2: Vasculitis & HTN Flashcards
What does the word vaculitis mean?
Vessel wall inflammation
What is the most common cause of vaculitis? (Hint: think general, not specific conditions)
Immune-mediate inflammation
Other general causes:
- Direct invasion of vascular wall by infectious pathogens
- Physical/mechanical injury
What are the clinical symptoms of vasculitis?
Fever, myalgias, arthralgias, malaise
What is the immune complex associated cause of noninfectious vasculitis?
SLE (Lupus)
- specific antigen rarely identified
- drug hypersensitivity and secondary to infections
What cause of noninfectious vasculitis is associed with anti-endothelial cell antibodies?
Kawasaki disease–> antibodys against your own endothelial cells
Which conditions fall under Large Vessel Vasculitis? (Picture the diagram!)
Takayasu Arteritis
Giant Cell Arteritis (Temporal Arteritis)
What conditions fall under Medium Vessel Vasculitis?
Polyarteritis Nodosa (PAN)
Kawasaki Disease
Where in the body would you expect to see the effects of Giant Cell Arteritis?
Temporal region–> because GCA is also known as Temporal Arteritis
Symptoms: headache, facial pain, temporal pain, jaw claudication
What gross pathology would you expect to see with Giant Cell Arteritis?
- Segmental nodular thickening of artery
- Narrowing of lumen (similar to atherosclerosis)
What microscopic pathology would you expect in active Giant Cell Arteritis?
- Mononuclear cell (macrophage, T lymphocyte) infiltration in internal elastic lamina and tunica media
- Granulomatous inflammation–> multinucleated giant cells at internal elastic lamina
- Focal involvement
What is the hallmark of the healed phase of Giant Cell Arteritis?
Elastic Fragmentation
Also would see: medial scarring and adventitial fibrosis
A 3yr old child is being seen in the ER with the following symptoms: fever, enlarged lymph nodes, strawberry tongue, edema of hands/feet, erythema of palms/soles, rash, and redness of the mucosal membranes.
What condition should you suspect?
Kawasaki Disease (AKA Mucocutaneaous Lymph Node Disease)
Hints
- On the test, anything with a kid is probably Kawasaki’s
- “Strawberry tongue”
- “Fever” –> Acute febrile (feverish), usually self-limited illness of infant/child
What is the expected microscopic pathology of Kawasaki Disease?
- Dense transmural inflammatory infiltrates (not symmetrical, more focal)
- Fibrinoid necrosis (less common)
- Thrombosis may occur
A 30yr old male with a history of smoking is urgently referred to the vascular clinic because he has been experiencing claudication in his lower legs, Raynaud’s like symptoms, and (per his primary care physician) superficial thrombophlebitis. The patient relates to you that he has severe pain, and ulcers on his hands and feet, with some of them turning black and he is very scared. Upon testing, you can tell that both his arteries and veins are being affected, and that he has HLA-A9 and HLA-B5 haplotypes.
What is your diagnosis?
Thromboangiitis Obliterans (Buerger Disease) –> hypersensitivity to tobacco antigens
Key points:
- Male, < 35yrs, cigarette smokers
- Triad: Raynaud phenomenon, claudication, superficial thrombophlebitis
- Severe pain, ulceration of toes/feet/fingers, gangrene
- Linked to HLA-A9 and HLA-B5 haplotypes
Treatment: STOP SMOKING
What are the two main components of Raynaud phenomenon?
Pallor and Cyanosis
All of the following regarding Immune-complex small vessel vasculitis is true except:
a. typically ANCA positive
b. presence of immune complex deposition
c. recurrence depends on source of immune complex
d. all of the above
e. a and c
a. typically ANAC positive–> immune complex small vessel vasculitis is typically ANCA negative
Which of the following vasculitis conditions are correctly matched?
a. Wegener granulomatosis: PR3-ANCA (c-ANCA)
b. Churg-Strauss: MPO-ANCA (p-ANCA)
c. IgA Vasculitis: C3P0-ANCA
d. Polyarteritis nodosa: (PAN-ANCA)
a. and b. are correctly matched
a. Wegener granulomatosis: PR3-ANCA (c-ANCA)
b. Churg-Strauss: MPO-ANCA (p-ANCA)
In a condition related to PR3-ANCA, where would you expect to see staining on a cell? What about MPO-ANCA?
PR3-ANCA: diffuse, finely granular staining of the cytoplasm –> c-ANCA for cytoplasm
MPO-ANCA: staining in rim around nucleus (because of artifact of fixation) –> p-ANCA for perinuclear
What are the principle antibodies in PR3-ANCA and MPO-ANCA?
PR3-ANCA: Proteinase 3
MPO-ANCA: Myeloperoxidase
A 40yr old man comes into the ER because he is having trouble bleeding and is coughing up blood and has patches of dark purple/black skin around his nostrils. His blood works shows that he has an increased level of Creatinine Kinase and he has c-ANCA antibodies in his bloodstream.
What do you suspect is the causative condition?
Granulomatosis w/ Polyangiitis (Wegener Granulomatosis)
What disease is characterized by the following triad of symptoms:
- Necrotizing/granulomatous vasculitis of small and medium sized vessels (most prominent in lung, upper airways, kidney)
- Necrotizing granulomas of upper respiratory and/or lower respiratory tract
- Focal/segmental necrotizing glomerulonephritis w/ crescent formation
Granulomatosis w/ Polyangiitis (AKA Wegeners Granulomatosis)
Which of the following conditions is not a consequence of untreated malignant HTN?
a. renal failure
b. hypertensive encephalopathy
c. cardiac failure
d. thrombotic microandiopathy
e. death
f. none of the above
f. none of the above
All of these are possible consequences of untreated malignant HTN.
**Death occurs within 2yrs
Where is the most significant pathology observed in benign hypertension?
Elastic and large muscular arteries:
- accelerated atherosclerosis
- degeneration of media and elastic layers–> aortic dissections/aneurysms
Small muscular arteries and arterioles:
- hyaline arteriolosclerosis (vessel wall thickening, lumen narrowing, eosinophilic material)
- arterial fibroelastic intimal hyperplasia (multilayering of elastic layers and collagen)
- hypertrophy/hyperplasia of medial smooth muscle
What microscopic pathologies would you expect to see in the small vessels in malignant HTN?
- Fibrinoid necrosis of small arteries/arterioles
- Microvascular thrombosis, RBC fragmentation
- Hyperplastic arterio- and arteriolosclerosis (“onion-skinning”)
+ proliferation of intimal myofibroblasts
+ multilayered deposition of extracellular matrix
+ severe narrowing of lumen
During an autopsy, you encounter a set of kidneys that show signs of benign nephrosclerosis, including reduced size, finely granular cortical surfaces, and cortical scarring. Under a microscope, you see hyaline arteriolosclerosis, aterial intimal fibrosis and mild hyperplasia, and chronic ischemic changes.
What do you think this patient suffered from that caused their benign nephrosclerosis?
Benign HTN
Clinical features: proteinuria, slow progression to renal failure
Which of these image sets are related to malignant HTN and it’s affects on the kidney?
a.
b.
c Uhhh, I thought this was brain cancer??
a. benign
b. malignant
In malignant HTN, what gross pathology would you expect to see on the kidneys?
Punctate hemorrhages–> looks like a stabbing pattern (petechiae)
What two kinds of brain aneurysms would you expect to see in hypertension cases?
- Saccular (“berry”) aneurysms in the circle of Willis
- Intracerebral microaneurysms (Charcot-Bouchard–> Basal Ganglia)
What heart (not vascular) related consequences would you see with HTN?
- Accelerated coronary atherosclerosis
- Concentric left ventricular hypertrophy (LVH)
