Pulmonary Diseases Of Vacular Origin

Question 1

Saddle embolus

Answer 1

PE lodging at bifurcation of pulmonary artery

Question 2

Consequence of large and small PE

Answer 2

Large - acute cor Pulmonale, death
Small - pulmonary hemorrhage; infarction

• 10% of PE causes pulmonary infarction
• great percentage affects the lower lobes
• presence of lines of Zhan in the thrombus

Question 3

Pulmonary infarct is morphologically ?

Answer 3

• Hemorrhagic but become pale due to RBC lysis with hemosiderin production

Ischemic necrosis admits hemorrhagic areas involving the alveolar walls, bronchioles and vessels

Question 4

Clinical features of PE? Which may mimick MI

Answer 4

Severe chest pain
Hemoptysis
Pleuritis
Dyspnea
Shock
fever
increased Serum lactose dehydrogenase

Question 5

Chest radiography of PE ?

Answer 5

Wedge shaped infiltrates
Diagnosis - Ct angiography
Diagnosis of DVT - Duplex ultrasonography
Treatment - use of anticoagulants preceded by thrombolytic agents

Question 6

Complications of pulmonary embolism

Answer 6

• pulmonary vascular sclerosis
• pulmonary hypertension
• chronic cor Pulmonale

Question 7

Pulmonary hypertension occurs when? ?

Answer 7

• Pulmonary system is a Low pressure system,
• Pulmonary hypertension occurs when the pulmonary pressure reaches 1/4 of the systemic levels

Question 8

Classification of pulmonary hypertension?

Answer 8

Pulmonary arterial hypertension
PH with left heart disease
pH associated with lung disease
PH due to chronic thrombotic/embolic disease
Miscellaneous PH

Question 9

Risk factors for Pulmonary Hypertension?

Answer 9

• COPD, ILD
• Recurrent thromboemboli
• CT disorders (systemic sclerosis)
• Onstructive sleep apnea in obesity
• congenital or acquired heart disease (mitral stenosis)

Question 10

Familial PH is due to the mutations in what?

Answer 10

BMPR2 - Bone morphogenetic protein receptor type 2 on Chromosomes 2q33

Question 11

Pathogenesis of secondary PH?

Answer 11

• increasing shear forces causes endothelial cell disorder (left to right shunts or thromboembolism)
• endothelial activation in the presence of reduced prostacyclin promote thrombogenesis and fibrin deposit

Question 12

Morphology of pulmonary hypertension

Answer 12

• Medial hypertrophy of muscular and elastic arteries
• Atheromas of pulmonary artery and it’s major branches
• right ventricular hypertrophy

Question 13

Treatment of Pulmonary Hyoertension

Answer 13

Lung transplantation fro selected individuals
CCB
Prostacyclin analogues
Digoxin
Diuretics
Anticoagulatioj
Endothelial receptors antagonists
Inhaled nitric oxide
Phosphodiestersse 5 inhibitors

Question 14

Diffuse pulmonary hemorrhagic syndrome includes? Interstitial disorders

Answer 14

• Goodpasture syndrome
• idiopathic pulmonary hemosiderosis
  vasculitis associated hemorrhage
• wegener grabulomatosis
• hypersensitivity angitis
• Lupus erythematous

Question 15

Goodpasture syndrome pathogenesis ?

Answer 15

• Circulating autoantibodies to alpha 3 chain of collagen type 4 the noncollagenous domain
• affects the kidneys and lung parenchyma
• destroys basement membranes
  Leading to necrotizing hemorrhagic interstitial pneumonitis and glomerulonephritis
• common in teens or 20s
• male predominance is (89%) with active smokers’
• HLA DRB1 - 1501 and 1502

Question 16

Morphological feature of Goodpasture syndrome ?

Answer 16

• Heavy lungs
• Red brown consolidation

Microscopy
- focal necrosis of alveolar walls
- intra alveoli hemorrhages
- hemosiderin laden macrophages within alveoli
- Fibrous septae Thickening
- hypertrophy of type 2 pnuemocytes
- Immunoglobulin deposits along septal wall diagnostic
Kidneys show
- focal proliferative glomerulonephritis
- Cresentric glomerulonephritis
- immunoglobulin deposit along GBM

Question 17

Clinical presentations

Answer 17

hemoptysis
Renal failure leading to uremia
Radiograph; Focal pulmonary consolidations

Treatment
- intensive plamsapharesis
- immunosuppressive therapy

Question 18

Idiopathic pulmonary hemosiderosis ?

Answer 18

• Rare disorder
• Intermittent diffuse alveolar hemorrhage
• young children, Adults rarely
• productive cough, hemoptysisc anemia, weight loss,
• diffuse pulmonary infiltration
• no antibodies have been detected in etiology
  Treatment
• long termmm immunosuppressive with prednisolone, azathioprine

Question 19

Wegener granulomastosis ?

Answer 19

• automimmune -
• invoices the URT and or the lungs
• hemoptysis

Diagnosis: trans-bronchial lung biopsy
- shows capillarities and scattered poorly forms granulomas