Vasculitis and Atherosclerosis Flashcards
1
Q
Polyarteritis Nodosa (PAN) (Presentation)
A
- Young adults, males > females
- Immune complex mediated
- Any organ can be affected except lungs
- As with any vasculitis fever, fatigue, weight loss and arthralgia/myalgia
- Symptoms according to systems:
- Renal: hypertension, glomerulonephritis, and renal failure
- Neurological: any large peripheral nerve especially peroneal neuropathy (foot drop) [if multiple peripheral neuropathies it is called Mononeuritis Multiplex] and also stroke in young age
- GI: abdominal pain that worsens by eating from vasculitis of mesenteric vessels, nausea, vomiting and GI bleeding
- Skin: lower extremity ulcers, livedo reticularis, purpura, nodules and rarely gangrene
2
Q
Polyarteritis Nodosa (PAN) (Complications)
A
- Thrombosis that can lead to infarction
- Aneurysms particularly in kidneys, heart and GI tract
3
Q
Polyarteritis Nodosa (PAN) (Diagnosis)
A
- Most accurate test is biopsy of a symptomatic site:
- Involvement of medium vessels
- Segmental necrotizing vasculitis (transmural inflammation)
- Acute lesions show fibrinoid necrosis and neutrophils
- Test all PAN for hepatitis B and C. HBsAg is +ve in 30%
- Perinuclear antineutrophil cytoplasmic autoantibodies (P-ANCA) [directed against myeloperoxidase] is +ve in less than 20% (all of them are microscopic polyangiitis variant)
- Angiography of renal, mesenteric or hepatic arteries shows abnormal dilatation or “beading” and innumerable renal microaneurysms
4
Q
Polyarteritis Nodosa (PAN) (Treatment and Prognosis)
A
- Prednisone and cyclophosphamide
- Fatal in most untreated cases and with Rx 90% will have long-term remission
5
Q
Eosinophilic Granulomatosis and Angiitis (Churg-Strauss syndrome)
(Presentation)
A
- It affects all systems including the lung
- Classically it is three stages:
- Allergic stage: asthma and allergic rhinitis
- Eosinophilic stage: fever, fatigue, weight loss and arthralgia/myalgia
- Vasculitis stage
- There may be an overlap among the stages and not all patients develop all stages
6
Q
Eosinophilic Granulomatosis and Angiitis (Churg-Strauss syndrome)
(Complications)
A
- Thrombosis that may lead to infarctions
- Peritonitis and perforations of GI tracts
- Myocarditis (the most common cause of death)
7
Q
Eosinophilic Granulomatosis and Angiitis (Churg-Strauss syndrome)
(Diagnosis)
A
- Biopsy of the symptomatic site:
- Involvement of small vessels
- Eosinophilic infiltration and granulomas
- P-ANCA present in 50% (it can be +ve also in microscopic angiitis of PAN and ulcerative colitis) and C-ANCA present in 35% of cases
- Increased IgE level
- Pauci-immune (no immunofluorescent staining for IgG) glomerulonephritis
8
Q
Eosinophilic Granulomatosis and Angiitis (Churg-Strauss syndrome)
(Treatment)
A
Prednisone and cyclophosphamide
9
Q
Granulomatosis with Polyangiitis (Wegener’s Granulomatosis)
Presentation
A
- Ages 40-60, males > females
- Any organ can be affected but more likely are respiratory system and kidneys
- As with any vasculitis fever, fatigue, weight loss and arthralgia/myalgia
- According to system:
- Respiratory: chronic sinusitis, otitis media, mastoiditis, saddle nose (due to septal perforation), bilateral pneumonitis with nodular and cavitary pulmonary infiltrates
- Renal: rapidly progressive glomerulonephritis (Crescent formation) or focal necrotizing (less common) in which both will lead to chronic renal failure
10
Q
Granulomatosis with Polyangiitis (Wegener’s Granulomatosis)
Diagnosis
A
- Best initial test is Cytoplasmic antineutrophil cytoplasmic autoantibodies (C-ANCA) [against proteinase 3]
- Most accurate test is biopsy being the lung first then renal then sinuses:
- Involvement of small vessels
- Necrotizing vasculitis (fibrinoid necrosis and neutrophils)
- Granulomas
- Pauci-immune (no immunofluorescent staining for IgG) glomerulonephritis
11
Q
Granulomatosis with Polyangiitis (Wegener’s Granulomatosis)
Treatment and Prognosis
A
- Prednisone and cyclophosphamide
- Untreated cases have 1 year mortality rate of 80%, but with treatment 90% will have long-term remission
12
Q
Microscopic Polyangiitis
Presentation
A
- Ages 40-60, males > females
- Any organ can be affected but more likely are respiratory system and kidneys
- As with any vasculitis fever, fatigue, weight loss and arthralgia/myalgia
- According to system:
- Respiratory(no nasopharyngeal involvement): bilateral pneumonitis with nodular and cavitary pulmonary infiltrates
- Renal: rapidly progressive glomerulonephritis (Crescent formation) or focal necrotizing (less common) in which both will lead to chronic renal failure
- Skin: palpable purpura and livedo racemosa may be present
13
Q
Microscopic Polyangiitis
Diagnosis
A
- Biopsy of the symptomatic site:
- Involvement of small vessels
- Necrotizing vasculitis with no granulomas
- P-ANCA (anti-myeloperoxidase)
- Pauci-immune (no immunofluorescent staining for IgG) glomerulonephritis
14
Q
Microscopic Polyangiitis
Treatment
A
Prednisone and Cyclophosphamide
15
Q
Giant Cell Arteritis (Temporal Arteritis)
Presentation
A
- Elderly people; female > male
- Associated with HLA-DR4 and polymyalgia rheumatica (pain and stiffness in shoulder and pelvic girdle muscles with difficulty in combing hair and rising from chair, elevated ESR, normochromic normocytic anemia, normal CPK and aldolase)
- Symptoms:
- Visual: blurred vision, double vision, or visual loss
- Jaw claudication
- Scalp tenderness
- fever, fatigue, weight loss and arthralgia/myalgia
16
Q
Giant Cell Arteritis (Temporal Arteritis)
Diagnosis
A
- ESR is elevated; it is very sensitive but not specific
- Biopsy of temporal artery:
- Involvement of large vessels (mostly branches of carotid artery)
- Focal segmental granulomatous inflammation (multinucleated giant cells and fragmentation of internal elastic lamina that may lead to fibrosis and lumen narrowing)
17
Q
Giant Cell Arteritis (Temporal Arteritis)
Treatment
A
High dose prednisone quickly prior to temporal artery biopsy to prevent blindness (which is irreversible and is secondary to ophthalmic artery occlusion)
18
Q
Takayasu’s Arteritis (Pulseless Disease)
Presentation
A
- Usually Asian females less than 40 years old
- As any other vasculitis fever, fatigue, weight loss and arthralgia/myalgia
- Weak upper extremities pulses
- Visual symptoms: visual loss, field defects and retinal hemorrhages
- Neurological abnormalities
19
Q
Takayasu’s Arteritis (Pulseless Disease)
Diagnosis
A
- Elevated ESR
- Angiography: shows irregular thickening and narrowing of aortic arch and narrowing of orifices of its major arterial branches
- Biopsy (which cannot be done):
- Involvement of large vessels
- Granulomas with extensive intimal fibrosis
20
Q
Takayasu’s Arteritis (Pulseless Disease)
Treatment
A
Corticosteroids
21
Q
Thromboangiitis Obliterans (Buerger's Disease) (Presentation)
A
- Usually males less than 40 years. Israel, India, Japan and South America
- Associated with heavy smoking
- Claudication in affected extremities, thrombophlebitis, secondary Raynaud phenomenon, ulceration and gangrene
22
Q
Thromboangiitis Obliterans (Buerger's Disease) (Diagnosis)
A
- It is a diagnosis of exclusion
- Angiography: may show diagnostic features for the disease like “crockscrew” appearance of arteries and “tree root” or “spider leg” of collateral circulation
- Biopsy (which is rarely done due to poor healing):
- Involvement of medium vessels
- Recurrent neutrophilic vasculitis with microabscesses, and segmental thrombosis of affected vessels which leads to vascular insufficiency
23
Q
Thromboangiitis Obliterans (Buerger's Disease) (Treatment)
A
- Smoking cessation (choose it for USMLE) will slow progression and decrease severity but won’t halt progression
- Acute cases use vasodilators like Limaprost, epidural anesthesia, and hyperbaric oxygen therapy to reduce pain
- Chronic cases: lumber sympathectomy and bypass surgery
24
Q
Kawasaki Disease (Mucocutaneous lymph node syndrome) (Presentation)
A
- Infants and young children less than 4 years. Males > females. Japan, Hawaii and U.S. mainland
- The endemic form is associated with HLA-B51
- Acute high fever which doesn’t respond to usual Rx (acetaminophen or ibuprofen) and continues at least 5 days, conjunctivitis, erythema and erosions of oral mucosa (blisters and strawberry tongue), skin pealing of finger and toe tips, generalized maculopapular rash, and lymphadenopathy.
25
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Kawasaki Disease (Mucocutaneous lymph node syndrome)
(Complications)
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- Coronary artery aneurysm
| - Death (1-2%) due to rupture of coronary artery aneurysm or coronary thrombosis
26
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Kawasaki Disease (Mucocutaneous lymph node syndrome)
(Diagnosis)
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- Diagnosed clinically
- Autopsy:
* Involvement of medium vessels
* Segmental necrotizing vasculitis with coronary arteries being the most affected (70%)
27
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Kawasaki Disease (Mucocutaneous lymph node syndrome)
(Treatment)
```
Hight dose aspirin and IV immunoglobulin
28
Henoch-Schonlein Purpura (IgA vasculitis)
| Presentation
- Most common childhood systemic vasculitis (IgA immune complex deposition)
- Often follows Upper respiratory tract infection, and associated with IgA nephropathy (Berger disease)
- Classic triad:
* Skin: palpable purpura on buttocks/legs
* Arthralgias
* GI: abdominal pain and bleeding
- Also may be present with hematuria
29
Henoch-Schonlein Purpura (IgA vasculitis)
| Diagnosis
- Most often is clinical
- The most accurate is biopsy which shows:
* Involvement of small vessels
* Leukocytoclastic vasculitis
- Increased serum IgA (not reliable and it is a wrong answer)
30
Henoch-Schonlein Purpura (IgA vasculitis)
| Treatment
- Most cases resolve spontaneously
- Steroids just for severe abdominal pain or progressive renal insufficiency (do not reverse the insufficiency but may decrease progression)
31
Henoch-Schonlein Purpura (IgA vasculitis)
| Most common associated microorganisms
- Beta-hemolytic group A streptococci
- Hepatitis B
- HSV
- Parvovirus B19
- Coxsakie virus
- Adenovirus
- H. pylori
- Measles
- Rubella
- Mumps
- Mycoplasma
32
Henoch-Schonlein Purpura (IgA vasculitis)
| Most common associated medications
- Vancomycin
- Cefuroxime
- Enalapril and Captopril (ACE inhibitors)
- Diclofenac
- Ranitidine
- Streptokinase
33
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Raynaud Disease (Primary Raynaud phenomenon)
(Cause, Presentation, Underlying pathology)
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- Small artery vasospasm of extremities, nose and ears
- Presents as blanching and cyanosis of fingers and toes that is precipitated by cold temperature and emotions
- No underlying disease or pathology
34
Secondary Raynaud Phenomenon
| Cause, Presentation, Underlying pathology
- Arterial insufficiency secondary to underlying disease
- Presents as blanching and cyanosis of fingers and toes
- Scleroderma (CREST), SLE, Buerger disease, and atherosclerosis
35
Large Vessels Vasculitis
| Enumerate
- Giant cell arteritis (Temporal arteritis)
| - Takayasu's arteritis
36
Medium Vessels Vasculitis
| Enumerate
- Polyarteritis Nodosa (PAN)
- Kawasaki disease (mucocutaneous lymph node syndrome)
- Thromboangiitis obliterans (Buerger disease)
37
Small Vessels Vasculitis
| Enumerate
- Granulomatosis with polyangiitis (Wegener granulomatosis)
- Microscopic polyangiitis
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
- Henoch-Schonlein purpura
38
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Monckeberg Sclerosis (Medial Calcific Sclerosis)
(Definition, Effect, X-ray appearance)
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- Calcification of internal elastic lamina and media of medium sized arteries like femoral, radial and ulnar arteries
- Vascular stiffening without obstruction (intima not involved)
- "Pipestem" appearance
39
Arteriolosclerosis
| Types
- Affects small arteries and arterioles
- Two types:
* Hyaline arteriolosclerosis (pink, glassy arterial wall thickening with luminal narrowing) seen in essential benign hypertension and DM
* Hyperplastic arteriolosclerosis (smooth muscle proliferation resulting in concentric "onion skin" thickening of arterial wall and luminal narrowing) seen in malignant hypertension
40
Atherosclerosis
| Types of vessels affected, Which ones are most commonly affected
- Affects elastic (conducting) arteries and large and medium sized muscular (distributing) arteries
- Abdominal aorta > coronary artery > popliteal artery > carotid artery
41
Atherosclerosis
| Presentation
- Could be asymptomatic
- Angina
- Claudication
42
Atherosclerosis
| Progression
- Fatty streaks (lipid-laden macrophages)
- Uncomplicated atheromatous plaque (grossly: raised, yellow-white plaque; Micro.: fibrous cap [composed of collagen, smooth muscle, lymphocytes and foam cells] which overlies a necrotic core called atheroma [composed of cholesterol clefts, lipid, foam cells and necrotic debris])
- Complicated atheromatous plaque: show dystrophic clacification, ulceration potentially with production of atheroemboli and plaque rupture (at shoulders) with superimposed thrombus
43
Normal Blood Pressure (JNC-7 classification)
| Systolic, Diastolic, Lifestyle modifications, Drug therapy with no comorbidities
- < 120 mmHg and
- < 80 mmHg
- Encourage
- No drug therapy
44
Prehypertension (JNC-7 classification)
| Systolic, Diastolic, Lifestyle modifications, Drug therapy with no comorbidities
- 120-139 mmHg or
- 80-89 mmHg
- Yes
- No anti-hypertensive drug is indicated
45
Stage I hypertension (JNC-7 classification)
| Systolic, Diastolic, Lifestyle modifications, Drug therapy with no comorbidities
- 140-159 mmHg or
- 90-99 mmHg
- Yes
- Thiazide diuretics for most patients; ACEIs, ARBs, beta-blockers, CCBs, or a combination may be considered
46
Stage II hypertension (JNC-7 classification)
| Systolic, Diastolic, Lifestyle modifications, Drug therapy with no comorbidities
- 160 mmHg or more or
- 100 mmHg or more
- Yes
- Two-drug combination for most patients (usually a thiazide diuretic plus an ACEI, an ARB, a beta-blocker or a CCB)
47
Hypertensive Crisis
| Presentation
Presents with end-organ damage (the principle of diagnosis not BP measurement) revealed by:
- Renal disease
- Chest pain (ischemia or MI)
- Back pain (aortic dissection)
- Changes in mental status (hypertensive encephalopathy)
48
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Hypertensive Urgency (Hypertensive Crisis)
(Diagnosis and Treatment)
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- Dx: elevated BP with mild to moderate symptoms (headache, chest pain) without end organ damage
- Rx: Oral anti-hypertensives (e.g., beta-blockers, clonidine, ACEIs) with goal of gradually lowering BP over 24-48 hrs
49
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Hypertensive Emergency (Hypertensive Crisis)
(Diagnosis and Treatment)
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- Dx: elevated BP with signs and symptoms of impeding end-organ damage such as acute renal injury, intracranial hemorrhage, papilledema, or ECG changes suggestive of ischemia or pulmonary edema
- Rx: IV medications (Labetalol, nitroprusside, nicardipine) with goal of lowering mean arterial pressure by no more than 25% over the first 2 hours to prevent cerebral hypoperfusion or coronary insufficiency
50
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Malignant Hypertension (Hypertensive Crisis)
(Diagnosis and Treatment)
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- Dx: BP is systolic 180 or more and/or diastolic 120 or more with progressive renal failure and/or encephalopathy with papilledema
- Rx: IV nitroprusside
51
Benign Hypertension
| Pathological Features
Hyaline arteriolosclerosis
52
Malignant Hypertension
| Pathological Features
- Macro.: Kidney will develop petechial hemorrhages "flea-bitten" appearance
- Micro.: hyperplastic arteriolosclerosis "onion-skin" with necrotizing arteriolitis (fibrinoid necrosis)
53
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Primary Hypertension
(Treatment Guidelines)
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- Weight loss is the single most effective life style modification
- The goal BP in otherwise healthy individuals is < 140/< 90 mmHg
- The goal BP in diabetics or patients with renal disease with proteinuria is < 130/<80 mmHg
- Diuretics, ACEIs, beta-blockers decrease mortality in uncomplicated hypertension. They are first line agents unless a comorbid condition is present
- Periodically test for end-organ complications including renal (BUN, creatinine, urine protein-to-creatinine ratio) and cardiac (ECG evidence of hypertrophy)
54
Primary Hypertension with CHF
| Treatment
- Diuretics
- Beta-blockers
- ACEIs
- ARBs
- Aldosterone antagonists
55
Primary Hypertension with Diabetes
| Treatment
- ACEIs
- ARBs
- Diuretics
- Beta-blockers
- CCBs
56
Primary Hypertension and Post-MI
| Treatment
- Beta-blockers
- ACEIs
- ARBs
- Aldosterone antagonists
57
Primary Hypertension with Chronic kidney disease
| Treatment
- ACEIs
| - ARBs
58
Primary Hypertension with BPH
| Treatment
- Alpha 1-adrenergic blockers
| - Diuretics
59
Isolated Systolic Hypertension
| Treatment
- Diuretics
- ACEIs
- CCBs (dihydropyridines)
60
Atherosclerotic Aneurysms
| Pathology
- Affects abdominal aorta below renal arteries
- Weakening of the media due to atheroma formation
- Associated with hypertension
- Aneurysms more than 6 cm in diameter, half of them will rupture within 10 years
61
Syphilitic Aneurysms
| Pathology
- Affects ascending aorta
- Syphilitic aortitis (tertiary syphilis) will cause obliterative endarteritis of vasa vasorum, leading to ischemia and smooth muscle atrophy of the aortic media
- May lead to dilatation of the aortic valve ring leading to aortic insufficiency
62
Aortic Dissecting Aneurysms
| Pathology
- Longitudinal intimal tear forming a false lumen which leads to cystic medial degeneration
- Risk factors are hypertension, bicuspid aortic valve and Marfan syndrome
63
Aortic Dissecting Aneurysms
| Presentation and Diagnosis
- Sudden onset tearing chest pain radiating to the back and/or markedly unequal BP in arms
- CXR shows mediastinal widening
64
Aortic Dissecting Aneurysms
| Types and Treatment
- Stanford type A (proximal): involves Ascending aorta, and may extend to aortic arch or descending aorta. may result in acute aortic regurgitation or cardiac tamponade. Rx: surgery
- Stanford type B (distal): involves descending aorta and/or aortic arch with no ascending aorta involvement. Rx: medically with beta-blockers then vasodilators
65
Traumatic Aortic Rupture
| Causes, Most common location
- Trauma and/or decelerating injury
| - Aortic isthmus (proximal descending aorta just distal to origin of left subclavian artery)
66
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Berry Aneurysm (Saccular aneurysm)
(Association, Most common sites)
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- Autosomal dominant polycystic kidney disease
- Sites (Circle of Willis):
* Anterior communicating artery
* Posterior communicating artery
* Middle cerebral artery
* Internal carotid artery
* Tip of Basilar artery (most common to rupture)
67
Kaposi Sarcoma
| Facts, Forms
- Low grade malignant tumor of endothelial cells associated with Kaposi sarcoma associated virus (HHV-8). Presents as red-purple patches, plaques or nodules that may remain confined to skin or may dessimenate
- Forms are:
* Classic European form: older men with lesions on lower extremities
* Transplant associated form: with immunosuppression after organ transplant; involves skin and viscera, and may regress with reduction of immunosuppression
* African form: in young children and adult men in whom generalized lymphatic spread is common
