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Vasculitis Flashcards

1
Q

What are the three principal layers of arteries and veins?

A

Intima, media, and adventitia

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2
Q

What are elastic arteries?

A

The aorta and all immediate branches that contain elastin in the tunica media and allow expainson/recoil with ventricular systole and diastole

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3
Q

What are muscular arteries?

A

Arteries that contain predominantly smooth muscle inthe tunica media and are subject to autonomic control

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4
Q

What are arterioles?

A

Resistance vessels of the body that have the highest smooth muscle/diameter ratio

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5
Q

What is the difference between a primary and secondary vasculitis?

A

Primary = comprised of diseases with proven or presumed autoimmune etiology

Secondary = disease secondary to infection, drugs, connective tissue disease, or malignancy

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6
Q

What are two examples of large vessel vasculitis?

A

Giant cell arteritis, takayasu arteritis

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7
Q

What are two examples of medium vessel vasculitis?

A

Polyarteritis nodosa and kawasaki disease

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8
Q

What are three examples of ANCA-associated vasculitis?

A

Granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis

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9
Q

What are four examples of immune complex small vessel vasculitis?

A

Anti-glomerular basement membrane disease (Goodpasture’s disease), cryoglobulinemic vasculitis, IgA vasculitis (Henoch-Schonlein purpura), and hypocomplementemic urticarial vasculitis

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10
Q

What are two examples of variable vessel vasculitis?

A

Behcet’s disease and Cogan’s syndrome

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11
Q

What are five examples of single organ vasculitis?

A

cutaneous leukocytoclastic angiitis, cutaneous arteritis, primary central nervous system vasculitis, isolated aortitis, and renal limited vasculitis

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12
Q

What are four examples of secondary systemic vasculitis?

A

Infectious vasculitis, drug-induced, connective tissue disease associated, and malignancy associated

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13
Q

What are the most susceptible demographics for giant-cell arteritis?

A

Above age 50, women more than men

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14
Q

What is giant-cell arteritis?

A

The most common form of arteritis, affects large cranial arteries including temporal artery

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15
Q

What is the clinical presentation of giant-cell arteritis?

A

Headache, jaw claudication, scalp tenderness, vision loss, and limb claudication

Can also present with hip and shoulder stiffness when associated with polymyalgia rheumatica

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16
Q

What are the pathological findings of giant-cell arteritis?

A

Granulomatous inflammation

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17
Q

What are the treatments for giant-cell arteritis?

A

First line = glucocorticoids

Steroid-sparing agents = Tocilizumab (targets IL-6 pathway)

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18
Q

What demographis are most susceptible to Takayasu arteritis?

A

Young women (under 40), people of Asian descent

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19
Q

What vessels are primarily affected by Takayasu arteritis?

A

Ascending aorta, its immediate branches, and the subclavian arteries

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20
Q

What is the pathology of Takayasu arteritis?

A

Granulomatous lesions with intimal fibrosis leading to luminal obstruction

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21
Q

What is the presentation of Takayasu arteritis?

A

Diminished peripheral pulses, constitutional symptoms, neurologic symptoms, and claudication

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22
Q

What is the treatment for Takayasu arteritis?

A

Glucocorticoids

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23
Q

What demographics are most susceptible to polyarteritis nodosa?

A

Middle-aged individuals

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24
Q

What is the pathogenesis of polyarteritis nodosa?

A

It affects small- to medium-sized arteries and is characterized by acute segmental transmural necrotizing inflammation of muscular arteries

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25
What are the causes of polyarteritis nodosa?
Idiopathic (most cases) or secondary to hep B, hep C, or hairy cell leukemia
26
What is the clinical presentation of polyarteritis nodosa?
Constitutional symptoms (fever, weight loss), mononeuritis multiplex (peripheral neuropathy), skin nodules, hematuria, new-onset hypertension, abdominal pain, occiasionally muscle involvement, and orchitis (testicle inflammation)
27
What is the treatment for polyarteritis nodosa?
initially glucocorticoids, progress to cyclophosphamide if severe case
28
What demographics are most susceptible to Kawasaki disease?
Mostly children under age 4
29
What is the pathogenesis of Kawasaki disease?
Arteritis affecting large- to medium-sized vessels with genetic, infectious, and environmental factors playing a role
30
What is the clinical presentation of Kawasaki disease?
Fever, conjunctivitis, strawberry tongue, lymphadenitis, and desquamative skin rash
31
What is the consequence of untreated Kawasaki disease?
20% risk of coronary artery ectasia (dilation) and aneurysms that are prone to thrombosis or rupture (leading to MI or death)
32
What is the treatment for Kawasaki disease?
Intravenous immunoglobulin and aspirin
33
What demographics are most susceptible to granulomatosis with polyangiitis?
Middle-aged adults
34
What is the pathology of granulomatosis with polyangiitis?
Necrotizing granulomas in small- and medium-sized vessels
35
What is the clinical presentation of granulomatosis with polyangiitis?
Oral ulcers, bloody nasal discharge/crusting, chronic sinusitis, subglottic stenosis,recurrent otitis media, pulmonary infiltrates, pulmonary nodules, alveolar hemmorhage, pauci-immune glomerulonephritis, constitutional symptoms ## Footnote *can also affect any organ*
36
What antibody is associated with granulomatosis with polyangiitis?
c-ANCA
37
What is the treatment for granulomatosis with polyangiitis?
High dose glucocorticoids with cyclophosphamide or rituximab (targets B cells)
38
What demographics are most susceptible to microscopic polyangiitis?
Middle-aged adults
39
What is the pathogenesis of microscopic polyangiitis?
A necrotizing vasculitis that affects small-caliber vessels (capillaries, arterioles, and venules) thatdoes not have granulomas
40
What is the clinical presentation of microscopic polyangiitis?
Constitutional symptoms, renal involvement (hematuria, rise in creatinine, RBC casts), rapidly progressive pauci-immune glomerulonephritis, pulmonary manifestations (diffuse alveolar hemorrhage presenting as dyspnea, cough, hemoptysis, etc), PNS involvement
41
What antibody is associated with microscopic polyangiitis?
p-ANCA
42
What is the treatment for microscopic polyangiitis?
Glucocorticoids, cyclophosphamide, and rituximab (targets B cells)
43
Can you have polyangiitis without ANCA positivity?
Yes - it is most common to have ANCA positivity corresponding to the condition, but it is not always present
44
What happens in the prodromal phase of eosinophilic granulomatosis with polyangiitis?
Asthma, allergic rhinitis, and nasal polyps
45
What happens in the eosinophilic phase of eosinophilic granulomatosis with polyangiitis?
Peripheral eosinophilia and eosinophilic tissue infiltration
46
What happens in the vasculitic phase of eosinophilic granulomatosis with polyangiitis?
There are manifestations that can include cutaneous lesions, cardiac involvement, heart failure, pericarditis, neurologic manifestations, renal involvement, eosinophilic gastroenteritis
47
What antibody is associated with eosinophilic granulomatosis with polyangiitis?
p-ANC, though less associatedthan in other polyangiitis conditions
48
What are the treatments for eosinophilic granulomatosis with polyangiitis?
Glucocorticoids, cyclophosphamide, and mepolizumab (targets IL-5)
49
What demographics are most affected by anti-GBM disease?
Older children and adults
50
What is anti-GBM disease?
An immune complex that affects small vessels and is usually idiopathic but can also occur after pulmonary infection
51
What is the clinical presentationof anti-GBM disease?
Acute renal failure (proteinuria, hematuria, casts), frequent pulmonary involvement (alveolar hemorrhage)
52
What antibodies are associated with anti-GBM disease?
anti-GBM antibodies, sometimes ANCAs if there is a concomitant ANCA associated vasculitis
53
What is the treatment for anti-GBM disease?
Plasmapheresis, glucocorticoids, and cyclophosphamide
54
What is cryoglobulinemic vasculitis?
An immune complex small-vessel vasculitis where immunoglobulins and complement are found in the vessel wall
55
What is the most common cause of cryoglobulinemic vasculitis?
Chronic hepatitis C infection
56
What is the clinical presentation of cryoglobulinemic vasculitis?
Cutaneous purpura, arthralgia/arthritis, neuropathy, renal involvement
57
What are the renal biopsy findings of cryoglobulinemic vasculitis?
Intraluminal thrombi, diffuse IgM on capillary loops, and subendothelial deposits on EM
58
What is the treatment for cryoglobulinemic vasculitis?
Rituximab (targets B cells) and glucocorticoids
59
What demographics are most susceptible to IgA vasculitis?
Children aged 3-15
60
What is the pathology of IgA vasculitis?
Necrotizing lesions with IgA deposit in the capillaries an dpost-capillary venules in small vessels
61
What often preceeds disease onset of IgA vasculitis?
Upper respiratory infection
62
What is the clinical presentation of IgA vasculitis?
Skin involvement (palpable purpura on extensor surfaces of arms, legs, and buttocks), arthritis of lower extremities, abdominal pain, nephritis, nephrotic syndrome, or hematuria
63
What is the treatment for IgA vasculitis?
Supportive care, glucocorticoids if there is renal involvement to prevent progression to CKD
64
What demographics are most susceptible to Behcets syndrome?
Most common in Turkey and along the silk road (mediterranean to eastern Asia) in people aged 20-40
65
What vessels are affected by Behcets syndrome?
Vessels of all sizes, both arteries and veins
66
What is the clinical presentation of Behcets syndrome?
Painful oral ulcers, genital ulcers, cutaneous lesions, uveitis, arthritis, vascular disease, and neurologic involvement
67
What is the treatment for Behcets syndrome?
Depends on which organ is involved
68
What disease is associated with hep B?
Polyarteritis nodosa
69
What disease is associated with mononeuritis multiplex?
Polyarteritis nodosa
70
What disease is associated with hip and shoulder weakness?
Giant-cell arteritis ## Footnote *due to association with polymyalgia rheumatica*
71
What disease is associated with strawberry tongue?
Kawasaki disease
72
What disease is associated with PR3-ANCA positivity?
Granulomatosis with polyangiitis (also called c-ANCA)
73
What disease is associated with MPO-ANCA positivity?
Microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis (to a lesser extent)
74
What disease is associated with asthma?
Eosinophilic granulomatosis with polyangiitis
75
What disease involves aspirin as a specific therapy?
Kawasaki disease
76
What disease is associated with frequent URI?
Granulomatosis with polyangiitis
77
What disease is associated with nasal polyps?
Eosinophilic granulomatosis with polyangiitis
78
What disease most commonly affects women of Asian descent?
Takayasu arteritis
79
What disease causes jaw claudication?
Giant-cell arteritis
80
What disease causes a desquamative skin rash?
Kawasaki disease
81
A 9 y.o. boy presents with abdominal pain and dark urine for a week. Exam shows purpuric lesions on his ankles and buttock. Serologic tests are negative for p-ANCA and c-ANCA. Skin biopsy shows necrotizing vasculitis of small dermal vessels. Renal biopsy shows immune complex deposition with IgA rich immune complexes. Which is the most likely diagnosis? a) Giant cell arteritis b) Henoch-Schonlein purpura b) PAN d) Takayasu e) Kawasaki's disease f) Telaniectasias g) GPA
b) Henoch-Schonlein purpura ## Footnote *also known as IgA vasculitis*
82
A 70 y.o. white man with chronic cough for past year. Exam shows nasal ulcers and lungs with diffuse crackles. Creatinine = 4.5 Urinalysis shows 20 RBCs +casts c-ANCA is positive CXR has small scattered pulmonary nodules, lung biopsy shows vasculitis of small peripheral arteries/arterioles + granulomatous inflammation adjacent to small arterioles What is the most likely diagnosis? a) fibromuscular dysplasia b) PAN c) takayasus d) GPA
d) GPA
83
A 34 y.o. woman is evaluated for pain and redness of her eye, double vision, and ocular movement impairment for the past 6 days. ESR is high, thyroid function studies/urinalysis/CXR are normal. MRI of orbit reveals superior oblique enlargement and enfiltration with edema. Testing for ANCA is negative. Biopsy of superior oblique muscle shows granulomatous inflammation,tissue necrosis, and capillaritis. What is the most likely diagnosis? a) GPA b) sarcoidosis c) Grave's thyroiditis d) lymphoma
a) GPA ## Footnote *it can be GPA even if ANCAs are negative*
84

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