Vascular Lung Disease

Question 1

Blood clots that occlude large pulmonary arteries are almost always ______ in origin

Answer 1

Embolic

Question 2

Usual source of Pulmonary Embolus

Answer 2

Thrombi in the deep veins of the leg (95%)

Question 3

Risk factors for pulmonary embolism

Answer 3

Immobilization
Hypercoagulable states
Indwelling central venous lines

Question 4

Examples of hypercoagulable states

Answer 4

PrimarY (APAS, prothrombin mutations)

Secondary (Cancer, pregnancy, OCP use)

Question 5

Histology of pulmonary infarction

Answer 5

Ischemic necrosis of the lung substance within the area of the hemorrhage

Question 6

Pulmonary hypertension

Answer 6

Pulmonary blood pressure reaches 1/4 of systemic blood pressure

Question 7

Groups of pulmonary hypertension

Answer 7

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

Question 8

Structural cardiopulmonary conditions that increase pulmonary blood flow or pressure, pulmonary vascular resistance, or left heart resistance to blood flow;

Answer 8

Chronic Obstructive or interstitial lung disease
Antecedent congenital or acquired heart disease
Recurrent thrombo-emboli
Connective tissue disease
Obstructive sleep apnea

Question 9

Chronic obstructive or interstitial LD

Answer 9

Destruction of lung parenhyma->fewer alveolar capillaries -> increased pulmonary arterial resitance -> elevated pressure

Question 10

Antecedent congenital or aqcuired heart disease

Answer 10

Mitral stenosis -> increase in Left atrial Pressure -> increased pulmonary venous pressure -> increase in pulmonary arterial pressure

Question 11

Recurrent thrombo-emboli

Answer 11

Obstructing emboli -> reduction in functional cross-sectional area of the pulmonary vascular resistance

Question 12

Connective tissue disease

Answer 12

Systemic sclerosis -> involve pulmonary vasculature -> inflammation; intimal fibrosis, medial hypertrophy -> pulmonary hypertension

Question 13

Obstructive sleep apnea

Answer 13

Significant contributor to pulmonary hypertension and cor pulmonale

Question 14

Mutations in the ___________________ signalling pathway can lead to pulmonary hypertension

Answer 14

Bone Morphogenic Protein receptor Type 2 signalling pathway

Question 15

Obstruction to the vasculature caused by proliferation of endotheial, sooth muscle and intimal cells accompanied by concentric laminar intimal fibrosis

Answer 15

Pulmonary hypertension

Question 16

BMPR2

Answer 16

Cell surface protein beonging to TGF -B

Signaling in vascular smooth uscles, causes inhibition of prolifration and favors apoptosis

Question 17

Secondary forms of pulmonary hypertension

Answer 17

Endothelial cell dystfunction (L to R shunts , fibrin)
Endothelial cell activation
Vasospastic
Ingestion of certain plants, medicines

Question 18

Morphology of Pulmonary hypertension

Answer 18

Medial hypertrophy of muscular and elastic arteries

Atheromas of pulmonary artery

Question 19

Tuft of capillary formations producing a network or web, spans, the lumen of thin walled small arteries

Answer 19

Plexiform lesion

Question 20

Idiopathic pulmonary hypertension

Answer 20

Women 20 to 40 years

Dyspnea, fatigue, chest pain

Question 21

Advance course of pulmonary hypertension

Answer 21

Respiratory disease, cyanosis, right ventricular hypertrophy
Death from decompensated cor pulmonale in 2 to 5 years
Superimposed thromboembolism and pneumonia

Question 22

Diffuse Pulmonary Hemorrhage Syndromes

Answer 22

Goodpasteur syndrome
Idiopathic pulmonary hemosiderasis
Wegener granulomatosis

Question 23

Goodpasteur Syndrome

Answer 23

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

Question 24

Environmental insults of goodpasteur syndrome

Answer 24

Viral infection, exposure to hydrocarbon solvents or smoking-required to unmask cryptoid epitopes

Question 25

Morphology in Goodpasteur syndrome

Answer 25

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

Question 26

Linear deposits of Ig along the BM of septal walls

Answer 26

Interstitial fibrosis

Question 27

Best way to diagnose Goodpasteur syndrome:

Answer 27

Staining with immunofluorescence

Question 28

Later signs of Goodpasteur syndrome

Answer 28

Fibrous thickening of septate
Hypertrophy of type II pneumocytes
Organization of blood in alveolar spaces

Question 29

idiopathic Pulmonary Hemosiderosis

Answer 29

Intermittent, diffuse alveolar hemorrhage
Usually in young children
Productive cough, hemoptysis, anemia, weight loss, associated with diffuse pulmonary infiltration

Question 30

Polyangitis with Granulomatosis

Answer 30

Wegener’s Granulomatosis
Autoimmune disease
Involves the upper respiratory tract and/or lungs
Hemoptysis
Capillaries and unscarred, poorly formed granulomas