Diseases of Vascular Origin and Congenital Lesions

Question 1

What are the common causes of pulmonary hypertension?

Answer 1

Obstructive lung disease, restrictive lung disease, recurrent thromboemboli, heart issues, sleep apnea, idiopathic

Question 2

What is the primary histological change in pulmonary hypertension?

Answer 2

Decreased small vessels in the capillary bed

Question 3

List some clinical manifestations of pulmonary hypertension.

Answer 3

Dyspnea, fatigue, cor pulmonale

Question 4

What are the types of diffuse pulmonary hemorrhage syndromes?

Answer 4

Goodpasture syndrome, Idiopathic pulmonary hemosiderosis, vasculitis-associated hemorrhage

Question 5

Describe Goodpasture Syndrome.

Answer 5

Autoantibodies target type IV collagen, affecting kidneys and lungs; leads to pulmonary hemorrhage and glomerulonephritis

Question 6

What type of antibody is involved in Goodpasture Syndrome?

Answer 6

Anti-GBM (anti-glomerular basement membrane)

Question 7

What histological pattern is seen in Goodpasture syndrome?

Answer 7

Linear deposition of antibodies along the basement membrane

Question 8

Describe Polyangiitis with Granulomatosis.

Answer 8

ANCA-associated vasculitis affecting lungs, kidneys; causes necrotizing and granulomatous vasculitis

Question 9

What does ANCA stand for?

Answer 9

Anti-Neutrophil Cytoplasmic Antibody

Question 10

Describe the pathogenesis of ANCA-associated vasculitis.

Answer 10

Antibodies damage neutrophils; damaged neutrophils lodge in capillaries, releasing enzymes that damage tissue (pauci-immune)

Question 11

What age group is commonly affected by Polyangiitis with Granulomatosis?

Answer 11

Typically affects individuals in their 5th decade

Question 12

What are clinical features of Polyangiitis with Granulomatosis?

Answer 12

Upper respiratory issues (e.g., rhinorrhea, ulcers), lung involvement (cough, hemoptysis), renal involvement

Question 13

What is an extralobar sequestration?

Answer 13

Pulmonary mass outside pleural investment, supplied by systemic arteries, congenital origin

Question 14

How does extralobar sequestration differ from intralobar sequestration?

Answer 14

Extralobar is outside pleura with systemic blood supply; intralobar is within pleura, associated with recurrent infections

Question 15

What are some clinical features of extralobar sequestration?

Answer 15

Often presents in infancy with cyanosis, dyspnea, feeding difficulty, associated with congenital anomalies

Question 16

Describe intralobar sequestration.

Answer 16

Pulmonary mass within pleura, but not connected to bronchial tree; often acquired from repeated infections

Question 17

How is blood supplied to an intralobar sequestration?

Answer 17

Systemic artery, with hypertrophic pulmonary ligament arteries

Question 18

What are clinical features of intralobar sequestration?

Answer 18

Chronic or recurrent pneumonia, cough, sputum production, typically not seen with congenital anomalies

Question 19

What is the significance of a ‘pauci-immune’ pattern in ANCA-associated vasculitis?

Answer 19

No significant antibody or antigen deposition, yet severe inflammation and tissue damage occur

Question 20

What type of mass is typically associated with extralobar sequestration?

Answer 20

Discrete mass outside the normal pleural lung investment

Question 21

What artery supplies extralobar pulmonary sequestrations?

Answer 21

Direct branch of the thoracic or abdominal aorta or other systemic arteries

Question 22

What lung disease involves anti-glomerular basement membrane antibodies?

Answer 22

Goodpasture Syndrome

Question 23

In which lung condition are granulomatous microabscesses observed?

Answer 23

Polyangiitis with Granulomatosis

Question 24

How does intralobar sequestration typically form?

Answer 24

Acquired through repeated episodes of pneumonia

Question 25

What is a wedge-shaped infarct associated with?

Answer 25

Pulmonary embolism

Question 26

What is a common location for extralobar sequestrations?

Answer 26

Located outside the lung pleura, typically thoracic or abdominal area