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Rheumatology > Vasculitides > Flashcards

Vasculitides Flashcards

1
Q

Polyarteritis Nodosa

  1. What is it?
  2. Most commonly involves what? 5
A
  1. Necrotizing arteritis of medium-sized vessels
  2. Most commonly involves the
    - skin,
    - peripheral nerves,
    - mesenteric vessels (including renal arteries),
    - heart, and
    - brain but can affect any organ
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2
Q

Polyarteritis Nodosa

  1. Most commonly involved in what population?
  2. Can also occur in who?
  3. Gender?
  4. Common?
  5. Idiopathic but may be related to what? 3
A 
Epidemiology
1. Most commonly in middle-aged or older adults
2. Can also occur in children
3. Males > females
4. Rare (3-4.5/100,000)
Etiology
5. Idiopathic but may be related to 
-Hepatitis B and 
-Hep C as well as 
-Hairy Cell leukemia
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3
Q

Polyarteritis Nodosa Pathogenesis
1. Some cases related to what formation?

  1. Thickening of the inflamed vessel wall leads to what?
  2. Reduced what? 2
  3. Thrombosis results in what?
  4. Inflammation can also cause weakening of the vessel wall that leads to _______ formation?
  5. Does not involve the what?
A
  1. immune complex
  2. luminal narrowing
  3. blood flow and thrombosis
  4. ischemia to the involved organ
  5. aneurysm
  6. veins
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4
Q

Polyarteritis Nodosa Presentation
Systemic symptoms? 5

Signs? 5

A
  1. Systemic symptoms
    - fatigue,
    - weight loss,
    - weakness,
    - fever,
    - arthralgias
  2. Signs
    - skin lesions,
    - hypertension,
    - renal insufficiency,
    - neurologic dysfunction,
    - abdominal pain of multisystem involvement
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5
Q

Skin manifestations of PAN
5

May be patterned how?
More common where?

A
  1. Tender erythematous nodules
  2. Purpura
  3. Livedo reticularis
  4. Ulcers (Infarction, gangrene)
  5. Bullous or vesicular eruption

May be focal or diffuse
More common on the lower extremities

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6
Q

PAN: What is the most commonly involved organ?

A

Kidneys

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7
Q

Renal Manifestations of PAN
1. Variable degrees of what? 2

  1. Rupture of renal arteries can result in what?
  2. Luminal narrowing leads to what but not inflammation or necrosis?
  3. UA may show what? 2
    - What should not be seen?
A
    • renal insufficiency and
    • HTN
  2. perirenal hematoma
  3. glomerular ischemia
    • minimal protein,
    • moderate hematuria.
    • NO RBC casts should be seen
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8
Q

Neurologic manifestations of PAN

2

A
  1. Motor and sensory deficits of the involved nerves

2. Generally asymmetric neuropathy at onset

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9
Q

GI manifestations of PAN

6

A
  1. Abdominal pain in those with mesenteric arteritis
  2. Nausea
  3. Vomiting
  4. Melena
  5. Diarrhea
  6. GI bleeding
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10
Q

Abdominal pain in those with mesenteric arteritis: Characteristics of this? 3

A
  1. Post prandial pain
  2. Weight loss
  3. Bowel infarction with perforation
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11
Q

Cardiovascular manifestations of PAN

A
  1. CAD
  2. HF
  3. MI is uncommon
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12
Q

PAN: MI is uncommon but can happen because of what?

HF from PAN is caused by what? 2

A
  1. Myocardial infarction is uncommon
    - may result from narrowing or occlusion of the coronary arteries
  2. Heart failure
    - From vasculitis of the coronary arteries, resulting in ischemic cardiomyopathy
    - or from uncontrolled hypertension caused by renal disease
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13
Q

Musculoskeletal manifestations of PAN

3

A
  1. Muscle involvement is common
  2. Myalgias
  3. Muscular weakness
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14
Q

PAN: Can manifest in other ways.

  1. GU?
  2. OBGYN? 2
  3. EYE? 2
A
  1. Orchitis
  2. Breast and uterine pain
  3. Eye
    - Ischemic retinopathy,
    - retinal detachment
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15
Q

PAN DX:
1. Based on?

  1. Confirm Dx with what?
  2. Basic evaluation should include what? 8 labs
A
  1. Based on
    - physical exam,
    - history,
    - laboratory evaluation
  2. Confirm diagnosis with
    - biopsy or
    - angiography if possible
    • CMP,
    • muscle enzymes (CPK),
    • HBV,
    • HCV,
    • UA,
    • ESR,
    • CRP,
    • ANCA (ANCA should be negative)
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16
Q

PAN treatment? 2

A
  1. High dose glucocorticoids
    - Prednisone 1mg/kg/day with a max dose of up to 80 mg per day
  2. Cyclophosphamide or other immunosuppresants like azathiprine or methotrexate
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17
Q

PAN

  1. If untreated, 5 year survival is only about ____%
  2. If treated, 5 year survival is about ___%
A
  1. 13

2. 80

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18
Q

Kawasaki Dz

  1. AKA?
  2. Typically self limiting lasting an average of ___ days without therapy
A
  1. AKA: Mucocutaneous lymph node syndrome

2. 12

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19
Q

Kawasaki’s

  1. Most common in ages what?
  2. More common in what gender?
  3. Most common in what culture? 2
  4. Increased incidence in what seasons? 2
A
  1. 3-5 (80-90% of cases)
  2. boys
  3. Asians or Pacific Islanders
  4. summer and winter
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20
Q

Pathophysiology of KD
1. Vasculitis caused by what?

  1. Can result int he destruction of what? 2
A
  1. Vasculitis caused by the infiltration of vessel walls with mononuclear cells (monocytes, lymphocytes and dendritic cells) and later IgA secreting plasma cells
  2. Can result in the destruction of the
    - tunica media and
    - aneurysm formation
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21
Q

DX of Kawasaki:
1. Requires the presence of a fever lasting how long?

  1. and at least 4 of the 5?
A
  1. 5 days
    • bilateral bulbar nonexudative conjunctivitis,
    • erythema of the lips and oral mucosa, rash,
    • peripheral extremity changes including erythema of palms or soles, edema of hands or feet and
    • Polymorphus rash
    • cervical lymphadenopathy typically develop after a brief nonspecific prodrome of respiratory or gastrointestinal symptoms
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22
Q

What is incomplete Kawasaki’s dz?

A

If only 2 of the criteria are met then it is considered an incomplete form

23
Q

KD: Classic syndrome

Often preceded by nonspecific symptoms for up to 10 days? 5

A
  1. Irritability or lethargy 50%
  2. Vomiting alone 44%
  3. Anorexia 37%
  4. Cough or rhinorrhea 35%
  5. Diarrhea, vomiting or abdominal pain 61%
24
Q
  1. How will the fever react to antipyretics for KD?

2. Consider KD in the DDx of all children with a fever for _______ or more

A
  1. Minimally responsive to antipyretics and typically remains above 38.5 (101.3)
    May be intermittent
  2. 5 days
25
Q

KD: Conjunctivitis

  1. Present in > ___% of cases
  2. Bulbar injection begins within _____ of onset of the fever
  3. May spare the ______
  4. Frequently occurs with what? 2
A
  1. 90
  2. days
  3. limbus
  4. photophobia and anterior uveitis
26
Q

KD: Mucositis

  1. Presents how? 2
  2. Severity?
  3. If they have what than likely this is not KD?
A
    • Cracked red lips
    • Strawberry tongue
  2. May be mild or not occur at all
  3. oral lesion such as vesicles, ulcers, tonsillar exudate
27
Q
  1. What is usually the last manifestation to appear in KD?
  2. Edema where?
  3. Erythema where?
A
  1. Extremity changes
  2. Edema of the dorsum of hands and feet
  3. Erythema of the palms and soles
28
Q

Whats the last phase of the dz for KD?

A

sheet like desquamation of hands and feet

29
Q

KD: Polymorphous rash

  1. Usually occurs when?
  2. May begin as perineal erythema and desquamation followed by what?
  3. If what than consider another diagnosis?
A
  1. Usually occurs in the first few days of the illness
  2. macular, morbilliform or targetoid skin lesion of the trunk and extremities
  3. vesicular or bullous lesions
30
Q

KD lymphadenopathy

  1. Common?
  2. Which nodes?
  3. May be only able to palpate what?
  4. When should we look for an alternative dx? 2
A
  1. Absent in up to 50-75% of patients
  2. Anterior cervical nodes
  3. May only be able to palpate a single large node
  4. If
    - diffuse lymphadenopathy or
    - splenomegaly look for an alternative diagnosis
31
Q

Kawasaki Disease has significant cardiovascular complications

5

A
  1. Coronary artery aneurysms
  2. CHF and decreased EF
  3. MI
  4. Arrhythmias
  5. Peripheral arterial occlusion
32
Q

Evaluation for KD?
1. Lab? 4

  1. Imaging? At what times should we do this? 2
  2. Why would we get a CXR?
A
  1. Lab
    - CBC,
    - CRP, ESR, = elevated
    - CMP,
  2. Echocardiogram
    At diagnosis and repeat at 2 weeks and 6-8 weeks
  3. CXR to evaluate for pulmonary edema
33
Q

What would the CBC (3) and CMP (2) show on KD?

A
  1. leukocytosis,
  2. thrombocytosis,
  3. anemia,
  4. abnormal LFTs,
  5. hyponatremia
34
Q

Treatment for KD? 2

A
  1. IV IG 2 g/kg infusion over 8-12 hours

2. + aspirin 80-100mg/kg/day divided into QID dosing

35
Q

IVIG for KD
1. If given within the first 10 days of the onset of illness can reduce the incidence of what?

  1. ___________ effect
  2. Reduces what? 2
  3. augments what activity?
  4. Resolves what?
A
  1. coronary aneurysm by 5X
  2. Anti-inflammatory
    • acute phase reactants,
    • cytokines,
  4. T cell suppressor
  5. fever
36
Q

Aspirin for KD:
1. After afebrile X 48 hours then ________ aspirin dose to 3-5 mg/kg/day and continue for about __ months

  1. Advantageous affects? 3
A
  1. decrease, 2
    • Antipyretic,
    • anti-inflammatory and
    • antiplatelet effects
37
Q

Second line therapies

for KD? 2

A
  1. Methylprednisolone

2. TNF inhibitors

38
Q

Wegener’s Granulomatosis

  1. AKA?
  2. Common?
  3. Associated with what?
  4. Without treatment survival?
A
  1. AKA Granulomatosis with polyangitis (newest terminology)
  2. Rare disorder (12 per million)
  3. ANCA associated
  4. Without treatment survival
39
Q

Wegener’s Granulomatosis
1. Affects which arteries and this leads to?

  1. Dx triad?
  2. Commonly what age?
  3. Gender?
A
  1. Affects small arteries
    Inflammation restricts blood flow
  2. Triad of
    - upper and
    - lower respiratory tract and
    - glomerulonephritis
  3. Commonly occurs in the fourth and fifth decade
  4. Affects men and women equally
40
Q

Describe the type of pathology that affects the upper, lower respiratory tract (1)and the kidneys (1)?

A
  1. Necrotizing granulomas of upper and lower respiratory tract
  2. Necrotizing glomerulitis and thromboses of capillary loops
41
Q

Wegener’s Granulomatosis

  1. Develops over how long?
  2. 90% of pts present with what?
  3. Such as? 6
A
  1. Develops over 4-12 months
  2. 90% of patients present with upper respiratory tract symptoms
    • Nasal congestion,
    • sinusitis,
    • otitis media,
    • mastoiditis,
    • inflammation of the gums,
    • stridor
42
Q

Wegener’s Granulomatosis
1. Patients may present with lower respiratory tract symptoms. Such as? 3

  1. Which other symptoms are common? 3
A
  1. Patients may present with lower respiratory tract symptoms
    - Cough ,
    - dyspnea,
    - hemoptysis
    • Fever,
    • malaise and
    • weight loss are common
43
Q 
Wegener’s Granulomatosis
Other symptoms are:
1. Skin?
2. Arthritis of?
3. Neurologic?
4. Kidneys?
5. Ocular disease? 2
A
  1. Purpura or other skin lesions
  2. Arthritis of the large joints
  3. dysthesia
  4. Renal insufficiency
    • Unilateral proptosis
    • Red eye
44
Q 
Wegener’s Granulomatosis
On PE 
1. Nose findings? 5
2. Ears? 1
3. Eyes? 4
4. CV? 2
A
  1. Nose
    - Congestion,
    - ulceration,
    - bleeding,
    - perforation of nasal septum,
    - saddle nose deformity
  2. Ears
    - Otitis media
  3. Eyes
    - Proptosis,
    - scleritis,
    - eipiscleritis,
    - conjunctivitis
  4. Findings suggestive of
    - DVT or
    - PE
45
Q

Wegener’s Granulomatosis
Other possible symptoms include?
4

A
  1. Eye inflammation
  2. Joint pain (arthritis) or muscle pain
  3. Rashes or skin sores
  4. Kidney inflammation* (*Renal involvement is usually subclinical until renal insufficiency is advanced)
46
Q

Wegener’s Granulomatosis
1. Labs? 5

  1. Chest CT is most sensitive to lung changes? 4
A
  1. Labs:
    - Mild anemia and leukocytosis
    - Elevated ESR
    - Hematuria
    - Blood cell casts
    - C-ANCA is highly specific, but not all patients show an elevated C-ANCA
  2. Chest CT is most sensitive to lung changes:
    - Infiltrates
    - Nodules
    - Masses
    - cavities
47
Q

Wegener’s Granulomatosis: Caused by autoantibodies against?

What is found in arteries and veins? 2

A
  1. Proteinase
    - pos c-ANCA
  2. Granulomas and pathcy necrosis
48
Q
  1. Wegener’s Granulomatosis
    Treatment? 3
  2. Improvement usually occurs when?
  3. When the disease is in remission, patients will reduce the dosage of these medicines, but will continue treatment until the disease has been in continuous remission for how long?
A
  1. Treatment:
    - Corticosteroids
    - Cyclophosphamide
    - Rituximab
  2. Improvement usually occurs within days to weeks
  3. one year.
49
Q
  1. What is the most common vasculitis in children?

2. Affects which vessels?

A
  1. Most common vasculitis in children
    Also may occur in adults
  2. Affects the small vessels
50
Q

HSP typical features

4

A
  1. Palpable purpura
  2. Abdominal rash
  3. Arthritis
  4. Hematuria or proteinuria
51
Q

HSP typical features:

  1. Palpable purpura: Typically where?
  2. Abdominal rash: Associated with what?
  3. Arthritis: Most common places? 2
  4. Hematuria or proteinuria: Manifests how?
A

Palpable purpura
1. Typically located on the lower extremities/Purple rash

  1. Abdominal pain
    Associated with GI bleeding
  2. Arthritis
    Knees and ankles most common
  3. Hematuria or proteinuria
    Glomerulonephritis
52
Q

HSP

  1. Prognosis?
  2. Lasts how long?
  3. More severe in who?
A
  1. Disease is usually self limiting
  2. Lasts from 1-6 weeks then subsides without sequelae if renal involvement is not severe
  3. Chronic courses with persistent or intermittent skin disease are more likely in adults than children
53
Q

Most Commonly affected organs:
1. Skin manifestations? 3

  1. Joints?
  2. GI? 6
  3. Kidneys? 5
A
  1. 100% of cases
    - Erythematous macular wheals,
    - ecchymosis petechiae,
    - palpable purpura
  2. 60-84%
    Transient or migratory arthritis, large joints
  3. up to 76%
    - N/V, abd pain,
    - ileus,
    - GI bleed,
    - bowel ischemia,
    - perforation,
    - intussuusception
  4. 21-54%
    - Hematuria,
    - + prot,
    - nephritic syndrome,
    - renal insuff,
    - nephrotic syndrome
54
Q

Prognosis and follow up of HSP

  1. Most cases resolve within?
  2. Severe cases treated with?
  3. Recurrence?
  4. Main cause of morbidity?
  5. Followup with? 2
  6. How often?
A
  1. Most cases resolve within a month
  2. Severe cases may be treated with systemic steroids
  3. Recurs in 1/3 of cases, usually within 54 months
  4. Main cause of morbidity is renal disease
  5. Follow up
    - UA and
    - BP monitoring
  6. Weekly X 2 months, then every 1-2 months for a year

Rheumatology (11 decks)

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