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MSK/Derm > SM_249a: Vasculitis > Flashcards

SM_249a: Vasculitis Flashcards

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1
Q

Vasculitides may be classified by ____

A

Vasculitides may be classified by vessel size

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2
Q

Large vessel vasculitis includes ____ and ____

A

Large vessel vasculitis includes Takayasu arteritis and giant cell arteritis

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3
Q

Giant cell arteritis affects ____ ____ most commonly

A

Giant cell arteritis affects older women most commonly

(most common form of arteritis, average onset 73 years, Northern European ancestry)

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4
Q

Giant cell arteritis clinical features include ____, ____, ____, ____, ____, and ____

A

Giant cell arteritis clinical features include new headache, constitutional symptoms, jaw claudication, symptoms of polymyalgia, partial or complete loss of vision, and arm claudication

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5
Q

Describe giant cell arteritis diagnostics

A

Giant cell arteritis diagnostics

  • Laboratory studies: marked elevation in ESR and CRP, thrombocytosis may be seen
  • Imaging: temporal artery ultrasound, MRA, PET
  • Biopsy: temporal artery, should be done urgently on all patients with suspected GCA, yield remains high even after several weeks of treatment
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6
Q

____ should be done urgently in all patients with suspected giant cell arteritis

A

Temporal artery biopsy on the affected side should be done urgently in all patients with suspected giant cell arteritis

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7
Q

Giant cell arteritis pathology involves ____, ____, ____, and ____

A

Giant cell arteritis pathology involves inflammation often centered on internal elastic lamina, multinucleated giant cells, granulomas, and focal fibrinoid necrosis

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8
Q

Giant cell arteritis treatment includes ____ and ____

A

Giant cell arteritis treatment includes high-dose corticosteroids and tocilizumab (anti IL-6)

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9
Q

Takayasu arterities is ____ but most commonly affects the ____, ____, or ____ arteries

A

Takayasu arteritis is a large vessel vasculitis that can affect any artery but most commony affects aorta, subclavia, or carotid arteries

(pulmonary artery involvement also common)

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10
Q

Takayasu arteritis is characterized by ____, ____, or ____

A

Takayasu arteritis is characterized by segmental stenosis, dilatation, or aneurysm formation

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11
Q

Takayasu arteritis is seen in patients aged ____, ____, and has greatest prevalence in ____

A

Takayasu arteritis is seen in patients aged ≤ 40 years old, females, and has greatest prevalence in Asia

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12
Q

Describe Takayasu arteritis clinical features

A

Takayasu arteritis clinical features

  • Depend on vascular bed
  • Symptoms to suspect Takayasu arteritis in patient ≤ 40: constitutional symptoms (absent in up to 50% at onset), unexplained lightheadedness, extemity pain / claudication, bruits, or absent/diminished pulses or BP difference > 10 mmHg
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13
Q

Takayasu arteritis laboratory findings show ____ and ESR and CRP ____

A

Takayasu arteritis laboratory findings show no specific lab abnormality and ESR and CRP are often elevated but can be negative despite active disease

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14
Q

Takayasu arteritis pathology includes ____, ____, ____, and ____

A

Takayasu arteritis pathology includes granulomatous panarteritis, intimal hyperplasia, fixed stenosis and occlusions, and occasional aneurysm

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15
Q

___ is crucial for Takayasu arteritis diagnosis

A

Imaging is crucial for Takayasu arteritis diagnosis

(noninvasive such as CTA / MRA / PET most common)

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16
Q

Describe Takayasu arteritis treatment

A

Takayasu arteritis treatment

  • High-dose corticosteroids initially: high relapse rate if tapered
  • Methotrexate or azathioprine are preferred steroid-sparing agents to help maintain remission
    Surgical bypass with good results in certain specific situations
  • Angioplasty ± stenting has generally been associated with high failure rates
  • Early recognition and initiation of therapy are critical because aortic regurgiation and acute MI are common and can cause death
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17
Q

Compare and contrast giant cell arteritis and Takayasu arteritis

A

Gziant cell arteritis and Takayasu arteritis

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18
Q

Medium sized vasculitides include ____ and ____

A

Medium sized vasculitides include polyarteritis nodosa and Kawasaki disease

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19
Q

Polyarteritis nodosa is ____, more common in ____, and typically presents between ages ____

A

Polyarteritis nodosa is a systemic necrotizing vasculitis of medium-sized muscular arteries (often at branch points), more common in males, and typically presents between ages 40-60

(but can affect any age group, primary or secondary)

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20
Q

Secondary polyarteritis nodosa is primarily associated with ____

A

Secondary polyarteritis nodosa is primarily associated with HBV

(also HCV, HIV, hairy cell leukemia)

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21
Q

Polyarteritis nodosa involves ____, ____, and ____

A

Polyarteritis nodosa involves transmural inflammation, no giant cells or granulomas, and often dfiferent phases of vasculitis occurring within different vessels or one vessel at the same time

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22
Q

Polyarteritis nodosa clinical features include ____, ____, ____, ____, ____, and ____

A

Polyarteritis nodosa clinical features include cutaneous, renal disease (not glomerular), neuropathy, mesenteric ischemia, orchitis, and myalgia/weakness

  • Cutaneous: nodules, purpura, livedo reticularis, ulcers
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23
Q

Describe polyarteritis nodosa treatment

A

Polyarteritis nodosa treatment

  • Mild: glucocorticoid monotherapy, methotrexate or azathioprine for resistant disease
  • Moderate to severe: glucorticoid and cyclophosphamide
  • Viral associated secondary polyarteritis nodosa: check for HBV, appropriate antiviral therapy (± glucocorticoids and/or immunosuppression)
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24
Q

Kawasaki disease is ____ and that is more common in ____ ____

A

Kawasaki disease is an acute febrile typically self-limited vasculitis seen in young children that is more common in Asian boys

(#1 cause of acquired heart disease in kids, presents betwen 6 months and 5 years of age)

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25
Kawasaki disease clinical manifestations include ____ and \_\_\_\_
Kawasaki disease clinical manifestations include strawberry tongue and erythematous rash
26
Kawasaki disease pathology primarily affects ____ and includes \_\_\_, leading to \_\_\_
Kawasaki disease pathology primarily affects medium-sized muscular arteries (coronary arteries) and includes destruction of luminal endothelial cells, elastic lamina, and smooth muscle cells of media, leading to arterial wall dilatation ± aneurysm formation
27
Kawasaki disease laboratory finds are ____ and include \_\_\_\_, \_\_\_\_, and \_\_\_\_
Kawasaki disease laboratory finds are generally nonspecific and include leukocytosis with left shift, normocytic anemia and often marked thrombocytosis, and transaminitis or mild hyperbilirubinemia due to intrahepatic congestion
28
Kawasaki disease treatment includes ____ and \_\_\_\_
Kawasaki disease treatment includes aspirin and IVIG * Aspirin: initially high-dose until fever resolves, anti-thrombotic dose continued for 6-8 weeks or until lab abnormalities resolve and there are no coronary abnormalities on echo * IVIG: significant reduction in risk of mortality related to coronary artery aneurysms if begun within 1st 10 days
29
ANCA-associated small vessel vasculitis includes \_\_\_\_, \_\_\_\_, and \_\_\_\_
ANCA-associated small vessel vasculitis includes microscopic polyangiitis, granulomatosis with polyangiitis, and eosinophilic granulomatosis with polyangiitis
30
\_\_\_\_ is associated with p-ANCA while ____ is associated with c-ANCA
MPO is associated with p-ANCA while PR3 is associated with c-ANCA
31
Describe pathogenesis of ANCA-associated small vessel vasculitis
ANCA-associated small vessel vasculitis pathogenesis * Activated neutrophils -\> express PR3 or MPO -\> antibodies stick to them -\> release cytokines -\> migrate into vessel wall -\> release more cytokines -\> cause inflammation
32
Granulomatosis with polyangiitis is a ____ vasculitis generally associated with \_\_\_\_, has average age onset of \_\_\_\_, and is more common in ____ patients
Granulomatosis with polyangiitis is a small-medium vasculitis generally associated with c-ANCA, has average age onset of 40-50s with second peak in elderly 70s, and is more common in white patients
33
Granulomatosis with polyangiitis pathology includes ____ and \_\_\_\_
Granulomatosis with polyangiitis pathology includes necrotizing granulomas and few or no immune deposits
34
Describe granulomatosis with polyangiitis clinical features
Granulomatosis with polyangiitis clinical features * Upper airway disease: nasal septum, sinuses, subglottic stenosis * Pulmonary disease: nodules, alveolitis/capillaritis, ILD-fibrosis * Glomerulonephritis * Arthritis/arthralgia * Vascular: gangrene, VTE * Neuropathy: sensory, motor, CNS (rare), sensorineural hearing loss * Ocular disease: scleritis/episcleritis, pseudotumor * Skin/mucosa: oral ulcers, purpura, nodules * Cardiac * GI disease
35
Microscopic polyangiitis is a ____ vasculitis most common in \_\_\_\_
Microscopic polyangiitis is a small vessel vasculitis most common in middle age
36
Microscopic polyangiitis pathology includes \_\_\_\_, \_\_\_\_, and \_\_\_\_
Microscopic polyangiitis pathology includes necrotizing arteritis, few or no immune deposits, and no granulomatous inflammation
37
Microscopic polyangiitis clinical features include \_\_\_\_, \_\_\_\_, \_\_\_\_, \_\_\_\_, \_\_\_\_, and \_\_\_\_
Microscopic polyangiitis clinical features include glomerulonephritis, pulmonary capillaries, peripheral neuropathy, skin lesions, weight loss, and fevers
38
Granulomatosis with polyangiitis and microscopic polyangiitis are treated initially with \_\_\_\_, \_\_\_\_, and \_\_\_\_
Granulomatosis with polyangiitis and microscopic polyangiitis are treated initially with steroids, rituximab or cyclophosphamide for severe disease, and methotrexate for non-severe disease
39
Eosinophilic granulomatosis with polyangiitis has ___ predominance, average age of \_\_\_, is associated with \_\_\_, and has ___ and \_\_\_\_
Eosinophilic granulomatosis with polyangiitis has a mild male predominance, average of 35-45, is associated with p-ANCA in 40% of cases but is often ANCA negative, and has eosinophilia/elevated IgE and adult onset asthma (renal involvement is less common)
40
Eosinophilic granulomatosis with polyangiitis pathology involves \_\_\_\_, \_\_\_\_, and \_\_\_\_
Eosinophilic granulomatosis with polyangiitis pathology involves necrotizing vasculitis, eosinophilic infiltration, and extravascular granuloma
41
Eosinophilic granulomatosis with polyangiitis clinical presentation includes the \_\_\_\_, \_\_\_\_, and ____ phases
Eosinophilic granulomatosis with polyangiitis clinical presentation includes the prodrome, eosinophilic, and vasculitis phases * Prodrome: asthma, allergic rhinitis, nasal polyp * Eosinophilic phase: tissue and periphery * Vasculitis phase: cutaneous, cardiac, neurologic, renal, and gastroenteritis
42
Eosinophilic granulomatosis with polyangiitis is treated initially with \_\_\_\_, \_\_\_\_, and \_\_\_\_
Eosinophilic granulomatosis with polyangiitis is treated initially with glucocorticoids, cyclophosphamide, and meopolizumab (anti IL-5)
43
Compare the contrast the ANCA vasculitides
ANCA vasculitides
44
Immune complex small vessel vasculitis includes \_\_\_\_, \_\_\_\_, \_\_\_\_, and \_\_\_\_
Immune complex small vessel vasculitis includes cryoglobulinemic vasculitis, IgA vasculitis (Henoch-Schonlein), hypocomplementemic urticarial vasculitis, and anti-GBM disease
45
Describe Anti-GBM disease (Goodpasture's)
Anti-GBM disease (Goodpasture's) * Immune complex small vessel vasculitis * Older children and adults * Usually idiopathic but can occur after URI * Diagnosis made by checking anti-GBM antibodies
46
Anti-GBM disease (Goodpasture's) clinical presentation includes \_\_\_\_, \_\_\_\_, and \_\_\_\_
Anti-GBM disease (Goodpasture's) clinical presentation includes acute renal failure, alveolar hemorrhage, and NO constitutional symptoms (can occur in addition to ANCA vasculitis)
47
Anti-GBM disease (Goodpasture's) is treated with \_\_\_\_, \_\_\_\_, and \_\_\_\_
Anti-GBM disease (Goodpasture's) is treated with plasmapheresis, glucocorticoids, and cyclophosphamide
48
Describe cryoglobulinemia
Cryoglobulinemia * Cryoglobulins: immunoglobulins or mixtures of immunoglobulins and complement that precipitate at temperatures less than 37C * 3 types: Type 1 - monoclonal Ig, Type II - mixed monoclonal Igs with and polyclonal Ig, Type III - polyclonal IgG and IgM * Hyperviscositiy (digital ischemia, livedo reticularis, skin necrosis) vs vasculitis (arthralgias, fatigue, myalgia, palpable purpura, peripheral neurpathy, glomerulonephritis) * Associated conditions: Type I is MGUS or B cell malignancy, Type II is HCV / HBV / HIV / autoimmune disease * Treatment depends on underlying condition and severity of disease
49
Cryoglobulinemia type II is associated with \_\_\_\_
Cryoglobulinemia type II is associated with Hepatitis C
50
IgA vasculitis (Henoch-Schonlein purpura) most commonly affects ____ and 2/3 of cases present after \_\_\_\_
IgA vasculitis (Henoch-Schonlein purpura) most commonly affects children and 2/3 of cases present after URI (children have better prognosis)
51
IgA vasculitis (Henoch-Schonlein purpura) pathology includes \_\_\_\_, \_\_\_\_, and \_\_\_\_
IgA vasculitis (Henoch-Schonlein purpura) pathology includes IgA and C3 complexes in blood vessel, granulocytes in walls of arterioles or venules, and necrotizing lesions
52
IgA vasculitis (Henoch-Schonlein purpura) clinical features include \_\_\_\_, \_\_\_\_, \_\_\_\_, and \_\_\_\_
IgA vasculitis (Henoch-Schonlein purpura) clinical features include rash / purpura, intestines (abdominal pain prior to rash, vomiting diarrhea), joint pains/myalgias, and kidney involvement (hematuria)
53
IgA vasculitis (Henoch-Schonlein purpura) treatment includes ___ and \_\_\_
IgA vasculitis (Henoch-Schonlein purpura) treatment includes supportive care and glucorticoids (glucocorticoids: pulse steroids for severe disease, used to prevent nephritic / nephrotic CKD)
54
Variable vessel vasculitis includes \_\_\_\_
Variable vessel vasculitis includes Behcets syndrome
55
Behcets syndrome is a ____ vasculitis with highest prevalence in ____ and onset at \_\_\_\_
Behcets syndrome is a mixed vessel vasculitis with highest prevalence in Mediterranean / Middle East / Far East and onset at 3rd-4th decade
56
Describe Behcets disease clinical features
Behcets disease clinical features * Oral ulcers * Genital ulcers * Cutaneous lesions (including pathergy) * Ocular involvement * Cardiovascular * Arthralgias * Neurologic * Gastrointestinal * Inner ear involvement
57
Behcets disease treatment is \_\_\_\_
Behcets disease treatment is organ based * Topical glucorticoids, colchicine, apremilast (phosphodiesterase 4 inhibitor), systemic glucocorticoids, azathioprine, TNF inhibitors, and cyclophosphamide
58
Secondary vasculitis etiology includes \_\_\_\_, \_\_\_\_, \_\_\_\_, and \_\_\_\_
Secondary vasculitis etiology includes infectious, drug-induced, connective tissue disease, and malignancy associated
59
Vasculitis mimics include \_\_\_, \_\_\_, and \_\_\_
Vasculitis mimics include congenital vascular disorders, atheroembolic disease, and thrombotic disorders
60
Describe the approach for vasculitis
Approach for vasculitis
61
Most likely diagnosis is \_\_\_\_
Most likely diagnosis is IgA vasculitis (Henoch-Schonlein purpura)
62
Most likely diagnosis is \_\_\_\_
Most likely diagnosis is granulomatosis with polyangiitis
63
Most likely diagnosis is \_\_\_\_
Most likely diagnosis is granulomatosis with polyangiitis

MSK/Derm (49 decks)

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