Vascular Lung Disease Flashcards
Blood clots that occlude large pulmonary arteries are almost always ______ in origin
Embolic
Usual source of Pulmonary Embolus
Thrombi in the deep veins of the leg (95%)
Risk factors for pulmonary embolism
Immobilization
Hypercoagulable states
Indwelling central venous lines
Examples of hypercoagulable states
PrimarY (APAS, prothrombin mutations)
Secondary (Cancer, pregnancy, OCP use)
Histology of pulmonary infarction
Ischemic necrosis of the lung substance within the area of the hemorrhage
Pulmonary hypertension
Pulmonary blood pressure reaches 1/4 of systemic blood pressure
Groups of pulmonary hypertension
Pulmonary arterial hypertension
Pulmonary hypertension with left heart disease
Pulmonary hypertension with lung disease and/or hyoxemia
Pulmonary hypertension due to chronic thrombosis and/or embolic disease
Miscellaneous pulmonary hypertension
Structural cardiopulmonary conditions that increase pulmonary blood flow or pressure, pulmonary vascular resistance, or left heart resistance to blood flow;
Chronic Obstructive or interstitial lung disease
Antecedent congenital or acquired heart disease
Recurrent thrombo-emboli
Connective tissue disease
Obstructive sleep apnea
Chronic obstructive or interstitial LD
Destruction of lung parenhyma->fewer alveolar capillaries -> increased pulmonary arterial resitance -> elevated pressure
Antecedent congenital or aqcuired heart disease
Mitral stenosis -> increase in Left atrial Pressure -> increased pulmonary venous pressure -> increase in pulmonary arterial pressure
Recurrent thrombo-emboli
Obstructing emboli -> reduction in functional cross-sectional area of the pulmonary vascular resistance
Connective tissue disease
Systemic sclerosis -> involve pulmonary vasculature -> inflammation; intimal fibrosis, medial hypertrophy -> pulmonary hypertension
Obstructive sleep apnea
Significant contributor to pulmonary hypertension and cor pulmonale
Mutations in the ___________________ signalling pathway can lead to pulmonary hypertension
Bone Morphogenic Protein receptor Type 2 signalling pathway
Obstruction to the vasculature caused by proliferation of endotheial, sooth muscle and intimal cells accompanied by concentric laminar intimal fibrosis
Pulmonary hypertension
BMPR2
Cell surface protein beonging to TGF -B
Signaling in vascular smooth uscles, causes inhibition of prolifration and favors apoptosis
Secondary forms of pulmonary hypertension
Endothelial cell dystfunction (L to R shunts , fibrin)
Endothelial cell activation
Vasospastic
Ingestion of certain plants, medicines
Morphology of Pulmonary hypertension
Medial hypertrophy of muscular and elastic arteries
Atheromas of pulmonary artery
Tuft of capillary formations producing a network or web, spans, the lumen of thin walled small arteries
Plexiform lesion
Idiopathic pulmonary hypertension
Women 20 to 40 years
Dyspnea, fatigue, chest pain
Advance course of pulmonary hypertension
Respiratory disease, cyanosis, right ventricular hypertrophy
Death from decompensated cor pulmonale in 2 to 5 years
Superimposed thromboembolism and pneumonia
Diffuse Pulmonary Hemorrhage Syndromes
Goodpasteur syndrome
Idiopathic pulmonary hemosiderasis
Wegener granulomatosis
Goodpasteur Syndrome
Kidney and lung injury caused by circulating autoantibodies against the noncollagenous domain of the a3 charin of collagen IV
Inflamamtory destruction of the absement membrane in renal glomeruli and pulmonary vessels
Environmental insults of goodpasteur syndrome
Viral infection, exposure to hydrocarbon solvents or smoking-required to unmask cryptoid epitopes
Morphology in Goodpasteur syndrome
Lungs are heavy, witha reas of red brown consolidation
Focal necrosis f alveolar walls with intre-alveolar hemorrhages
Alveoli contain hemosiderin-laden macrophages
Interstitial fibrosis
Linear deposits of Ig along the BM of septal walls
Interstitial fibrosis
Best way to diagnose Goodpasteur syndrome:
Staining with immunofluorescence
Later signs of Goodpasteur syndrome
Fibrous thickening of septate
Hypertrophy of type II pneumocytes
Organization of blood in alveolar spaces
idiopathic Pulmonary Hemosiderosis
Intermittent, diffuse alveolar hemorrhage
Usually in young children
Productive cough, hemoptysis, anemia, weight loss, associated with diffuse pulmonary infiltration
Polyangitis with Granulomatosis
Wegener’s Granulomatosis
Autoimmune disease
Involves the upper respiratory tract and/or lungs
Hemoptysis
Capillaries and unscarred, poorly formed granulomas
