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Dermatology Board Prep > DERM (20) Vascular Disorders > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

What are the 4 vessel size classifications of vasculitis?

A

Small, mixed small and medium vessel, medium vessel and large vessel

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2
Q

What are the primary clinical features of small vessel vasculitis?

A

Palpable purpura, petechiae, vesicles, pustules

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3
Q

What are the hallmark clinical features of mixed medium and small-vessel vasculitis?

A

Mixture of features from small/medium vasculitis

-palpable purpura, petechiae, vesicles, pustules, livido reticularis, retiform purpura, ulcers, subcutaneous nodules

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4
Q

What are the hallmark clinical features of medium vessel vasculitis?

A

Livido reticularis, Subcutaneous nodules, retiform purpura, ulcers

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5
Q

What are the hallmark clinical features of large vessel vasculitis?

A

Specific to dz

Temporal arteritis: erythematous tender nodules or ulceration on the frontotemporal scalp

Takayasu’s arteritis: erythematous, subcutaneous nodules, PG-like lesions on the lower extremity (more so than upper)

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6
Q

What are the 6 main groups of small vessel vasculitis?

A

Henoch-schonlein purpura, Acute hemorrhagic edema of infancy, urticarial vasculitis, erythema elevatum diutinum, granuloma faciale, secondary vasculitis (drug, infection, malignancy, autoimmune)

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7
Q

What are the main groups of mixed small and medium-sized vessel vasculitis?

A

Mixed cryoglobulinemia types II and III, ANCA-associated vasculitis: Microscopic polyangitis, Wegener’s granulomatosis, Chrug-Strauss syndrome

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8
Q

What are the hallmark dz’s of medium vessel vasculitis?

A

Polyarteritis nodosa and Kawasaki dz

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9
Q

What are the two main large-vessel vasculitides?

A

Temporal arteritis, Takayasu’s arteritis

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10
Q

What are the 5 main triggers of small vessel vasculitis?

A

infection (15-20%), inflammatory disorders (15-20%), Drug (10-15%), Neoplasm (<5%), other (thrombotic, embolic, cryoglobulinemia

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11
Q

Who gets cutaneous small vessel vasculitis most commonly?

A

Adults> children

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12
Q

What is the pathophysiology of cutaneous small vessel vasculitis?

A

Immune complex deposition in post-capillary venules which activates complement leading to a neutrophilic inflammatory response

  • This causes vessel damage, hemorrhage, and tissue ischemia
  • Fibrinoid necrosis of blood vessels arises via lysosomal enzymes (collagenases and elastases) and reactive oxidative species

This is why lower extremity is the most common, these deposits follow gravity, settle into lower extremity vessels

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13
Q

What are the most common infections associated with small-vessel vasculitis?

A

Bacterial: group A β-hemolytic Streptococci, Staphylococcus aureus, Chlamydia, Neisseria, Mycobacterium

Viral: hepatitis C > B ≫ A, HIV Fungal/yeast: Candida

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14
Q

What are the most common connective tissue diseases to cause small vessel vasculitis?

A

SLE, Sjogren’s, RA >>DM, scleroderma, polychondritis

IBD

Behcet’s

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15
Q

What are the most common drugs to cause small-vessel vasculitis?

A

Antibiotics: β-lactams (penicillin, cephalosporins), sulfonamides, minocycline, quinolones
Antiinflammatory: NSAIDS, COX-2 inhibitors

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16
Q

Most common neoplasms to be a/w small vessel vasculitis?

A

Hematologic malignancy (MM, monoclonal gammopathies) T-cell leukemia, MF, AML, CML, diffuse large cell leukemia, hairy cell leukemia

Solid-organ ca are much less commonly associated

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17
Q

What is the presentation of small vessel vasculitis?

A

Crops of partially blanchable, symmetric, palpable purpura on the lower extremities, dependent areas, and under tight clothing

Other manifestations: erythematous papules, urticaria,

vesicles, pustules, and livedo reticularis

Rarely happens on the face, palms, soles, or mucous membranes

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18
Q

What is the timing usually from exposure to onset of small vessel vasculitis lesions?

A

7-10 days

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19
Q

How long does small vessel vasculitis usually take to resolve?

A

3-4 weeks w/ some hyperpigmentation or atrophy

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20
Q

What kind of symptoms may be a/w small vessel vasculitis?

A

Skin: Asymptomatic, pruritus, or burning/pain

Systemic: fever, malaise, arthralgias, myalgias, and GI/GU sx’s.

If you note systemic sx’s need to consider a systemic vasculitis

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21
Q

In what percentage of patients is the small vessel vasculitis chronic and follows a relapsing course?

A

10%

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22
Q

What is the timing of the DIF and H&E biopsies to maximize diagnostic yield?

A

H&E: within 18-48 hrs

DIF: 8-24 hrs

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23
Q

What will be the DIF findings in small vessel vasculitis?

A

80% w/ perivascular C3 and IgM

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24
Q

What is the histology of small vessel vasculitis?

A

Perivascular neutrophilic infiltrate (w/ leukocytoclasis) centered around post-capillary venules w/ fibrinoid necrosis of vessel walls and endothelial swelling, and RBC extravasation

Concomitant involvement of the deeper larger vessels would suggests systemic vasculitis

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25
Q

What lab values would be suggestive of more systemic disease?

A

Elevated ESR and significant complement consumption

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26
Q

What should be done if there is suspicion of systemic involvement?

A

CBC (eosinophilia in CSS), BMP (looking for creatine), ESR (>40?), LFTs, UA, serial UA’s, hepatitis panel, ASO titer, CXR, ANA, RF, C3,C4, CH50, C1q, ANCA, anti-phospholipid antibodies, SPEP/UPEP, blood smear, cryoglobulins

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27
Q

What is the treatment approach to mild cases of small vessel vasculitis?

A

Supportive measures: 90% will have a spontaneous resolution, 10% will have a chronic course

Remove suspected meds

Leg elevation and compression stockings NSAIDS for arthralgias is controversial H1 blockers, H2 blockers
Topical steroids

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28
Q

What is the treatment approach to chronic or moderate cases of small vessel vasculitis?

A

Colchicine (0.6 mg, 2–3x/day)
Dapsone (100–200 mg/day)
Combination of colchicine + dapsone or

colchicine + pentoxifylline is more efficacious than monotherapy

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29
Q

What is the treatment for severe small vessel vasculitis w/ ulceration?

A

Oral prednisone (0.5–1 mg/kg with a 4–6 wk taper)

Can add immunosuppressive agents including: Azathioprine (1–2 mg/kg a day) Mycophenolate mofetil (up to 2g daily) Cyclophosphamide (1–2 mg/kg a day) Cyclosporine (2.5–5 mg/kg a day)

IVIG (in an immunodeficient patient) Plasmapheresis (in refractory cases)

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30
Q

What is the epidemiology of Henoch-Schonlein purpura (IgA vasculitis)

A

M/c pediatric vasculitis

  • 90% of cases occur in children, often <10 y/o w/ male’s getting more often
  • Tends to occur in the winter
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31
Q

What is the pathophysiology of Henoch-Schonlein purpura?

A

IgA vascular deposition in small blood vessels results in:

  • Activation of several cytokines
  • Neutrophil activation of nitric oxide and ROS
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32
Q

What are the most common triggers of Henoch-schonlein purpura?

A
  • Occurs 1–2 weeks after a URI or Streptococcus infection
  • Other infections: Bartonella henselae, Parvovirus B19, S. aureus, H. pylori, and Coxsackievirus
  • Drug exposure reported in a minority of patients
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33
Q

What is the tetrad of the clinical presentation of Henoch-Sconlein Purpura?

A

Palpable purpura on the Buttocks and lower extremities (100)

Musculoskeletal: arthralgias (75%), arthritis of the knees and ankles

GI: Colicky abdominal pain (65%), diarrhea, hematochezia

Renal (40-50%): hematuria w/ risk of nephritis –> end-stage renal failure seen in 1-3%

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34
Q

How many of those with Henoch-Scholein Purpura get end-stage renal failure?

A

1-3%

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35
Q

What is the difference in prognosis for HSP in adults?

A

More likely to have an aggressive course w/ diarrhea and chronic renal insufficiency

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36
Q

What are two important predictors of IgA glomerulonephritis in adult pts w/ HSP?

A

Elevated ESR, fever, and purpura above the waist

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37
Q

What types of neoplasms are more a/w HSP in adults?

A

More commonly a/w solid organ tumors (especially lung cancer) over hematologic ones

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38
Q

What percent of adult HSP recurs?

A

40%

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39
Q

What percent of children with HSP get severe renal dz?

A

40%

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40
Q

What is a significant predictor of nephritis in children w/ HSP?

A

Abdominal pain

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41
Q

When should prednisone and or cyclosporine be added to HSP?

A

If there is abdominal pain, arthritis, or severe nephritis

These are largely symptomatic, it is controversial if prednisone is preventative of renal dz (Cochrane review did not find enough evidence to support benefit)

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42
Q

What is the role of ranitidine in the tx of HSP?

A

If there is abdominal pain it can decrease duration and severity

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43
Q

When should IVIG be considered in HSP?

A

In the setting of rapidly progressive glomerulonephritis

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44
Q

What is the DIF findings in HSP?

A

IgA in blood vessel walss

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45
Q

What type of follow-up is needed in HSP?

A

Long term f/u w/ serial UA’s required (hematuria, proteinuria, and abnormal creatine)

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46
Q

What is seen in the histology of HSP?

A

LCV

DIF = IgA deposits, C3, and fibrin in dermal small blood vessels

rate of renal dz is higher if no eos are seen on skin bx

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47
Q

What is the epidemiology of acute hemorrhagic edema of infancy?

A

Children <3 y/o

70% are boys

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48
Q

what is the pathophysiology of acute hemorrhagic edema of infancy?

A

Immune complex deposition in small blood vessels (similar to HSP but no systemic)

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49
Q

Clinical presentation of acute hemorrhagic edema of infancy?

A

Large annular or targetoid/cockade, edematous, hemorrhagic plaques on the head (cheeks and ears) and upper extremities

*spares trunk

Tender non-pitting acrofacial edema

Not ill appearing

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50
Q

What is the prognosis of acute hemorrhagic edema of infancy?

A

Resolves in 1-3 weeks

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51
Q

What is the histology of acute hemorrhagic edema of infancy?

A

LCV, DIF can show IgA

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52
Q

Treatment for acute hemorrhagic edema of infancy?

A

Can give histamines for sx’s but largely supportive

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53
Q

What are the two major types of urticarial vasculitis?

A

Normocomplemetemic (70-80%) and hypocomplementemic (20-30%)

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54
Q

What is the difference in prognosis between normocomplementemic and hypocomplementemic urticarial vasculitis?

A

The normocomplementemic version is skin-limited and idiopathic, the hypocomplementemic version is highly a/w systemic dz

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55
Q

What is the epidemiology of urticarial vasculitis?

A

Females > 50 y/o are the most common people to get this especially the hypocomplementemic variety

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56
Q

What is the pathophysiology of urticarial vasculitis?

A

Complement and immune complex deposition in blood vessel wall and activation of the complement cascade

Hypocomplementemic form: IgG antibodies bind C1q leading to reduced serum levels of C1q

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57
Q

What autoimmune dz are most often a/w urticarial vasculitis and what subtype are they most commonly associated with?

A

SLE (hypocomplementemic) and Sjogren’s (both)

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58
Q

What infections are associated with urticarial vasculitis?

A

Hepatitis B/C, EBV

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59
Q

What medications are most often a/w urticarial vasculitis?

A

NSAIDs, MTX, TNF-α inhibitors, Cimetidine, Fluoxetine, Potassium iodide

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60
Q

What malignancies are most often a/w urticarial vasculitis?

A

Leukemia/lymphoma and gammopathies (IgM and IgG)

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61
Q

What is the clinical presentation of urticarial vasculitis?

A

Painful/burning urticarial lesions lasting >24 hrs (vs < 24 hrs for normal urticaria) on trunk and LE; resolves w/ hyperpigmentation or purpura; may have concomitant angioedema

Recurrent episodes lasting months to years

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62
Q

What are the most common systemic symptoms/findings in urticarial vasculitis?

A

Most commonly seen in the hypocomplementemic version

MSK (50%): Arthralgias, myalgias

GI (15-30%): recurrent abdominal pain, diarrhea, N/V

Pulm (20%): SOB, severe COPD, laryngeal edema

Renal (20-30%): glomerulonephritis or interstitial nephritis

Ocular (10%): Uveitis, conjunctivitis, episcleritis

Constitutional sxs: fever, malaise, arthralgias, myalgias

63
Q

What lab findings help distinguish the hypocomplementemic version from the normocomplementemic version?

A

Decreased CH50, C3 and C4; anti-C1Q Ab (~100% of HUV patients) and elevated ESR

-Should check ANA given strong association of HUV with SLE

(up to 50%)

64
Q

What is Schnitzler’s syndrome?

A

urticarial vasculitis + IgM gammopathy + two of the following: fever, arthralgia, bone pain, elevated ESR, or elevated WBC

65
Q

What is the treatment of primary urticarial vasculitis?

A

Antihistamines, oral steroids, indomethacin

alternatives: Dapsone, Colchicine, hydroxychloroquine

66
Q

What is the treatment of severe urticarial vasculitis?

A

Prednisone + MMF

Rituximab

IVIG

67
Q

What is the most common association with erythema elevatum diutinum?

A

HIV

68
Q

What is the epidemiology of erythema elevatum diutinum?

A

Rare

Chronic condition of middle-aged and older pts

A/w HIV

69
Q

Clinical presentation of erythema elevatum diutinum?

A

Early lesions: red-brown violaceous papulonodules and plaques on extensor surfaces and near joints

  • Later lesions: firm nodules and masses at previously inflamed sites
  • Systemic associations: ocular (scleritis/uveitis) and arthralgias
70
Q

What is the histology of erythema elevatum diutinum?

A

Early: LCV w/ interstitial neuts resembling neutrophilic dermatoses

Late: perivascular storiform fibrosis (onion skin fibrosis) around dermal vessels and neutrophils

71
Q

Treatments for erythema elevatum diutinum?

A

Dapsone is treatment of choice

can consider NSAIDS, tetracyclines and cochicine

72
Q

What is the epidemiology of granuloma faciale?

A

Adults, caucasian>African American, and M>F

73
Q

What is the clinical presentation of granuloma faciale?

A

Single or multiple discrete red-brown papules, plaques, and nodules on the face, especially the nose, malar prominence, forehead, and ear

  • Some consider granuloma faciale and EED to be the same entity with different anatomic predilections
  • May have follicular prominence, telangiectasias, or a “peau d’orange” appearance
74
Q

What is the histology of granuloma faciale?

A

LCV (findings may be difficult to identify)

Grenz zone

Dense mixed dermal infiltrate consisting of eosinophils, neutrophils, lymphocytes and plasma cells

75
Q

Treatment for granuloma faciale?

A

Intralesional triamcinolone (2.5–5 mg/mL)

Cryotherapy + intralesional triamcinolone

Topical steroids

Topical tacrolimus

If unresponsive, consider dapsone (50–100 mg/day), colchicine, or plaquenil

PDL

76
Q

What is the pathophysiology of ANCA + vasculitis?

A

The ANCA-mediated vascular injury is caused by neutrophils and monocytes that produce toxic oxygen metabolites

77
Q

What ist he main c-ANCA associated vasculitis?

A

Wegners >>MPA

78
Q

What is more specific, p-ANCA or c-ANCA?

A

c-ANCA is much more specific (p-ANCA is a/w most of the ANCA vasculitis entities)

79
Q

What are the main p-ANCA associated vasculitides?

A

Levamisole-induced vasculitis, Churg-Strauss syndrome, MIcroscopic polyangiitis, and minocycline-induced lupus erythematosus

80
Q

What is the pathophysiology of Wegener’s granulomatosis/granulomatosis w/ polyangiitis?

A

c-ANCA mediated (anti-PR3) Th1 immune response leading to granuloma formation

Unknown triggers

81
Q

What is the specific antibody associated with granulomatosis w/ polyangiitis?

A

anti-PR3

82
Q

What is the clinical triad seen in granulomatosis w/ polyangiitis?

A
  1. Necrotizing granulomas of the upper and lower respiratory tract: cough hemoptysis, SOB,
  • Nasal/sinus inflammation: rhinorrhea, sinusitis, and purulent or bloody nasal discharge
  1. Systemic vasculitis
  • Musculoskeletal: arthralgias
  • Ocular: conjunctivitis, proptosis, and keratitis
  • CNS: peripheral neuropathy and CVA
  1. Glomerulonephritis
    - Death from renal disease if left untreated (>80% 1

year mortality)

83
Q

Most common cause of death in untreated granulomatosis w/ polyangiitis?

A

Renal dz (80% 1 year mortality if untreated)

84
Q

How common are cutaneous findings in granulomatosis w/ polyangiitis?

A

10-21% at initial presentation but 15-46% throughout the course of dz

85
Q

What are the most common skin findings in granulomatosis w/ polyangiitis?

A

Palpable purpura in dependant areas

  • Oral ulcers common, gingival hyperplasia w/ strawberry gums is less common but near pathognomonic
  • Can have “pyoderma-like” nodules or necrotic ulcers
  • Cutaneous dz can be a/w wearlier onset and more widespread dz
86
Q

What are the limited forms of granulomatosis w/ polyangiitis?

A

Can have limited cutaneous or limited pulmonary

87
Q

What is the histology of granulomatosis w/ polyangiitis?

A

LCV + extravascular necrotizing palisading granulomas w/ basophilic debris

88
Q

What laboratory abnormalities can be seen in patients w/ granulomatosis with polyangiitis?

A

Can see an increase in ESR and WBC, can also see c-ANCA (sensitivity up to 90% and specificity 80-100%)

  • Abnormal UA: microscopi hematuria or RBC casts
  • Abnormal CXR: nodules, infiltrates, and cavities often found
  • Sinus involvement: abnormal sinus X-ray, CT sinus or nasal bx
89
Q

What ist he treatment for granulomatosis with polyangiitis?

A

Prednisone (1mg/kg/day) + cyclosphosphamide (2mg/kg/day)

Maintence: MTX (20-25mg/kg) and or oral steroids, azothioprine (2mg/kg/day)

90
Q

What is the prognosis of granulomatosis w/ polyangiitis?

A

50% relapse within 5 years

91
Q

What conditions is microscopic polyangiitis associated with?

A

Endocarditis, medications, and malignancies

92
Q

What is the clinical presentation of microscopic polyangiitis?

A

Cutaneous palpable purpura, petechiae > livedo reticularis, retiform purpura, ulcers, and splinter hemorrhages

Constitutional symptoms may be present for months to years

  • Most common cause of pulmonary-renal syndrome
93
Q

What are the most common systemic sx’s seen with microscopic polyangiitis?

A

Renal (70-90%): focal segmental necrotizing glomerulonephritis

Pulmonary (25-50%): pulmonary capillaritis, pulmonary hemorrhage

Neurological (up to 33%): Mononeuritis multiplex, peripheral neuropathy

94
Q

What are some key differences between microscopic polyangiitis vs polyarteritis nodosa?

A

Renal glomerulonephritis not seen as often as are pulmonary sx’s and ANCA is left often present in microscopic polyangiitis.

Tends to have HTN and microaneurysms as well as a hepatitis Bor C association (PAN)

95
Q

What is the histology of microscopic polyangiitis?

A

LCV w/ segmental small > medium vessel vasclulitis

No granuloma formation, unlike WG or Churg-Strauss syndrome

96
Q

What is the specific antibody seen in microscopic polyangiitis most commonly?

A

+ANCA –> specific antibody will be Anti-MPO most commonly (60%)

97
Q

What other lab/workup abnormalities can be seen in microscopic polyangiitis /

A

p-ANCA + (MPO)

  • abnormal UA (proteinuria/hematuria)
  • Abnormal CXR or CT chest
  • Abnormal EMG or lung/nerve/kidney biopsy
98
Q

Treatment for microscopic polyangiitis?

A

Induction:

Cyclophosphamide (2 mg/kg per day) + oral steroids

(1 mg/kg per day)

Rituximab + cyclophosphamide

Remission:

-MTX or azathioprine, similar to WG

  • Localized
  • TMP-SMX + oral steroids
99
Q

What are the possible triggers of Churg-Strauss syndrome?

A

Rapid steroid taper, vaccination, leukotriene inhibitors, and anti-IgE ab (omalizumab)

100
Q

What are the 3 classic clinical states of Churg-Strauss syndrome?

A
  1. Adult-onset asthma, nasal polyps, and allergic rhinitis
  2. Eosinophilia, pneumonia, GI: N/V, abdominal pain
  3. Systemic: necrotizing vasculitis, neurologic: mononeurtiis multiplex, symmetric polyneuropathy, cardiac: pericarditis, valvular disease, endocardiomyopathy
101
Q

What is the primary cause of death in Churg-Strauss syndrome?

A

Endocardiomyopathy

102
Q

How often does Churg-Strauss present with cutaneous lesions?

A

14% as an initial presentation, but the vast majority will develop lesions through the course of the disease

103
Q

What are the clinical presentations of Churg-Strauss Syndrome?

A

Palpable purpura on the lower extremities, painful symmetric subcutaneous nodules of the extremities and scalp

104
Q

What is the histology of Churg-Strauss Syndrome?

A
  • LCV w/ mixed infiltrate of eosinophils, neutrophils, lymphocytes, and macrophages
  • Palisading neutrophilic and eosinophilic extravascular granulomas with degenerated collagen fibers (“red granulomas”)
105
Q

What laboratory findings can be seen in Churgg-Strauss Syndrome?

A

+ ANCA (linked to neurologic and renal dz)

  • ANCA (more linked to cardiac dz)
  • p-ANCA > C-ANCA in leukotriene-associated dz
  • Eosinophilia (eos > 1500)
  • Leukocytosis
  • Increased IgE
  • CXR: patchy infiltrates, interstitial dz, and nodular masses
106
Q

Treatment for Churg-Strauss syndrome?

A

Oral steroids (1 mg/kg a day)

Internal organ involvement:

  • Cyclophosphamide (2 mg/kg a day) + oral steroids
107
Q

What are the possible triggers of polyarteritis nodosa?

A

Possible triggers: meds, infections, inflammatory disease (IBD, SLE), and hairy cell leukemia; may be immune complex-mediated

a/w hepatitis B (5%–7%) and hepatitis C

Cutaneous form:

a/w streptococcal infection in children

Has been a/w minocycline

108
Q

What is the most common specific antibody associated with p-ANCA?

A

Anti-myeloperoxidase

109
Q

What is the staining pattern for p-ANCA?

A

Perinuclear staining (Churg-Stauss syndrome, MPA>PAN, chronic infection, other autoimmune dz

110
Q

What is the staining pattern of c-ANCA?

A

Granular cytoplasmic staining (Wegener’s>>MPA)

111
Q

What test must be also drawn with an ANCA?

A

ANA, can obscure the p-ANCA especially (the staining can overlap). If you see a + ANA need to discuss w/ lab, need to be really careful w/ procedure

112
Q

What are the two forms of PAN?

A

Classic: systemic dz w/ multi-system vasculitis

Cutaneous: subtype w/ limited systemic involvement

113
Q

What clinical features do you see w/ PAN?

A
  • Palpable purpura on the lower extremities, painful single/multiple subcutaneous nodules on lower extremities which can ulcerate, Nodules may follow the course of superficial blood vessels, Livedo reticularis, rarely can have digital or penile infarction
  • The cutaneous subtype: pink to purple-red nodules on LE near the malleoli and may extend proximally
  • Atrophie blanche; atrophic ivory, stellate scars, livedo reticularis, digital gangrene
114
Q

What systemic findings can be seen in PAN?

A

Constitutional sx’s: fever, weight loss, arthralgias, malaise

Renal: HTN and renal failure (most common cause of death)

Cardiac: cardiomyopathy, MI, arrhythmias

Neuro: paresthesias, motor or polyneuropathies

GI: N/V, bowel infarction, hemorrhage, mesenteric ischemia

GU: orchitis

115
Q

What is the histology of PAN?

A

LCV

Necrotizing arteritis of medium-sized arteries

  • In skin, these vessels are located in subcutis and at the dermopannicular junction
  • Microaneurysms leading to thrombosis, ischemia, and necrosis
  • Microaneurysms seen on angiography of medium-

sized vessels (coronary, renal, celiac, and mesenteric arteries)

Later course defined by fibrosis

DIF: IgM and/or C3 in the walls of cutaneous blood

vessels

116
Q

What is DIF finding of PAN?

A

IgM and/or C3 in the walls of cutaneous blood vessels

117
Q

Laboratory findings of PAN?

A

CBC: anemia and leukocytosis

  • UA for hematuria and RBC casts
  • Hepatitis B and C
  • ANCA (p-ANCA <20% positive)
  • Consider angiography if suspect microaneurysm or stenosis
  • In children, consider anti-streptolysin O
118
Q

What is the treatment for PAN?

A

-Interferon-α +/− vidarabine/lamivudine + plasma exchange

Cutaneous subtype:

Intralesional steroids, NSAIDS, and oral steroids for 3

to 6 months if severe skin involvement

Children: consider penicillin, given the association

with streptococcal infection • Severe systemic disease:

Cyclophosphamide (2 mg/kg a day) + oral steroids for 12 months

MTX (7.5–20 mg/week)

IVIG
-Hepatitis B (+): Interferon-α +/− vidarabine/lamivudine + plasma exchange

119
Q

What is the epidemiology of Kawasaki dz?

A

80% cases in kids <5 y/o; M>F

Increased incidence in Japanese

120
Q

What is the pathophysiology of Kawasaki dz?

A

Unclear etiology, likely 2/2 infection by unknown agent

Inflammation, scarring, stenosis, and aneurys formation in the small, medium, and large musculoelastic arteries including the coronary artery

121
Q

What is required for the dx of Kawasaki dz?

A

Needs fever >= 5 days, + 4/5 of the below findings:

Conjunctival injection (usually non-exudative)

Mucous membrane: lip/oral mucosa erythema, fissured lips, strawberry tongue, and injected oral and pharyngeal mucosa

Cutaneous: polymorphous eruption including psoriasiform, morbilliform, scarlatiniform (particularly perineal with desquamation), and EM-like lesions on hands/feet

Cervical lymphadenopathy

Extremity changes: peripheral edema/erythema of hands/feet, or periungual desquamation

122
Q

What nail findings may occur in Kawasaki disease?

A

Orange-brown or white transverse nail discoloration

123
Q

What systemic complications can be seen Kawasaki dz?

A

Cardiac: coronary artery aneurysms/ectasia (secondary to vasculitis) and myocarditis

Musculoskeletal: arthritis/arthralgias

Pulmonary: pneumonitis

CNS: aseptic meningitis and facial nerve palsies

Ophtho: anterior uveitis

GI: gastroenteritis, hepatomegaly, bile duct

inflammation/hepatitis, jaundice, and pancreatitis

124
Q

Laboratory findings in Kawasaki dz?

A

Increased CRP and ESR, LFT’s, and GGT

CBC (anemia, leukocytosis, increased neutrophil/eosinophils, and thrombocytosis)

Decreased albumin, sodium, potassium, and HDL

Check echocardiogram at diagnosis, 2, 6, and 8 weeks

125
Q

What must be serially checked in Kawasaki’s dz?

A

Check echocardiogram at diagnosis, 2, 6, and 8 weeks

126
Q

Treatment of Kawasaki’s dz?

A

High-dose Aspirin (80–100 mg/kg a day) + IVIG (2 g/kg)

-If given within first 10 days leads to a decreased rate of coronary artery issues

Resistant cases: IVIG + steroids, cyclophosphamide, cyclosporine/CIs, plasma exchange, TNF-α inhibitors, MTX, rituximab, and anakinra

Maintenance: aspirin

127
Q

What is the #1 cause of acquired pediatric heart dz in the US?

A

Kawasaki’s dz

128
Q

What is the difference in the prognosis of children w/ Kawasaki’s dz who are <12 months old?

A

They do not tend to respond to treatment as well

129
Q

Epidemiology of temporal arteritis?

A

More common in Caucasians, females

->50 yo

130
Q

Pathophysiology of temporal arteritis?

A

Vessel involved: any medium to large vessel (especially temporal artery)

Granulomatous vasculitis leading to ischemia, occlusion, infarction, and aneurysm

131
Q

What are the clinical findings in large vessel vasculitis?

A

Early: tenderness and erythema along scalp and temples with possible cord-like nodule along the temporal scalp

Other cutaneous symptoms: erythema, purpura,

alopecia of overlying skin, and scalp necrosis

Unilateral temporal headache

Loss of temporal pulse

Jaw claudication

Glossitis, necrosis of anterior tongue (lingual artery)

Late: ulceration or gangrene

132
Q

Systemic findings in large vessel vasculitis?

A

Systemic findings:

Polymyalgia rheumatica (40%–60%) with limb and girdle muscle pain, stiffness, and weakness

Fever and weight loss

Neurologic: vision loss (14%), stroke, subarachnoid

hemorrhage, and altered mental status

133
Q

What is the histology of large vessel vasculitis?

A
  • Segmental granulomatous large vessel arteritis with giant cells
  • Disruption of media with fragmentation of the internal elastic lamina
134
Q

What is the laboratory workup for large vessel vasculitis?

A

Increased ESR and CRP

  • Anticardiolipin antibody (may be increased)
  • MRA and Temporal artery biopsy helpful for w/u
135
Q

What is the treatment of large-vessel vasculitis?

A

Aspirin 81 mg/day + oral steroids (40–60 mg/day)

Consider methylprednisolone (1 g/day for 3–5 days) if acute visual loss

136
Q

What is the pathophysiology of Takayasu’s arteritis?

A

The vessel involved: aorta and its main branches

Granulomatous vasculitis leading to stenosis, occlusion, and aneurysms

137
Q

What is the clinical findings in Takayasu’s arteritis?

A

Cutaneous symptoms seen in 50% of individuals, including: Purpura, Erythematous subcutaneous nodules, EN-like lesions, PG-like lesions, Raynaud’s phenomenon and digital gangrene

Systemic symptoms: Constitutional symptoms: fever, fatigue, malaise, night sweats, and weight loss, HTN, Loss of carotid or radial pulse

138
Q

What are the workup findings in Takayasu’s arteritis?

A

Increased esr and MRA w/ visualization of all branches of the aortic arch needed

139
Q

Treatment of Takayasu’s arteritis?

A

Oral prednisone (1 mg/kg) for 1 to 3 months with a 6 to 12-month taper

MTX 15–25 mg/week + prednisone

Cyclophosphamide

Trials with infliximab or etanercept are promising

Surgical intervention for cerebral hypoperfusion, valvular

insufficiency, and aneurysms

140
Q

What disease is cryoglobulinemias a/w?

A

Likely related to HCV

141
Q

What are cryoglobulins?

A

Immunoglobulins that precipitate at colder temps

142
Q

Which type of cryoglobulinemia has a monoclonal cryoglobulinemia?

A

Type I (IgM>>IgG, IgA, light chains)

143
Q

What is the histology of type I cryoglobulinemia?

A

Complete occlusion of vessel lumens w/ hyaline material
-Lacks LCV

144
Q

What are the two types of mixed cryoglobulinemia?

A

Type II and III

145
Q

Which types of cryoglobulinemia lack LCV?

A

Type I

146
Q

Which cryoglobulinemia have LCV?

A

Type II and III

147
Q

What are the clinical findings in cryoglobulinemia type I?

A

Raynaud’s phenomenon
-Purpura, livedo reticularis, and ulceration

-Cold-induced acrocyanosis of helices

148
Q

Cryoglobulinemia types II and III have what typical cutaneous findings?

A

Palpable purpura and urticarial lesions

-Systemic findings common

149
Q

What are the histological findings of cryoglobulinemia?

A

Type I: occlusive vasculopathy with vessels completely filled by homogenous hyaline material; lacks LCV

Types II and III: characteristic features of LCV

150
Q

What labs are found in cryoglobulinemia?

A

Increased cryoglobulins
-Complement (hypocomplementemia in 90%; decreased C4)

  • RF(+) (Types 2 and 3)
  • Hepatitis B/C
  • LFTs
151
Q

What are the types of cryoglobulinemia associated with + RF?

A

Types 2/3

152
Q

What are the immunoglobulins involved in type II cryoglobulinemia and are they polyclonal or monoclonal?

A

IgG=polyclonal and IgM=Monoclonal

153
Q

What are the immunoglobulins involved in type III cryoglobulinemia and are they polyclonal or monoclonal?

A

IgG=polyclonal and IgM = polyclonal

154
Q

What is the treatment of cryoglobulinemia?

A

HCV-related:

Interferon-α +/− ribavirin to prevent relapse

Low dose oral steroids (0.1–0.3 mg/kg per day if

arthralgia/weakness present)

High dose (0.5–1.5 mg/kg per day) for renal or

nervous system involvement

HCV-unrelated disease (connective tissue or malignancy

related) is less clear

DERM (20) Vascular Disorders (3 decks)

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