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Flashcards in Vascular Lung Disease Deck (19)
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What patient population is typically affected by primary PAH?

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.


What causes hyaline Membrane Disease in neonates?

Premature birth is associated with the neonatal respiratory distress syndrome due to the lack of surfactant production prior to the third trimester of pregnancy.

Although the pathogenesis of the neonatal respiratory distress syndrome is very different from the acute respiratory distress syndrome, the histopathology is essentially the same.


MOA of CF?

AR. In US, approximately 1 in 2500 live births have disease



They stem from obstruction ad infection

The bronchioles are often distended with thick mucus, exhibit hyperplasia and hypertrophy of the mucus secreting cells

Lung abscesses are common


What is an infection that is rising in CF patients?