Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Vasculitis > Vasculitis and Uncommon Arteriopathies > Flashcards

Vasculitis and Uncommon Arteriopathies Flashcards

1
Q

What is the most common systemic vasculitis in adults?

A

Giant cell arteritis (GCA)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Name large vessel vasculitides.

A
  1. GCA

2. Takayasu

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Name medium vessel vasculitides.

A
  1. Kawasaki

2. Polyarteritis nodosa

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Name small vessel vasculitides.

A
  1. Granulomatosis with polyangiitis (Wegener’s)
  2. Microscopic polyangiitis
  3. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What vessels to large vessel vasculitides affect?

A

Aorta and its branches

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Describe the epidemiology of GCA

A
  1. Gender: Affect women more than men (2-4x)
  2. Age: Affects older individuals (> 50) with incidence highest in individuals 70-80 years old
  3. Geography: Most common in Northern Europeans and rare in African Americans or Asians
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is the pathogenesis of GCA?

A
  1. Mechanisms is unknown

2. Hypothesis that an environmental factor may trigger a genetically susceptible individual

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What genetic polymorphisms are at risk for developing GCA?

A

HLA-DRB104 allele
HLA-DRB101 allele
Polymorphisms in genes encoding cytokines and other immunoregulatory proteins have also been associated with developing GCA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What infectious agents may trigger GCA?

A
  1. Parvovirus B19
  2. Parainfluenza virus
  3. Chlamydia pneumoniae
  4. Mycoplasma pneumoniae
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What histopathologic features would you see on temporal biopsy for GCA?

A

Inflammatory infiltrates composed primarily of T cells and macrophages.
Multinucleated giant cells are typically adjacent to a fragmented internal elastic lamina.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Describe the inflammatory process for GCA.

A

The inflammatory process is thought to arise in the adventitial layer of the arterial wall.

Activated, mature dendritic cells attract CD4 T cells to the artery through the vasa vasorum, and upon activation the T cells undergo clonal expansion.

T cells then release cytokines including IFN-gamma which stimulate macrophages and induce formation of multinucleated giant cells.

Macrophages then amplify inflammation via secretion of cytokines and induce tissue damage by releasing matrix metalloproteinases and ROS.

In response to this injury, the artery releases growth and angiogenic factors (PDGF, VEGF) which induces proliferation of myofibroblasts, new vessel formation and marked thickening of the intima.

This process of intimal expansion and hyperplasia leads to narrowing and possibly vessel occlusion resulting in ischemia, jaw claudication, visual loss and stroke.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Describe the clinical presentation of GCA

A
  1. Suspected in people > 50 years old with new onset headache and presence of systemic inflammation
  2. Temporal: headache or scalp tenderness, thickening or nodular temporal arteries, jaw claudication
  3. Vision: diplopia, decreased vision, vision loss, amaurosis fugax
  4. Neuro: stroke, TIA, neuropathy
  5. Polymyalgia rheumatica (pain and stiffness in the neck and proximal extremities)
  6. Constitutional symptoms: fever, chills, weight loss, fatigue
  7. Stenoses of proximal vessels may present with upper extremity claudication or asymetric BP
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Explain the American College of Rheumatology Criteria for GCA.

A

3 of the 5 should be present for a sensitivity of 93.5% and specificity of 91.2%

  1. Age of onset > 50
  2. New headache (localized pain in the head)
  3. Temporal artery abnormality (tenderness to palpation, decreased pulsation)
  4. Elevated ESR (>50mm/hr by the Westergren method)
  5. Abnormal artery biopsy (predominance of mononuclear cell infiltration or granulomatous inflammation, usually with multinucleated giant cells).
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is the diagnostic evaluation for a patient with GCA?

A
  1. Physical exam involving vitals (BP in both arms), neurovascular exam with pulses documented in all extremities, and temporal palpation
  2. Inflammatory markers: ESR, CRP
  3. CBC: thrombocytopenia, normochromic normocytic anemia
  4. Temporal artery biopsy (gold standard) with an adequate length 2-3cm with bilateral biopsies preferable
  5. Imaging with MRA/CTA or angio
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What percentage of patients with GCA have stenoses of vessels from the arch?

A

10-15%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What percentage of patients with GCA affect the aorta?

A

10%

17
Q

What is the risk of developing thoracic and abdominal aneurysms in patient with GCA?

A

17x risk for thoracic aneurysms

2.4x risk of abdominal aneurysms

18
Q

What are the goals of treatment in patients with GCA?

A

Treat symptoms

Prevent sequelae from ischemic events

19
Q

Why can we initiate treatment for GCA almost immediately while awaiting temporal biopsy?

A

Histopathologic evidence of arteritis persists for several weeks

20
Q

What is the treatment for GCA?

A

Guidelines recommend intial oral dose of prednisone 40-60 mg PO DIE x 4 weeks and then tapered by 10% every 2-4 weeks
Methylprednisolone IV 1g DIE for 3 days may be considered for patients with visual loss

If no contraindication to antiplatelet therapy, los dose ASA should be started bevause it may reduce the risk of ischeic events such as visual loss

21
Q

What are steroid-related complications in patients being treated for GCA?

A
  1. DM
  2. DLP
  3. Osteoporosis
  4. PCP**
22
Q

The addition of this medication may decrease prednisone dose as a steroi-sparing immunosuppressive.

A

Methotrexate

23
Q

What is idiopathic aortitis?

A

Characterized by giant cell or lymphoplasmacytic inflammation of the aorta.

24
Q

Where does idiopathic aortitis typically occur?

A

Ascending aorta accounting for 2-4% of aneurysms

25
Q

What is the epidemiology of idiopathic aortitis?

A
  1. Age: older individuals

2. Gender: more commonly in females

26
Q

How is idiopathic aortitis typically diagnosed?

A

Incidentially in individuals without a history of rheumatic disease.

27
Q

What is polyarteritis nodosa (PAN)?

A

PAN is a form of necrotizing vasculitis that affects medium sized arteries of the kidney, GIT, skin, muscles and nerves

28
Q

How is PAN classified?

A

Primary (idiopathic)

Secondary (related to hepatitis B virus)

29
Q

Describe the American College of Rheumatology criteria for diagnosing PAN.

A

3 of the 10 should be present for sensitivity of 82.2% and specifcity of 86.6%

  1. Nonintentional weight loss of >4 kg
  2. Livedo reticularis (mottled reticular pattern over the skin of portions of the extremities or torso)
  3. Testicular pain or tenderness
  4. Myalgias, weakness or leg tenderness (excluding shoulder or hip girdle)
  5. Mono or polyneuropathy
  6. Diastolic BP > 90
  7. Elevated BUN or creatinine
  8. HBV (presence of HB surface antigen or Ab in serum)
  9. Angiographic abnormality (aneurysms or occlusions in the visceral arteries)
  10. Biopsy of small or medium sized artery containing PMN (presence of granulocytes or mononuclear leukocytes in the arterial wall)
30
Q

Describe the epidemiology of PAN.

A
  1. Age: can occur in any age but typically between 40-60
  2. Gender: more common in males
  3. Related to HBV and since the declining rates of HBV, rates of PAN are also declining
31
Q

What is the pathogenesis of PAN?

A

In most cases, the cause is unknown
1/3 or cases are associated with hepatitis B infection with the mean time of diagnosis of hepatitis and PAN being 7 months.

32
Q

What viruses are associated with PAN?

A

HBV

H!V

33
Q

PAN may occur as a paraneoplastic process in what malignancies?

A

Hematologic malignancies (particularly hairy cell leukemia)

34
Q

Describe the histologic process of PAN.

A

PAN is a focal, segmental necrotizing vasculitis affecting medium sized arteries.

The arterial wall inflammation is characterized by fibrinoid necrosis of the media and a cellular infiltrate primarily composed of neutrophils and lymphocytes.

Localized thromboses can occur at the site of inflammation, and arterial aneurysms may form as a result of weakening of the vessel wall by the necrotizing process, ultimately leading to end organ ischemia, infarct and hemorrhage.

35
Q

What is the mechanism of hepatitis B related PAN?

A

Deposition of the viral antigen complexes can lead to the activation of the complement cascade which results in neutrophil attraction and activation with T cells (CD8) having been identified in inflammatory infiltrates and may be involved in the pathogenesis

36
Q

What is the clinical presentation for patients with PAN?

A
  1. Constitutitonal symptoms: fever, fatigue, weight loss
  2. Neuro: 70% of patients and manifests as a mononeuritis multiplex or sensorimotor peripheral neuropathy
  3. GI: 40-60% mesenteric ischemic, GI hemorrhage, bowel perforation and pancreatitis
  4. Renal: glomerular ischemia and infarction, renal insufficiency and hypertension, microaneurysms can rupture and cause renal hematomas
  5. MSK: myalgias due to skeletal muscle involvement and large joint arthralgias are common
  6. Testicular pain due to testicular ischemia occuring in 20% of patients
  7. Skin: livedo reticularis, tender subcutaneous nodules and skin ulcerations
  8. Peripheral arterial occlusions result in ischemia and gangrene of upper or lower extremities
  9. Cardiac: CMP and coronary vasculitis
37
Q

What is the diagnostic evaluation of PAN?

A 
Clinical evaluation
Labs: ESR, CRP
CBC: Anemia, leukocytosis
SMA10: Elevated BUN/creat
LFTs
Hepatitis serologies
Muscle enzyme concentrations
UA: proteinuria and modest hematuria
Imaging: CTA, angiogram (typical microaneurysm of the celia, SMA and renal branches)
38
Q

what is the medical treatment of PAN?

A

Glucocorticoid treatment is the mainstay (prednisone 1 mg/kg/day x 4 weeks with taper) with courses lasting 9-12 months
Patients with end organ dysfunction require glucocorticoids with cyclophosphamide to induce disease remission (cyclophosphamide 1.5-2.0 mg/kg/day PO or IV 600-750 mgx 6 months)
ACEI for hypertension
For patients with hepatitis B: antiretroviral therapy (lamivudine or entecavir) and limited immunosuppresive therapy is employed with glucocorticoids. Plasma exchange for removal of immune complexes can be considered for patients with HBV related PAN and severe vasculitis

Vasculitis (1 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions