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
Q
Kawasaki Disease (Mucocutaneous lymph node syndrome) (Complications)
A
- Coronary artery aneurysm
- Death (1-2%) due to rupture of coronary artery aneurysm or coronary thrombosis
26
Q
Kawasaki Disease (Mucocutaneous lymph node syndrome) (Diagnosis)
A
- Diagnosed clinically
- Autopsy:
- Involvement of medium vessels
- Segmental necrotizing vasculitis with coronary arteries being the most affected (70%)
