SFP: vascular lung disease Flashcards
What three diseases are associated with alveolar hemorrhage?
Goodpasture syndrome, idiopathic pulmonary hemosiderosis, and granulomatosis with polyangiitis
What is the triad associated with alveolar hemorrhage syndromes?
Hemoptysis, anemia, and diffuse pulmonary infiltrate on imaging
Describe Goodpasture syndrome generally.
Antibodies to basement membranes cause dysfunction of vessels of lungs and kidneys that causes bleeding
What causes hemorrhagic interstitial pneumonitis?
Goodpasture syndrome
Goodpasture syndrome may present with… (3)
Proteinuria, hematuria, acute renal failure
Describe what is seen in the lungs with Goodpasture syndrome.
Hemorrhage in the alveoli, inflamed capillaries, hypertrophy, and possible hemosiderin
What is a signature pattern seen in testing of Goodpasture syndrome?
Linear immunofluorescence of immunoglobulin
Describe idiopathic pulmonary hemosiderosis.
A rare condition usually in kids and infants that causes bleeding in the alveolar spaces without presence of renal disease, antibodies to basement membrane, and variable clinical course and response to steroids. Lots of hemosiderin-laden macrophages in alveolar spaces
What is the triad seen with granulomatosis with polyangiitis?
Necrotizing granulomas, pulmonary vasculitis, and crescentic glomerulonephritis
What is seen on x-ray with granulomatosis with polyangiitis?
White infarcts due to necrotizing vasculitis of medium sized arteries
What is the typical patient population of granulomatosis with polyangiitis?
50-year-old men with sinusitis, pneumonitis, and renal disease
c-ANCA is seen in…
granulomatosis with polyangiitis
What is the cornerstone treatment of granulomatosis with polyangiitis?
steroids and immunosuppressants
What is an issue with granulomatosis with polyangiitis?
opportunistic infection and renal failure
What do we see in the lungs with granulomatosis with polyangiitis?
necrotizing vasculitis, parenchymal granulomas, nodules
___ impacts capillaries while ___ can impact other/larger vessels.
Goodpasture’s, granulomatosis with polyangiitis
What are predisposing factors to pulmonary thromboembolism?
Trauma, stasis, surgery, contraceptive pills
Describe clinical outcomes of pulmonary embolism.
Many are small and silent and are eventually lysed. Large ones may be instantly fatal, or some may cause infarction. People can also have recurrent PEs
What can recurrent PEs cause?
Pulmonary hypertension and chronic right heart strain
What is a main factor that may cause varied presentation of pulmonary infarction?
Dual blood supply to the lung (pulmonary and bronchial arteries)
What infarct shape is associated with pulmonary embolism?
Wedge-shaped
What are some treatments of PE?
Anticoagulant, thrombolysis, surgery
What are the qualifications for pulmonary hypertension?
Mean pulmonary artery pressure greater than 25 at rest or 30 with exercise
Is primary or secondary pulmonary hypertension more common?
Secondary
Describe primary pulmonary hypertension.
Pulmonary hypertension with no other identifiable cause. It is rare and is often in women of reproductive age
What pathophysiologic mechanisms can cause secondary pulmonary hypertension?
- Increased pulmonary blood flow (VSD, PDA)
- Prolonged left atrial hypertension (LV failure)
- Vascular obstruction (PE, stenosis, pulmonary veno-occlusive disease)
- Pulmonary vasoconstriction (COPD, sleep apnea, kyphoscoliosis)
People with sickle cell are prone to…
Pulmonary hypertension via vascular obstruction
What are plexiform lesions?
Lesions formed only in primary hypertension
