Vascular Lung Disease Flashcards
What patient population is typically affected by primary PAH?
Uncommon
Predominantly females in 3rd – 5th decades
What causes primary PAH?
mutation in BMPR2
What are plexiform lesions?
a network of capillary formation span the lumen of pulmonary arteries
When are plexiform lesions most often seen?
primary PAH and congenital heart disease with left-to-right shunts
What are some diseases with vasculitis-associated hemorrhage?
- lupus erythematous
- Wegener granulomatosis
What does Goodpasture’s (aka Anti-GBM) syndrome show histologically?
Necrotizing hemorrhagic interstitial pneumonitis due to autoimmune alveolar basement membrane destruction is characterized by necrosis of the alveolar walls and intraalveolar hemorrhage
How are GP patients treated?
plasmapheresis and immunosuppression
What does immunohistological staining of GP show?
linear pattern with C3 and IgG
What is the triad of Wegener’s granulomatosis?
- necrotizing angiitis
- aseptic necrosis of URT an lungs
- focal glomerulonephritis
What causes Wegener’s granulomatosis?
Likely a T-cell mediated hypersensitivity reaction, the close relationship between c-ANCA titers suggests a pathogenic role
C-ANCA likely directly activates neutrophils to release oxygen radicals and proteolytic enzymes damaging endothelium
How does Wegener’s granulomatosis present?
May also involve temporal artery, cutaneous small vessels and cause extrapulmonary masses; rarely involves eyes, skin and heart
All ages, but most common with ages 45+; more common in males
Rarely associated with diffuse pulmonary hemorrhage
What happens in congenital cystic disease?
Bronchogenic cysts arise from abnormal detachments of primitive foregut and usually present with compression of nearby structures or are found incidentally
What is pulmonary sequestration?
discrete area of lung tissue that lacks connection to the airway system and has an abnormal blood supply arising from the aorta.
What is Meconium Aspiration Syndrome?
The baby has passed meconium into the amniotic fluid during labor or delivery
may occur if the baby breathes in (aspirates) this fluid into the lungs
In most cases, the outlook is excellent and there are no long-term health effects.
What would cause a baby to pass meconium in the uterus?
This will happen when babies are “under stress” because their supply of blood and oxygen decreases. This is often due to problems with the placenta or the umbilical cord.
