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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
Q

Kawasaki disease clinical manifestations include ____ and ____

A

Kawasaki disease clinical manifestations include strawberry tongue and erythematous rash

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26
Q

Kawasaki disease pathology primarily affects ____ and includes ___, leading to ___

A

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
Q

Kawasaki disease laboratory finds are ____ and include ____, ____, and ____

A

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
Q

Kawasaki disease treatment includes ____ and ____

A

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
Q

ANCA-associated small vessel vasculitis includes ____, ____, and ____

A

ANCA-associated small vessel vasculitis includes microscopic polyangiitis, granulomatosis with polyangiitis, and eosinophilic granulomatosis with polyangiitis

30
Q

____ is associated with p-ANCA while ____ is associated with c-ANCA

A

MPO is associated with p-ANCA while PR3 is associated with c-ANCA

31
Q

Describe pathogenesis of ANCA-associated small vessel vasculitis

A

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
Q

Granulomatosis with polyangiitis is a ____ vasculitis generally associated with ____, has average age onset of ____, and is more common in ____ patients

A

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
Q

Granulomatosis with polyangiitis pathology includes ____ and ____

A

Granulomatosis with polyangiitis pathology includes necrotizing granulomas and few or no immune deposits

34
Q

Describe granulomatosis with polyangiitis clinical features

A

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
Q

Microscopic polyangiitis is a ____ vasculitis most common in ____

A

Microscopic polyangiitis is a small vessel vasculitis most common in middle age

36
Q

Microscopic polyangiitis pathology includes ____, ____, and ____

A

Microscopic polyangiitis pathology includes necrotizing arteritis, few or no immune deposits, and no granulomatous inflammation

37
Q

Microscopic polyangiitis clinical features include ____, ____, ____, ____, ____, and ____

A

Microscopic polyangiitis clinical features include glomerulonephritis, pulmonary capillaries, peripheral neuropathy, skin lesions, weight loss, and fevers

38
Q

Granulomatosis with polyangiitis and microscopic polyangiitis are treated initially with ____, ____, and ____

A

Granulomatosis with polyangiitis and microscopic polyangiitis are treated initially with steroids, rituximab or cyclophosphamide for severe disease, and methotrexate for non-severe disease

39
Q

Eosinophilic granulomatosis with polyangiitis has ___ predominance, average age of ___, is associated with ___, and has ___ and ____

A

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
Q

Eosinophilic granulomatosis with polyangiitis pathology involves ____, ____, and ____

A

Eosinophilic granulomatosis with polyangiitis pathology involves necrotizing vasculitis, eosinophilic infiltration, and extravascular granuloma

41
Q

Eosinophilic granulomatosis with polyangiitis clinical presentation includes the ____, ____, and ____ phases

A

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
Q

Eosinophilic granulomatosis with polyangiitis is treated initially with ____, ____, and ____

A

Eosinophilic granulomatosis with polyangiitis is treated initially with glucocorticoids, cyclophosphamide, and meopolizumab (anti IL-5)

43
Q

Compare the contrast the ANCA vasculitides

A

ANCA vasculitides

44
Q

Immune complex small vessel vasculitis includes ____, ____, ____, and ____

A

Immune complex small vessel vasculitis includes cryoglobulinemic vasculitis, IgA vasculitis (Henoch-Schonlein), hypocomplementemic urticarial vasculitis, and anti-GBM disease

45
Q

Describe Anti-GBM disease (Goodpasture’s)

A

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
Q

Anti-GBM disease (Goodpasture’s) clinical presentation includes ____, ____, and ____

A

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
Q

Anti-GBM disease (Goodpasture’s) is treated with ____, ____, and ____

A

Anti-GBM disease (Goodpasture’s) is treated with plasmapheresis, glucocorticoids, and cyclophosphamide

48
Q

Describe cryoglobulinemia

A

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
Q

Cryoglobulinemia type II is associated with ____

A

Cryoglobulinemia type II is associated with Hepatitis C

50
Q

IgA vasculitis (Henoch-Schonlein purpura) most commonly affects ____ and 2/3 of cases present after ____

A

IgA vasculitis (Henoch-Schonlein purpura) most commonly affects children and 2/3 of cases present after URI

(children have better prognosis)

51
Q

IgA vasculitis (Henoch-Schonlein purpura) pathology includes ____, ____, and ____

A

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
Q

IgA vasculitis (Henoch-Schonlein purpura) clinical features include ____, ____, ____, and ____

A

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
Q

IgA vasculitis (Henoch-Schonlein purpura) treatment includes ___ and ___

A

IgA vasculitis (Henoch-Schonlein purpura) treatment includes supportive care and glucorticoids

(glucocorticoids: pulse steroids for severe disease, used to prevent nephritic / nephrotic CKD)

54
Q

Variable vessel vasculitis includes ____

A

Variable vessel vasculitis includes Behcets syndrome

55
Q

Behcets syndrome is a ____ vasculitis with highest prevalence in ____ and onset at ____

A

Behcets syndrome is a mixed vessel vasculitis with highest prevalence in Mediterranean / Middle East / Far East and onset at 3rd-4th decade

56
Q

Describe Behcets disease clinical features

A

Behcets disease clinical features

  • Oral ulcers
  • Genital ulcers
  • Cutaneous lesions (including pathergy)
  • Ocular involvement
  • Cardiovascular
  • Arthralgias
  • Neurologic
  • Gastrointestinal
  • Inner ear involvement
57
Q

Behcets disease treatment is ____

A

Behcets disease treatment is organ based

  • Topical glucorticoids, colchicine, apremilast (phosphodiesterase 4 inhibitor), systemic glucocorticoids, azathioprine, TNF inhibitors, and cyclophosphamide
58
Q

Secondary vasculitis etiology includes ____, ____, ____, and ____

A

Secondary vasculitis etiology includes infectious, drug-induced, connective tissue disease, and malignancy associated

59
Q

Vasculitis mimics include ___, ___, and ___

A

Vasculitis mimics include congenital vascular disorders, atheroembolic disease, and thrombotic disorders

60
Q

Describe the approach for vasculitis

A

Approach for vasculitis

61
Q

Most likely diagnosis is ____

A

Most likely diagnosis is IgA vasculitis (Henoch-Schonlein purpura)

62
Q

Most likely diagnosis is ____

A

Most likely diagnosis is granulomatosis with polyangiitis

63
Q

Most likely diagnosis is ____

A

Most likely diagnosis is granulomatosis with polyangiitis

MSK/Derm (49 decks)

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