liver disease , portal hypertension
HIV infection
Ingestants / inhalants
Collagen vascular disease
PULMONARY HYPERTENSION
Clinical Classification
• Pulmonary arterial hypertension
• Pulmonary hypertension with left heart
disease
• Pulmonary hypertension with lung
diseases / hypoxaemia
• Pulmonary hypertension due to chronic
TE disease
PULMONARY HYPERTENSION
Heath and Edwards Grades
I
medial hypertrophy
II
intimal proliferation – mild
III
intimal fibrosis – moderate
IV
plexiform or dilatation lesions, necrotizing
arteritis – severe
PULMONARY HYPERTENSION
Pathological classification – Venice 2003
• Pulmonary arteriopathy
Medial hypertrophy, isolated or with intimal proliferation,
concentric laminar, eccentric, adventitial fibrosis, plexifiorm and /
or dilatation lesions, arteritis
• Pulmonary occlusive venopathy
• Pulmonary Microvasculopathy
PULMONARY HAEMORRHAGE
& VASCULITIS
ALVEOLAR HAEMORRHAGE SYNDROMES
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Goodpasture’s syndrome = Antibasement membrane disease
Idiopathic pulmonary haemosiderosis (IPH)
Wegener’s granulomatosis (WG)
CVD esp. acute Lupus
Drugs, inhalants
Idiopathic RPGN
SECONDARY ALVEOLAR HAEMORRHAGE
LOCALIZED HAEMORRHAGE
WEGENER’S GRANULOMATOSIS
(WG)
TRIAD
• Granulomatous inflammmation of URT & LRT
• Generalized vasculitis
• Glomerulonephritis
• LUNG most frequently affected
• Middle aged adults – but wide age range
• Fever, malaise, wt loss, cough, chest pain, hemoptysis,
renal failure, anaemia, sinusitis
• Radiology – multiple lung masses resembling mets or
cavitating abscesses
• Serology – Antineutrophil cytoplasmic antibodies
(ANCA)
WEGENER’S
Multifocal ischaemic
necrosis has resulted
in numerous
cavitating lesions
scattered throughout
upper and lower lobes
of left lung.
Dark haemorrhagic
lung parenchyma
between the cavities to
the upper right
ANCA
- fluorescent microscopy
C-ANCA, diffuse granular
cytoplasmic staining pattern
in WG
P-ANCA, perinuclear staining
pattern in microscopic polyangiitis
WG in the LUNG
• Necrotizing granulomatous inflammation
• Necrotizing vasculitis
• Large geographic areas of necrosis (dirty
/ basophilic / blue under the microscope)
• Microabscesses – neutrophils
• MNGCs
• Ddx: mycobacterial & fungal infection
VASCULAR
DISORDERS OF THE
LUNG
•PULMONARY OEDEMA
•PULMONARY EMBOLI / INFARCT
•PULMONARY HYPERTENSION
•PULMONARY HAEMORRHAGE &
VASCULITIS