vascular disorders of the lung
DESCRIPTION
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 - PowerPoint PPT PresentationTRANSCRIPT
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”
• 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 EMBOLI / INFARCT
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
• Reclassification at Venice 2003
• Pulmonary arterial hypertension
• Pulmonary hypertension with left heart disease
• Pulmonary hypertension with lung diseases / hypoxaemia
• Pulmonary hypertension due to chronic TE disease
PULMONARY HYPERTENSIONClinical Classification
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
• 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 arterial hypertension
• Pulmonary hypertension with left heart disease
• Pulmonary hypertension with lung diseases / hypoxaemia
• Pulmonary hypertension due to chronic TE disease
PULMONARY HYPERTENSIONClinical Classification
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 arteriopathyMedial hypertrophy, isolated or with intimal proliferation, concentric laminar, eccentric, adventitial fibrosis, plexifiorm and / or dilatation lesions, arteritis
• Pulmonary occlusive venopathy
• Pulmonary Microvasculopathy
PULMONARY HYPERTENSIONPathological classification – Venice 2003
PULMONARY HAEMORRHAGE & VASCULITIS
ALVEOLAR HAEMORRHAGE SYNDROMES• 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’SWEGENER’S
Multifocal ischaemicnecrosis has resultedin numerous cavitating lesionsscattered throughout upper and lower lobesof left lung.
Dark haemorrhagiclung parenchyma between the cavities tothe 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