Vascular Diseases - Jarzembowski Flashcards
Distinguish between a true and false aneurysm.
Which are symmetric: Saccular or fusiform aneurysms?
True aneurysms involve ballooning of all three vessel wall layers. False involves a defect in one or more and leads to mural hematoma.
Fusiform are symmetric.
According to Pathoma, all dissections (and aneurysms) require two things: High stress and wall weakness. What usually constitutes these two criteria?
High stress = Mostly happen in aorta, where pressure is highest.
Wall weakness = Hypertension (causing cystic medial degeneration) or inherited connective tissue disease.
In addition to Marfan syndrome, another connective tissue disease known as _____ can promote aneurysm formation because of defective synthesis of type ___ collagen.
Ehler-Danlos, III.
What role do MMPs and TIMPs play in aneurysm formation?
MMPs degrade connective tissue, TIMPs inhibit MMP action.
How does hypertension cause medial degeneration?
How does atherosclerosis cause medial degeneration?
Which of these more typically affects the abdominal aorta?
Hypertension compresses the vasa vasorum, causing ischemia of the tunica media in large vessels (actually hyaline arteriolosclerosis).
Atherosclerosis builds plaques that inhibit oxygen diffusion, causing ischemia of the tunica media in medium vessels (no vasa vasorum present)
Abdominal: Atherosclerosis
Besides HTN and atherosclerosis, what are some other, rarer causes of aneurysms?
Congenital defects (eg Berry), infections (eg mycotic, syphilis), trauma, and other vasculites.
What is the most common aortic aneurysm?
Which demographics are at highest risk?
How does it present?
Abdomanal Aortic Aneurysm, AAA.
Older men who smoke and have hypertension. Or some combination thereof.
A pulsatile abdominal mass that grows over time.
What are some of the complications of AAAs?
How are they treated?
Rupture is the most serious. May cause impingement of nearby structures (ureter, renal/mesenteric/spinal arteries). May form thrombi/emboli.
If large, must be treated agressively with stenting or open repair.
What is the classic cause of thoracic aortic aneurysms? (hint: Not just hypertension)
What are some of its complications?
Syphilis!
Mediastinal encroachment (can compress trachea, esophagus, recurrent laryngeal nerve), aortic dilation & insufficiency, and aortic rupture.
Who are at highest risk of aortic dissections?
What exactly occurs, and where?
Those with hypertension (“40-60yrs of age”), or with connective tissue disorders.
A tear in the tunica intima (usually within first 10cm of ascending aorta) allows blood to penetrate and rupture the tunica media, forming a false lumen.
Where does aortic dissection usually progress?
What are some complications of aortic dissection?
Usually anterograde (“out”).
Blood may “reenter” and form a double-barreled lumen (google it). If dissection occurs retrograde, cardiac tamponade may result. Can also rupture pleurally/peritoneally.
What is the classic presentation of an aortic dissection?
Distinguish between a type A and B dissection.
Tearing/stabbing, 10 out of 10 pain in the anterior chest that radiates to the back.
A: Starts proximal to the aortic arch. B: Starts distal to the arch.
What are the three etiologies of vasculitis?
Immune-mediated, infectious, or idiopathic.
What are ANCAs?
What are the signifiances of PR3-ANCAs and MPO-ANCAs?
ANCA: Anti-neutrophil cytoplasmic antibody, targeted against something in neutrophil granules.
PR3: A proteinase. ANCA+ in Wegner’s Granulomatosis.
MPO: Myeloperoxidase. ANCA+ in Microscopic Polyangiitis and Churg-Strauss Syndrome.
Describe how infections can contribute to vasculitis.
Which vasculites have unknown etiologies?
Either by direct invasion of the vessel wall, or by triggering an autoimmune reaction.
Unknown: Giant cell arteritis, Takayasu Arteritis, Polyarteritis Nodosa (all large/medium)
Recall which vasculites affect:
Large vessels
Medium vessels
Small vessels
Large: Giant cell arteritis, Takayasu disease
Medium: Polyarteritis Nodosa, Kawasaki disease
Small: Microscopic Polyangiitis, Wegener’s Granulomatosis, Churg-Strauss syndrome…
Giant Cell (Temporal) Arteritis
Describe its etiology and pathogenesis.
Who is generally affected?
What are its complications?
Giant Cell (Temporal) Arteritis)
Unknown cause (maybe T-cells against elastin), affects aorta and major branches, eg temporal/vertebral/ophthalmic. Most common.
Older patients.
Headache, diplopia, visual loss (can progress to blindness, treat aggressively).
Giant Cell (Temporal) Arteritis)
How is it diagnosed?
How is it treated?
Giant Cell (Temporal) Arteritis)
Biopsy of 2-3cm of temporal artery shows nodular thickenings, granulomatous inflammations and destruction of IEL. Oh, and increased sed rate.
Treat with steroids.
Takayasu Arteritis
Describe its etiology and pathogenesis.
Who generally suffers from it?
How is it diagnosed and treated?
Takayasu Arteritis
A lot like Giant Cell; unknown etiology (autoimmune?), afflicts aortic arch & branches with granulomatous inflammation. Also affects pulmonary arteries, and causes upper extremity pulselessness.
Young asian women.
Biopsy reveals lymphocytes, giant cells, fibrosis (like temporal arteritis…). Treat with steroids (also like temporal arteritis).
Kawasaki Syndrome
Describe its etiology and pathogenesis.
Who is susceptible?
What are its complications?
Kawasaki Syndrome
Unknown etiology (antibodies against vessel wall? Viral trigger?). Involves many vessels.
Young asian children.
Often involves the coronaries (causes aneurysm, thromboembolism), also “mucocutaneous lymph node syndrome”.
Kawasaki Syndrome
What does biopsy/histology reveal?
How is it treated?
Kawasaki Syndrome
Histology: “Necrosis and inflammation, aneurysms, resembles polyarteritis nodosa”
From Pathoma: Give aspirin*, usually self-limited (*risk of embolism trumps risk of Reye’s)
Polyarteritis Nodosa
Describe its etiology & pathogenesis. What is affected?
Who is affected?
How does it present?
Polyarteritis Nodosa
Autoimmune; necrotizing inflammation of just about every vessel and organ except the lungs. (especially renal!)
Presents in young adults (sometimes HBsAg+)
Rapidly accelerating hypertension, abdominal pain with melena, neurologic and skin disturbances.
Polyarteritis Nodosa
How does it appear on histology?
How is it treated?
Polyarteritis Nodosa
All stages of inflammation & fibrosis are present; forms “string-of-pearls” appearance.
Treat with steroids / cyclophosphamide.
Microscopic Polyangiitis
Describe its etiology and pathophysiology.
Describe its serology and histology.
What are its clinical features?
Microscopic Polyangiitis
Autoimmune reaction to something. Affects multiple organs, especially lungs & kidnesy (necrotizing glomerulonephritis & pulmonary capillaritis).
MPO-ANCA+, Histo shows fibrinoid necrosis, neutrophils, karyorrhexis.
Features depend on affected organs: hemoptysis, hematuria, proteinuria, muscle pain, purpura…
Wegener’s Granulomatosis
This disease has a very similar etiology and pathogenesis as Microscopic polyangiitis. What are some key differences?
Who is affected, and what is the outlook?
How are both of these diseases treated?
Wegener’s Granulomatosis
Nasopharyngeal involvement. PR3-ANCA+, not MPO-ANCA+. More pulmonary involvement (pneumonitis, sinusitis).
M>F, 80% 1-yr mortality rate if untreated.
Treat both with steroids & cyclophosphamide.
Churg-Strauss Syndrome
Describe its etiology and pathogenesis.
What are its symptoms?
Churg-Strauss Syndrome
Allergic granulomatosis causing small vessel inflammation with peripheral eosinophilia. Associated with asthma/rhinitis.
Symptoms: Palpable purpura, GI/renal symptoms, cardiomyopathy. Sometimes MPO-ANCA+.
Buerger Disease
What is its other name?
How does it present, and who does it affect?
What are its etiology and treatment?
Buerger Disease
Thromboangiitis Obliterans.
Smokers (often young, of israeli/indian/japanese descent) developing painful ischemia/gangrene of the digits (affects tibial/radial arteries).
Presumably tobacco-based endothelial toxicity. Stop smoking!
Raynaud Phenomenon
What is it?
Who does it affect?
Reynaud Phenomenon
A recurrent vasospasm triggered by cold. Digits turn pale, then cyanotic and flushed after warming.
Young, healthy women, but sometimes secondary to SLE/scleroderma/atherosclerosis/Buerger.
