Vascular lung diseases Flashcards
Primary hypercoagulable states
Factor V Leiden mutation
Antiphospholipid antibody syndrome
Prothrombin mutation
Secondary hypercoagulable states
Obesity
OCP use
Pregnancy
Recent surgery
Cancer
Mechanism of increased pulmonary vascular resistance in PE
Vascular obstruction
Vascular constriction mediated by thromboxane A2 and serotonin
Mechanism of hypoxemia in PE
Increased perfusion of lung unites with low ventilation (V/Q mismatch)
Decreased CO with decrease in mixed venous PO2
Mechanism of increased alveolar dead space in PE
Vascular obstruction
Absent perfusion with preserved ventilation (V/Q mismatch)
Mechanism of decreased lung compliance in PE
Loss of surfactant causing alveolar edema and hemorrhage
Mechanism of increased airway resistance in PE
Reflex bronchoconstriction
Mechanism of increased respiratory rate in PR
Reflex stimulation of irritant receptors
Measurement of a degradation product of cross-linked fibrin
D-dimer
ECG findings in PE
S1Q3T3 pattern
Sinus tachy
Describe S1Q3T3 pattern on EKG
Large S wave in lead I
Q wave in lead III
Inverted T wave in lead III
Complications of lung infarct
Septic infarcts
Abscess
Mutation associated with idiopathic pulmonary HTN
BMPR2
Function of BMPR2
Binds to TGF-beta, BMP, activin, and inhibin. Leads to dysfunction and proliferation of endothelial cells and vascular smooth muscle cells
Inheritance of BMPR2 mutation
Autosomal dominant
Typical demographic for idiopathic pulmonary HTN
Female between 20-40 yo
Mechanism of secondary pulmonary HTN in chronic obstructive or interstitial lung diseases
Obliterate alveolar capillaries and increase pulmonary resistance
Grade 1 vascular changes in pulmonary HTN
Medial hypertrophy of the pulmonary muscular and elastic arteries
Grade 2 vascular changes in pulmonary HTN
Intimal and medial hypertrophy
Grade 3 vascular changes in pulmonary HTN
Pipestem fibrosis of arteries
Grade 4 vascular changes in pulmonary HTN
Plexiform lesions –> tuft of capillary formations
CXR findings in pulmonary HTN
Enlarged central pulmonary arteries
Complication of pulmonary HTN
Cor pulmonale
Treatment of primary pulmonary HTN
Vasodilators
Autoimmune disease defined by presence of anti-glomerular basement membrane antibodies
Goodpasture syndrome
Typically demographic for Goodpasture syndrome
Young males
Type II hypersensitivity reaction with antibodies against alveolar and glomerular basement membranes
Goodpasture syndrome
Auto-antibodies to non-collagenous domain of the alpha-3 chain of collagen IV of the basement membranes
Goodpasture syndrome
Young male pt presents with rapidly progressive glomerulonephritis and necrotizing hemorrhagic interstitial pneumonitis
Goodpasture syndrome
Microscopy findings of the lungs in Goodpasture syndrome
Focal necrosis within alveolar walls with intra-alveolar hemorrhages and hemosiderin-laden macrophages. Fibrous thickening of septum.
Immunofluorescence findings in the lungs in Goodpasture syndrome
Linear deposits of immunoglobulins along the basement membranes of the septal wall
Clinical features of Goodpasture syndrome
Crescentic glomerulonephritis and renal failure
Hemoptysis
Causes of diffuse pulmonary hemorrhage
Goodpasture syndrome
Idiopathic pulmonary hemosiderosis
Vasculitis associated –> polyangiitis with granulomatosis
Autoimmune disease involving upper respiratory tract and/or lung
Polyangiitis with granulomatosis/Wegener granulomatosis
Diagnostic test for polyangiitis with granulomatosis
Transbronchial lung biopsy
Transbronchial lung biopsy findings in polyangiitis with granulomatosis
Scattered, poorly formed granulomas
Triad of polyangiitis with granulomatosis
Acute necrotizing granulomas of URT and/or LRT
Necrotizing or granulomatous vasculitis
Focal necrotizing, often crescentic, glomerulonephritis
