Pulmonary Vascular Diseases Flashcards
Who is primary pulmonary HTN typically seen in?
• how common is this?
• what mutation is this typically associated with?
Primary Pulmonary HTN is extremely rare but when it does present, its typically a dyspniec and/or fatgued female between 30 and 50.
• Typically associated with BMPR2 mutation that is autosomal dominant with partial penetrance
Notice medial hyperplasia on the picture below
What are the 3 general categories of things that cause secondary pulmonary HTN?
Lung Parenchyma:
• Loss of (COPD) or fibrosis of lung parenchyma reduces the net vascular cross-sectional area
Cardiogenic:
• Congestive Heart Failure
• L to R VSD, or ASD
Pulmonary Vascular:
• Recurrent Thromboemboli -> loss of functional capillaries
What is shown here?
• when is this pathology usually associated with?
Plexiform Lesion, typically seen in people in Muthia’s group 1 PAH. These are people with autoimmune diseases that have caused HTN, have IPF, or have BMPR2 mutation.
**Also seen in congenital L to R shunt
What autoimmune disease may have caused this appearance?
Goodpastures because these people have anti-type IV collagen alpha-3 antibodies leading to destruction of lung parenchyma
• Lungs are heavy and show diffuse dark red consolidation
What disease is this pathological pattern indicative of?
Goodpasture’s - notice necrosis of alveolar walls (nuclear fall out) and intraalveolar hemorrhage
Remember this person would have an elevated DLCO because of free Hbg’s ability to bind CO
What triad are you looking for in Wegner’s?
- Necrotizing angiitis
- Aseptic necrosis of respiratory tract and lungs (remember collapse of the nose bridge)
- Focal Glomerulonephritis
What are the key features to note in these pictures?
You can see ECCENTRICintimal proliferation and inflammation. Also there arepalisaded histiocytesandmultinucleatedgiant cells to the right of the arrow.PMNs everywhere indicated the lesion is a microabscess in the vessel wall.
This is a Wegner’s pattern
What does you typical Wegner’s Granulomatosis patient present like?
• what cells mediate this hypersensitivity?
All ages are affected, but most commonly its a 45+ male. Typically pulmonary hemorrhage is NOT diffuse.
• T cells are likely responsible for this reaction
**Note: it may also invovle the temporal artery, cutaneous smal vessels and cause extrapulmonary masses; rarely invovles eyes, skin and heart
What is this and what causes it?
Bronchogenic Cysts: causes by abnormal detachments of primative foregut and usually presents with compression of nearby structures or are found incidentally
What is pulmonary sequestration?
Area of the lung that lacks connection to the airway system and has an abnormal blood supply arising from the aorta
A baby is born and experiences some respiratory distress, a picture representative of the pathology in this baby’s lung is shown below. What has happened?
• key histological features?
Meconium Aspiration Syndrome
• baby passed meconium into the aminotic fluid during labor or delivery
• Outcomes of this can be severe
Key Features:
Notice the presence of squamous cells and debris and also meconium seen as the dense particles pointed out at the bottom right
Cause:
• baby is under stress in delivery and their supply of blood and oxygen decreases so they breath in these amniotic contents
NOTE: meconium - material that has sloughed off the GI tract of the baby vore it is bore
What has likely caused this pathology?
Cystic Fibrosis
• Distended broncioles (bronchiectasis) from extensive mucus plugging
• Pseudomonas giving the green consolidated appearance in the parenchyma
Note: H. flu and Staph a. are also common causes of infection in these patients, we also see BURKHOLDARIA in some of these kids
