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USMLE Pathoma > Vascular pathology part 1 (vasculitis) > Flashcards

Vascular pathology part 1 (vasculitis) Flashcards

1
Q

3 layers of the arterial wall include:

A

Endothelial intimal (internal elastic lamina)

Smooth muscle media (external elastic lamina)

Connective tissue adventitia (CT, nerve fibers, & vasa vasorum feeds it!)

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2
Q

Granulomatous/large vessel vasculitis classically involves branches of which blood vessels? Which age group/gender is this most common in?

A

Carotid artery & most common in older females (>50)

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3
Q

How does LVV/Granulomatous vasculitis present as?

A

1) Headache (temporal artery)
2) Visual disturbances (Opthalmic artery)
3) Jaw claudication (impaired movement)
4) Flu-like symptoms & joint pain
5) ESR is elevated

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4
Q

1) Headache (temporal artery)
2) Visual disturbances (Opthalmic artery)
3) Jaw claudication (impaired movement)
4) Flu-like symptoms & joint pain
5) ESR is elevated

A

the classic presentation of LVV/Granulomatous vasculitis

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5
Q

What does a biopsy of LVV/Granulomatous vasculitis show?

A

1) Inflamed vessel walls with giant cells & intimal fibrosis
2) Segmental lesions

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6
Q

What is the Rx of LVV/Granulomatous vasculitis? Without Rx what is the patient at risk of?

A

Corticosteroids & without Rx patient has a high risk of blindness

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7
Q

Blood vessel walls are made up of 3 basic components (not layers)

A

Endothelial cells
Smooth muscle cells
ECM (elastin, collagen, & glycosaminoglycans)

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8
Q

________ are principal points of physiologic resistance to blood flow.

A

Arterioles

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9
Q

Arteries of 3 types:

Large/elastic arteries

Give an example & what is the media rich in?

A

Aorta & its large branches
Media is rich in elastic fibers

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10
Q

Arteries of 3 types:

Medium/muscular arteries. Give an example & what is the media rich in?

A

Coronary & renal arteries
Media is rich in smooth muscle cells

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11
Q

Arteries of 3 types:

Small arteries/Arterioles. Where are they located?

A

Within the substance of tissues & organs

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12
Q

Inflammation:

Describe a Type II hypersensitivity

A

Complement mediated

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13
Q

Inflammation:

Describe a Type III hypersensitivity

A

Immune complex mediated

It’s responsible for most cases of vasculitis

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14
Q

Inflammation:

Describe a Type IV hypersensitivity

A

Cell mediated

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15
Q

Describe the pathophysiology of Vasculitis induced by type III hypersensitivity?

A
  • Immune complex is deposited in BV, this activated the complement & releases C5a
  • C5a recruits neutrophils which damage the endothelium causing thrombosis (narrowed lumen) leading to ischemia
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16
Q

Henoch-Schönlein purpura is an example of which type of vasculitis

A

Immune complex (III) mediated vasculitis

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17
Q

Describe Vasculitis induced by type IV hypersensitivity?

A

A delayed hypersensitivity reaction that can be involved in some types of vasculitis that have granulomas

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18
Q

Temporal arteritis is an example of which type of vasculitis induced by hypersensitivity reactions

A

Type IV hypersensitivity induced vasculitis

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19
Q

Rickettsia, Meningococcus, & fungus can all cause vasculitis due to

A

Direct invasion of a pathogen

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20
Q

What are two key lab findings used when looking for vasculitis?

A

ANCA (Antineutrophil cytoplasmic antibodies)
ESR (Erythrocyte sedimentation rate)

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21
Q

How do ANCA’s cause vessel damage & how are they detected?

A

Seen in some small vessel vasculitis

ANCAs activate neutrophils & cause a release of enzymes of free radicals that cause vessel damage

Detected with immunofluorescence

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22
Q

What are the 2 types of ANCAs:

Describe Cytoplasmic (c-ANCAs)

What are the vasculitides associated with it?

A

Cytoplasmic (c-ANCAs) that have Ab’s against proteinase 3 in cytoplasmic granules which show cytoplasmic staining pattern

Wegners
Chrug-strauss
Microscopic polyangitis

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23
Q

What are the 2 types of ANCAs:

Wegener’s granulomatosis is an example of

A

Cytoplasmic (c-ANCAs)

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24
Q

What are the 2 types of ANCAs:

Describe Perinuclear (p-ANCAs)

A

Perinuclear (p-ANCAs) has Abs against myeloperoxidase, it shows perinuclear staining

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25
Q

What are the 2 types of ANCAs:

Churg-Strauss syndrome & Polyarteritis nodosa is an example of

A

Perinuclear (p-ANCAs) diseases

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26
Q

Giant cell arteritis (granulomatous) & Takayasu’s arteritis are examples of

A

Large vessel vasculitis

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27
Q

Polyarteritis nodosa, Kawasaki’s disease, & Thromboangitis obliterans are examples of

A

Medium vessel vasculitis

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28
Q

Hypersensitivity vasculitis (Henoch Schönlein purpura), Churg-Strauss syndrome, & Wegener granulomatosis are all examples of

A

Small vessel vasculitis

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29
Q

Describe the clinical representations of Large vessel vasculitis

A

It presents with loss of pulse
(vasculitis -> narrowed lumen -> less blood flow)
OR
Stroke (due to carotid artery involvement)

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30
Q

Describe the clinical representations of Medium vessel vasculitis

A

Presents with infarction or aneurysm (due to involvement of med arteries i.e renal, coronary, popliteal infarct damage can also lead to an aneurysm)

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31
Q

Describe the clinical representations of Small vessel vasculitis

A

Presents with palpable purpura

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32
Q

What are general warning signs of vasculitis

A

Fever, Weight loss, malaise, & myalgias

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33
Q

What’s the disease?

A

Temporal arteritis look for:
- Prominent temporal artery
- Biopsy shows multinucleated giant cells (granulomatous vasculitis)

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34
Q

Old female patient

Presentation:
Headache in the temporal region
Pain in the jaw while chewing
Muscle aches and pains
Develops problems with vision.

Exam & labs:
Nodular and palpable temporal artery.
Elevated ESR

Biopsy:
Granulomatous inflammation & giant cells

What is the diagnosis?

A

Giant cell (temporal) arteritis

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35
Q

Describe the following aspects of Giant cell (temporal) arteritis

  • Presentation (key points)
  • Exam/labs
  • Biopsy
A

Usually an older female patient

Presentation:
Headache in the temporal region
Pain in the jaw while chewing
Muscle aches and pains
Develops problems with vision.

Exam & labs:
Nodular and palpable temporal artery.
Elevated ESR

Biopsy:
Granulomatous inflammation & giant cells

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36
Q

Describe the pathology of Giant cell arteritis

A

The vessels tend to be cord-like & show nodular thickening

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37
Q

Describe the microscopy of Giant cell arteritis

A
  • Focal granulomatous inflammation of the temporal artery
  • Fragmented internal elastic lamina
  • Giant cell
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38
Q

Describe the findings in the histo slide & the likely diagnosis

A

The Elastic tissue stain shows a section of a temporal artery with:
- Focal destruction of the internal elastic membrane & Intimal thickening (characteristic of long-standing/healed arteritis)

Diagnosis: Temporal (giant cell) arteritis

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39
Q
  • Temporal/unilateral headaches (near the TA)
  • Painful/Palpably enlarged temporal artery
  • Generalized muscle ache/stiffness
  • Temporary/permanent blindness (Opthalmic artery)
  • Jaw Claudication

Key indicators of which condition?

A

Giant cell temporal arteritis

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40
Q

Describe the lab findings of Giant cell (temporal) arteritis

A
  • Elevated ESR
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41
Q

How often is a temporal artery biopsy positive?

A

Approximately 60% of cases

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42
Q

This X-ray is indicative of to numerous areas of constriction in the subclavian arteries, patient likely has absent or very weak pulses in the upper extremities. What is the likely diagnosis?

A

Takayasu’s arteritis

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43
Q

Middle aged Asian woman

Presentation:
- Visual disturbances
- Decreased BP in upper extremity
- Absent/very weak pulses in upper extremities

Exams/labs:
- Narrowed aortic arch vessels on angiography

Biopsy:
- Granulomatous inflammation with giant cells

Diagnosis?

A

Takayasu’s arteritis (aka pulseless disease)

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44
Q

Describe the following aspects of Takayasu’s arteritis
- Presentation (key points)
- Exam/labs
- Biopsy

A

Usually middle aged Asian women

Presentation:
- Visual disturbances
- Decreased BP in upper extremity
- Absent/very weak pulses in upper extremities

Exams/labs:
- Narrowed aortic arch vessels on angiography

Biopsy:
- Granulomatous inflammation with giant cells

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45
Q

What are the key differences between Giant cell (temporal) arteritis & Takayasu’s arteritis?

A

GCTA:
- Older women (over 50)

Takayasu’s Arteritis:
- Middle aged Asian women (under 50)

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46
Q

What arteries does Takayasu’s arteritis typically involve?

A

Usually the aorta & aortic arch vessels (carotids, subclavian, pulmonary, renal, & coronary)

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47
Q

Granulomatous inflammation/vasculitis is due to what type of hypersensitivity reaction?

A

Type IV hypersensitivity reaction

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48
Q

What are the findings in the following histological view? What’s the diagnosis?

A
  • Destruction of the arterial media via mononuclear inflammation with giant cells

Diagnosis: Takayasu’s arteritis

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49
Q

Describe the pathophysiology of Takayasu’s arteritis

A

Thickening of the vessels (the aorta & its branches) with narrowing (stenosis) of the lumen causing less blood flow

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50
Q

Describe the key clinical findings of Takayasu’s arteritis

A
  • Dizziness
  • Syncope (fainting)
  • Absent upper extremity pulse
  • Blood pressure discrepancy (low upper extremity BP)
  • Visual disturbance
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51
Q

What is the crucial test to find Takayasu’s

A

Angiography

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52
Q
  • Dizziness
  • Syncope (fainting)
  • Absent upper extremity pulse
  • Blood pressure discrepancy (low upper extremity BP)
  • Visual disturbance

Describes which condition?

A

Takayasu’s arteritis

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53
Q

Young male IV drug abuser with a history of HBV

Presentation:
- Hypertension
- Abdominal pain
- Melena (black/tarry stools)
- Muscle aches/pains
- Skin nodulations

Exams/labs:
- HBsAg +ve
- pANCA +ve

Biopsy:
- Segmental transmural inflammation with fibrinoid necrosis (Blood vessels)

Diagnosis:?

A

Polyarteritis nodosa (PAN)

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54
Q

Describe the following aspects of PAN arteritis
- Presentation (key points)
- Exam/labs
- Biopsy

A

Young male IV drug abuser with a history of HBV

Presentation:
- Hypertension
- Abdominal pain
- Melena (black/tarry stools)
- Muscle aches/pains
- Skin nodulations

Exams/labs:
- HBsAg +ve
- pANCA +ve

Biopsy:
- Segmental transmural inflammation with fibrinoid necrosis (Blood vessels)

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55
Q

Describe the pathophysiology of Polyarteritis nodosa

A

It involves multiple medium sized vessels & it’s associated with segmental transmural inflammation of the vessel walls. This weakens the wall focally which causes dilation (aneurysm) that are responsible for the nodules on the skin

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56
Q

A systemic disease that affects medium & small sized muscular arteries (i.e kidney, heart, liver, GIT, & skin NOT the lungs) with a strong association to HBV antigenemia hypersensitivity to drugs (ex IV amphetamines)

A

Polyarteritis nodosa (PAN)

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57
Q

Polyarteritis nodosa is mediated by which type of Hypersensitivity reaction?

A

Type III hypersensitivity (Immune complex deposition via HBsAg/anti-HBsAg)

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58
Q

The histological slide shows what? What is the likely diagnosis?

A
  • Fibrinoid necrosis & disruption of the vessel wall
  • Neutrophil infiltrates

Likely diagnosis: Polyarteritis

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59
Q

The histological slide shows what? What is the likely diagnosis?

A
  • Segmental fibrinoid necrosis & thrombotic occlusion of the lumen of a small artery
  • Transmural inflammation (all layers)

Diagnosis: Polyarteritis nodosa

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60
Q

Describe the consequences of thrombosis & Weakened focal BV wall in Polyarteritis nodosa

A

Thrombosis = Infarction
Weakened vessel wall = Aneurysms (Kidney, heart, & Gi)

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61
Q

What are the key findings in the target organs (Kidneys, GI, skin, & Coronary arteries) of Polyarteritis nodosa?

A

Ischemic damage:

Kidneys = Vasculitis/infarction causes hypertension, hematuria, & albuminuria

GI tract = Bowel infarction causes abdominal pain & melena

Skin = Ischemic ulcers & nodules

Coronary arteries = Aneurysms & MI

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62
Q

What is the most common cause of death in Polyarteritis nodosa?

A

Renal failure due to MC COD

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63
Q

Describe the common lab findings in Polyarteritis nodosa

A
  • HbsAg +ve in 30% of cases
  • Hematuria with RBC casts
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64
Q

What is the Rx of Polyarteritis nodosa? What is a major complication if it’s left untreated?

A

Rx: Corticosteroids & Cyclophosphamide

Untreated = Almost fatal

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65
Q

Describe the key pathological findings of Churg-Strauss syndrome

A

A variant of PAN it’s a systematic vasculitis that happens in people with asthma (small & medium BVs)

  • Inflammation of the vessel wall (lots of eosinophils
  • Fibrinoid necrosis
  • Thrombosis & infarction
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66
Q

A variant of PAN it’s a systematic vasculitis that happens in people with asthma (small & medium BVs)

  • Inflammation of the vessel wall (lots of eosinophils
  • Fibrinoid necrosis
  • Thrombosis & infarction

Describes which condition?

A

Churg-Stauss syndrome

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67
Q

What’s the Rx for Churg-Strauss syndrome?

A

Corticosteroids

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68
Q

What are the key clinical features of Churg-Strauss syndrome?

A
  • History of atopy
  • Bronchial asthma
  • Allergic rhinitis
  • Peripheral blood eosinophilia
69
Q

What are the usual lab findings for Churg-Strauss syndrome?

A
  • Peripheral eosinophilia
  • High serum IgE
  • pANCA
70
Q

4-yr old Japanese child

Presentation:
- Fever
- Red eyes/mouth
- Rash (trunk/extremities)
- Peeling skin
- Cervical lymphadenopathy
- Edema

Exam/Labs:
- ECG changes consistent with myocardial ischemia

Diagnosis?

A

Kawasaki disease (mucocutaneous lymph node syndrome)

71
Q

Describe the following aspects of Kawasaki Disease
- Presentation (key points)
- Exam/labs
- Biopsy

A

4-yr old Japanese child

Presentation:
- Fever
- Red eyes/mouth
- Rash (trunk/extremities)
- Peeling skin
- Cervical lymphadenopathy
- Edema

Exam/Labs:
- ECG changes consistent with myocardial ischemia

72
Q

Describe the key etiological features and pathophysiology of Kawasaki disease

A

A childhood vasculitis (under 5yrs) more common in Japanese & Hawaiian children

Transmural inflammation of the vessels via neutrophils & fibrinoid necrosis can lead to coronary thrombosis or aneurysm

73
Q

What’s the condition?

A

Kawasaki Disease

74
Q

What’s the condition?

A

Kawasaki disease

75
Q

Describe the clinical findings of Kawasaki disease

A

High fever
Erythematous rash (trunk/extremities)
Desquamation/peeling skin
Mucosal inflammation (red eyes/mouth)
Erythema/Edema of hands/feet
Cervical lymphadenopathy
Acute MI (Children)

76
Q

Describe the common lab findings of Kawasaki disease

A

Neutrophilic leukocytosis
Thrombocytosis **
High ESR
Abnormal ECG (hints at acute MI)

77
Q

What is the Rx of Kawasaki disease?

A
  • Aspirin can be used to reduce the long term sequelae of KD
78
Q

What Rx do you want to avoid giving patients with Kawasaki disease? What are the complications of giving this particular Rx?

A

avoid giving corticosteroids! Unlike most other vasculitis-related conditions it increases the risk for coronary aneurysms/ruptures

79
Q

Young male from Israel who smokes

Presentation:
- Pain in the foot (severe + present at rest)

Exam/Labs:
- Ulcers/necrosis (fingers/toes)
- missing fingers (potentially)

Biopsy:
- Acute inflammation of BV wall with obliteration of the lumen due to a thrombus

Diagnosis?

A

Thromboangitis Obliterans (Buerger’s disease)

80
Q

Describe the following aspects of Thromboangitis Obliterans (Buerger’s disease)
- Presentation (key points)
- Exam/labs
- Biopsy

A

Young male (from Israel NOT always) who smokes

Presentation:
- Pain in the foot (severe + present at rest)

Exam/Labs:
- Ulcers/necrosis (fingers/toes)
- missing fingers (potentially)

Biopsy:
- Acute inflammation of BV wall with obliteration of the lumen due to a thrombus

81
Q

Peripheral vascular disease common in smokers

A

Thromboangitis Obliterans aka Buerger’s disease

82
Q

Describe the features of the earliest stage of Thromboangitis Obliterans (Buerger’s disease)

A
  • Acute inflammation (small/medium arteries) in the extremities
  • Thrombus formation (due to the inflammation)
  • Involvement of the entire neurovascular compartment
83
Q

What does the histo slide show? What’s the condition?

A

An occluded lumen (due to a thrombus) & leukocyte infiltration of the BV wall

Diagnosis: Thromboangitis Obliterans (Buerger’s disease)

84
Q

What are the early manifestations of Thromboangitis Obliterans (Buerger’s disease)

A

Intermittent Claudication of hands/feet & cramping in muscles after exercise (relieved by rest)

85
Q

Age range for Thromboangitis Obliterans (Buerger’s disease)

A

25-40yr old men that are heavy smokers, usually from Israel, Japan, or India

86
Q

What are the late manifestations of Thromboangitis Obliterans (Buerger’s disease)

A

Painful ulceration of digits & eventual gangrene that need amputation

87
Q

How do you diagnose & treat Thromboangitis Obliterans (Buerger’s disease)

A

Diagnose via a biopsy

Treat the early states by smoking cessation

88
Q

What is the key differential between Hypersensitivity (leukocytoclastic) vasculitis a Small vessel vasculitis & Polyarteritis nodosa a medium vessel vasculitis

A

Leukocytoclastic:
- lesions tend to be the same age
- necrotizing glomerulonephritis & involvement of pulmonary capillaries is common
- Purpura are palpable

89
Q

Describe the features of Hypersensitivity (leukocytoclastic) vasculitis

A

A type III hypersensitivity (immune-complex) mediated vasculitis that’s characterized by
- acute inflammation of small BV
- Palpable Purpura (it doesn’t usually affect other organs)

90
Q

Which triggers can precipitate Hypersensitivity (Leukocytosis) vasculitis

A

Exogenous antigens triggered by:
- Drugs (aspirin, penicillin, & thiazide diuretics)
- Infectious organisms (Strep/staph, TB, & viruses)
- Foods

Chronic diseases:
- SLE
- RA

91
Q

Describe the pathological features of Hypersensitivity (Leukocytosis) vasculitis

A

Acute inflammation (small BV)
Leukocytoclastic (neutrophils undergoing karyorrhexis)
Erythrocyte extravasation

92
Q

What findings are in the skin biopsy slide? What’s the likely diagnosis?

A

Fragmentation of neutrophils in & around the BV walls, the likely diagnosis is Hypersensitivity (Leukocytosis) vasculitis

93
Q

What is the skin biopsy & image showing? What condition is this?

A

The image shows:
Hypersensitivity (leukocytoclastic) vasculitis

  • Palpable purpuric tender papules
    The biopsy shows:
  • A BV surrounded by pink fibrin & neutrophils most of which are fragmented (leukocytoclastic)
  • Extravasated RBCs
94
Q

How does Hypersensitivity (Leukocytosis) vasculitis typically present?

A
  • Usually as palpable/non-blancing purpura on the skin of the lower extremities
  • Hemoptysis (coughing blood)
  • Abdominal pain
  • Hematuria
  • Arthralgia
95
Q

How do you diagnose & treat Hypersensitivity (leukocytoclastic) vasculitis

A

Diagnose with a skin biopsy
Rx: Remove the trigger

96
Q

14yr old child with a history of URT

Presentation:
- Polyarthritis*
- Colicky abdominal pain
- Hematuria with RBC casts
- Palpable purpura (lower limbs & butt)*

Exams/Labs:
- Neutrophilic leukocytosis
- IgA-C3 Immune complex deposition in skin & kidneys (Type III hypersensitivity)

Diagnosis:?

A

Henoch Schönlein purpura

97
Q

Describe the following aspects of Henoch Schönlein purpura
- Presentation (key points)
- Exam/labs

A

14yr old child with a history of URT

Presentation:
- Polyarthritis*
- Colicky abdominal pain
- Hematuria with RBC casts
- Palpable purpura (lower limbs & butt)*

Exams/Labs:
- Neutrophilic leukocytosis
- IgA-C3 Immune complex deposition in skin & kidneys (Type III hypersensitivity)

98
Q

Descried the etiopathogenesis of Henoch Schönlein purpura

A

A variant of hypersensitivity vasculitis in CHILDREN. It usually happens after an URT infection & is caused by deposition of IgA-C3 immune complexes in the BV wall

Involves small BV, skin, GI, Kidney, Musculoskeletal system

99
Q
A

Henoch Schonlein purpura (HSP): Patient has multiple palpable purpuric lesions in the lower limbs and buttock area.

100
Q

What is the Rx for Henoch Schönlein purpura?

A

Steroids

101
Q

What is Wegener Granulomatosis characterized by?

A
  • Necrotizing granulomas & vasculitis in the URT/LRT (Granuloma formation with giant cells)
  • Granulomatous vasculitis of URT/LRT & kidneys (small vessels)
  • High association with cANCA
102
Q

What types of hypersensitivity reactions contribute to Wegener Granulomatosis

A

Type II, III, & IV hypersensitivity reactions

103
Q

What do the following slide & image show & what’s the condition?

A

Slide:
- Vasculitis
- Giant cells

Image:
- Fatal Wegener granulomatosis with large nodular lesions

104
Q

Describe the following

A

A saddle nose deformity because the septum is destroyed by granulomatous inflammation

Note congenital syphilis & lepromatous leprosy can have the same deformity

105
Q

Describe the classical triad of Wegener Granulomatosis

A
  1. Granulomas in URT/LRT
  2. Saddle nose
  3. Granulomatous inflammation of kidneys (causes Renal disease i.e. crescentic glomerulonephritis)
106
Q

middle aged men with the peak being between 40-50yrs old

Presentation:
- URT = Chronic sinusitis, ulcers of nasopharyngeal mucosa, & saddle nose
- LRT = Rec. pneumonia with nodular lesions
- Kidney = Cresentric glomerulonephritis

Exams/Labs:
- cANCA (90% cases)
- Bilateral nodular infiltrates/cavitary lesions (CT)

Diagnosis:?

A

Wegener Granulomatosis

107
Q

What’s the diagnostic tool & treatment for Wegener Granulomatosis

A

Biopsy
&
Cyclophosphamide & steroids

108
Q

What are the dangers of treating Wegener Granulomatosis with cyclophosphamide? what’s the death rate without Rx?

A

Hemorrhagic cystitis & transitional cell carcinoma

Death rate without Rx = 80% within a year

109
Q

What are the culprits for fungal vasculitis?

A

MAC:
Mucor
Aspergillus
Candida

110
Q

What are the culprits for Rocky Mountain spotted fever?

A

Caused by Rickettsia rickettsiae (transmitted via the hard dermacentor andersoni tick)

Look for: fever, rash, & hist tick bite!
- Petechial lesions spreading from extremities to trunk

111
Q

What are the culprits for Disseminated meningococcemia?

A

Small vessel vasculitis causing petechial hemorrhages

112
Q

What are the culprits for viral vasculitis?

A

HBV & HCV = immune complex mediated

113
Q

What are the culprits for infective endocarditis?

A

Immune complex vasculitis thought to be responsible for:
- Roth’s spots in retina
- Janeway’s lesions on hands (painless)
- Osler’s nodes on hands (painful)
- Glomerulonephritis

114
Q

Child presents with palpable purpura on the butt & legs, GI pain/bleeding, & hematuria. They previously had a respiratory tract infection. What is the MOA of the disease?

A

Vasculitis due to IgA immune complex deposition (Henoch-Schönlein purpura)

115
Q

Patient with a history of asthma, presents with elevated serum p-ANCA levels & eosinophilia surrounding multiple organs especially the heart & lungs. What is the likely diagnosis?

A

Churg-Strauss Syndrome

116
Q

A middle aged-male with presents with elevated serum p-ANCA, hemoptysis with bilateral nodular lung infiltrates, & hematuria. Upon examination there’s necrotizing vasculitis involving multiple organs especially the kidneys & lungs. What is the condition & how would you treat it?

A

Microscopic Polyangiitis Rx with corticosteroids & cyclophosphamide

117
Q

A middle aged man presents with sinusitis/nasopharyngeal ulceration, hemoptysis with bilateral nodular lung infiltrates, & hematuria. Labs show elevated serum c-ANCA levels. A biopsy would reveal what? What is the condition & the treatment?

A

Biopsy shows = Large necrotizing granulomas with adjacent necrotizing vasculitis

Condition: Wegener Granulomatosis

Rx: Cyclophosphamide & steroids

118
Q

A man who is a heavy smoker comes to the office presenting with blackened finger tips. What is the cause of the necrosis? What is the condition? What is the treatment?

A

Smoking causes necrotizing vasculitis of the digits leading to ulceration, gangrene, & auto amputation.

Condition: Buerger disease (Thromboangitis Obliterans)

Rx: Smoking cessation

119
Q

A 3yr old Asian child is brought to the ER for fever, conjunctivitis, erythematous rash of the palms & feet, & localized cervical lymphadenopathy.
1. What is the condition?
2. Which artery is involved and what is the child at risk of developing?
3. What’s the treatment?

A
  1. Kawasaki disease
  2. Coronary artery involvement can lead to thrombosis with MI which can cause aneurysms & ruptures
  3. Rx with aspirin & IVIG (the disease is self-limiting)
120
Q

A young woman presents to the clinic with hypertension, abdominal pain, tarry stools (melena), neurological disturbances, & skin lesions.
1. What artery is likely involved to be causing melena?
2. What’s the condition?
3. What serum Ab is this condition associated with?
4. What’s the treatment?

A
  1. Mesenteric artery = melena
  2. Polyarteritis nodosa
  3. HBsAg
  4. Rx with corticosteroids & cyclophosphamide (if not treated it’s fatal within 1 yr)
121
Q

A young man with a history of HBV presents to the clinic for a wellness check & constipation yielding black stools. Upon examination it’s determined he has hypertension & imaging shows lesions with a “string of pearls” appearance
1. What’s the condition
2. What do the lesions consist of?

A
  1. Early stage Polyarteritis nodosa
  2. Lesions consist of transmural inflammation with fibrinoid necrosis
122
Q

A 40-yr old female from China presents to the ER with visual disturbances, weak/absent pulse in the upper extremities. Labs show her ESR are markedly elevated.
1. What is the condition?
2. What artery is classically involved?
3. What’s the treatment?

A
  1. Takayasu arteritis
  2. It classically involves granulomatous vasculitis in the aortic arch at the branching points
  3. Rx with corticosteroids
123
Q

A 70-yr old female presents with a headache, visual disturbances, jaw claudication, & flu-like symptoms.
1. A biopsy would be likely to reveal what findings?
2. What’s the condition & arteries involved?
3. What’s the treatment?

A
  1. Inflamed vessel wall with giant cells & intimal fibrosis (Segmental lesions)
  2. Temporal (giant cell) arteritis involving the Temporal & Opthalmic arteries
  3. Rx with corticosteroids (risk of blindness without treatment)
124
Q

Antibodies against neutrophil proteinase 3 indicates, what does it present with & what type of vasculitis?

A

Nasal mucosal ulcerations and hematuria

125
Q

A 5-year-old boy is brought to the physician by his parents because of a 4-day history of arthralgia, abdominal pain, and lesions on his arms and legs. Ten days ago, he had an upper respiratory tract infection. A photograph of his right leg is shown. Further evaluation is most likely to show which of the following?

A

IgA vasculitis (Henoch-Schönlein purpura.)

126
Q

A 63-year-old man comes to the physician because of a 2-day history of fever and blood-tinged sputum. He has also had a productive cough for 1 year and has had 3 episodes of sinusitis during this time. Physical examination shows palpable erythematous skin lesions over his hands and feet that do not blanch on pressure. There are ulcerations of the nasopharyngeal mucosa and a perforation of the nasal septum. His serum creatinine is 2.6 mg/dL. Urinalysis shows acanthocytes, 70 RBCs/hpf, 2+ proteinuria, and RBC casts. An x-ray of the chest shows multiple, cavitating, nodular lesions bilaterally. Further evaluation of this patient is most likely to show which of the following findings?

A

Elevated c-ANCA titers

127
Q

A 54-year-old man comes to the physician because of a cough with blood-tinged sputum for 1 week. He also reports fever and a 5-kg (11 lb) weight loss during the past 2 months. Over the past year, he has had 4 episodes of sinusitis. Physical examination shows palpable nonblanching skin lesions over the hands and feet. Examination of the nasal cavity shows ulceration of the nasopharyngeal mucosa and a depressed nasal bridge. Oral examination shows a painful erythematous gingival enlargement that bleeds easily on contact. Which of the following is the most likely cause of the patient’s symptoms?

A

Neutrophil-mediated damage

128
Q

A 32-year-old woman who recently emigrated to the USA from Japan comes to the physician because of a 3-month history of night sweats, malaise, and joint pain. During this time, she has also had a 6-kg (13-lb) weight loss. Physical examination shows weak brachial and radial pulses. There are tender subcutaneous nodules on both legs. Carotid bruits are heard on auscultation bilaterally. Laboratory studies show an erythrocyte sedimentation rate of 96 mm/h. A CT scan of the chest shows thickening and narrowing of the aortic arch. Microscopic examination of the aortic arch is most likely to show which of the following findings?

A

Granulomatous inflammation of the media, diagnosis is Takayasu’s arteritis

129
Q

A 38-year-old man comes to the physician because of a 3-week history of a painful rash affecting his left foot. For the past 2 years, he has had recurrent episodes of color changes in his fingers when exposed to the cold; his fingers first turn white and then progress to blue and red before spontaneously resolving. He has smoked two packs of cigarettes daily for 20 years. His blood pressure is 115/78 mm Hg. Physical examination shows multiple tender, dark purple nodules on the lateral surface of the left foot with surrounding erythema that follow the course of the lateral marginal vein. There are dry ulcers on the tip of his right index finger and on the distal aspect of his right hallux. Serum lipid studies show no abnormalities. Biopsy of the dorsalis pedis artery will most likely show which of the following findings?

A

Segmental thrombosing inflammation with sparing of the internal elastic lamina

130
Q

A 45-year-old man with asthma comes to the physician because of a 1-month history of progressively worsening shortness of breath and cough. He also has a history of chronic sinusitis and foot drop. Current medications include an albuterol inhaler and inhaled corticosteroid. Physical examination shows diffuse wheezing over both lung fields and tender subcutaneous nodules on both elbows. Laboratory studies show a leukocyte count of 23,000/mm3 with 26% eosinophils and a serum creatinine of 1.7 mg/dL. Urine microscopy shows red blood cell casts. Which of the following is the most likely diagnosis in this patient?

A

Eosinophilic granulomatosis with polyangiitis

131
Q

A previously healthy 48-year-old man comes to the physician for a 3-month history of myalgias and recurrent episodes of retrosternal chest pain and dizziness. He has had a 5-kg (11-lb) weight loss during this period. His temperature is 39.1°C (102.3°F), pulse is 90/min, and blood pressure is 160/102 mm Hg. Physical examination shows lacy, purplish discoloration of the skin with multiple erythematous, tender subcutaneous nodules on the lower legs. Some of the nodules have central ulcerations. Serum studies show an erythrocyte sedimentation rate of 76 mm/h and creatinine level of 1.8 mg/dL. Renal MR angiography shows irregular areas of dilation and constriction in the renal arteries bilaterally. Further evaluation of this patient is most likely to show which of the following?

A

Transmural inflammation with fibrinoid necrosis on arterial biopsy, Diagnosis polyarteritis nodosa

132
Q

transmural inflammation of the arterial wall with leukocytic infiltration and fibrinoid necrosis

A

Polyarteritis nodosa

133
Q

Wegners granulomatosis (Granulomatosis eith polyangitis)

Features include

A
  1. C-ANCA
  2. Effects:
    - Lungs
    - Nasopharynx
    - Kidneys
  3. Small arteries
134
Q
  1. C-ANCA
  2. Effects:
    - Lungs
    - Nasopharynx
    - Kidneys
  3. Small arteries
A

Wegners granulomatosis (Granulomatosis WITH polyangitis)

135
Q

Microscopic polyangitis

features include:

A
  1. P-ANCA
  2. Lungs & Kidneys only
  3. Small arteries
136
Q
  1. P-ANCA
  2. Lungs & Kidneys only
  3. Small arteries
A

Microscopic polyangitis

features include:

137
Q

Henoch-Schonlein purpura (IgA vasculitis)

Features includes:

A

It’s due to IgA deposits in the arteries (Small)

Hematuria
Palpable purpura
Arthralgia
Stomach pain**
Recent URI (B19 or S.aureus)**

138
Q

It’s due to IgA deposits in the arteries (small)

Hematuria
Palpable purpura
Arthralgia
Stomach pain**
Recent URI (B19 or S.aureus)**

A

Henoch-Schonlein purpura (IgA vasculitis)

Features includes:

139
Q

Cryoglobulinemic vasculitis (Hep C vasculitis)

Features include

A

Due to a Hep C infection (small arteries)

Hematuria
Palpable purpura
Arthralgia
Hep C & Cryoglobulin deposits **

Rx the underlying Hep C

140
Q

Chrug-Strauss (Eosinophilic granulomatous with polyangitis)

Features include:

A

Causes necrotizing eosinophilic granulomatous infiltration of arteries (small)

141
Q

Causes necrotizing eosinophilic granulomatous infiltration of arteries (small)

A

Chrug-Strauss (Eosinophilic granulomatous with polyangitis)

Features include:

142
Q

Polyarteritis nodosa (PAN)

Features include:

A

Causes transmural arteritis with fibrinoid necrosis

Involves renal A, coronary A, & Mesenteric A (med)

String of pearls (pattern of necrosis0

143
Q

Causes transmural arteritis with fibrinoid necrosis

Involves renal A, coronary A, & Mesenteric A (med)

String of pearls (pattern of necrosis0

A

Polyarteritis nodosa (PAN)

Features include:

144
Q

Buerger’s (Thromboangitis Obliterans)

A

Usually men 30-50yrs old, caused by heavy smoking & treated with cessation (med arteries)

Distal digit thrombosis (segmental thrombosis)
Autoamputation of digits

145
Q

Usually men 30-50yrs old, caused by heavy smoking & treated with cessation (med arteries)

Distal digit thrombosis (segmental thrombosis)
Autoamputation of digits

A

Buerger’s (Thromboangitis Obliterans)

146
Q

Kawasaki

Features include:

A

Necrotizing vasculitis involving the coronary artery in (Conjunctivitis) children (Med arteries)

MI in kids
Cervical lymphadenopathy
Strawberry tongue
Arthralgia/edema hand/feet
Conjunctivitis
Desquamating skin

Rx with aspirin & immunoglobulins (side effect retts)

147
Q

Necrotizing vasculitis involving the coronary artery in (Conjunctivitis) children (Med arteries)

MI in kids
Cervical lymphadenopathy
Strawberry tongue
Arthralgia/edema hand/feet
Conjunctivitis
Desquamating skin

Rx with aspirin & immunoglobulins (side effect retts)

A

Kawasaki

Features include:

148
Q

Takayasu (Pulseless disease)

Features include:

A

Transmural fibrous thickening of aortic branches, usually Japanese women under 40yrs (large arteries)

Subclavian artery degeneration
Weak/absent upper pulse
low/uneven upper BP
Paresthesia

149
Q

Transmural fibrous thickening of aortic branches, usually Japanese women under 40yrs (large arteries)

Subclavian artery degeneration
Weak/absent upper pulse
low/uneven upper BP
Paresthesia

A

Takayasu (Pulseless disease)

Features include:

150
Q

Giant cell (Temporal) arteritis

Features include:

A

Intimal thickening & lamina fragmentation (large arteries)

High ESR
Jaw claudication
Temporal headaches (TA)
Visual disturbances (OA)
Polymyalgia rheumatica

Rx corticosteroids before biopsy!!!

151
Q

Intimal thickening & lamina fragmentation (large arteries)

High ESR
Jaw claudication
Temporal headaches (TA)
Visual disturbances (OA)
Polymyalgia rheumatica

Rx corticosteroids before biopsy!!!

A

Giant cell (Temporal) arteritis

Features include:

152
Q

Vasculitis with IgA deposition in response to a GI or Respiratory tract infection (mucosal layers)

A

Henoch Schönlein purpura

Comon in children, presents with non-blanching/ palpable purpura on the butt & legs

153
Q

What would you see histologically in someone with Giant cell (temporal) arteritis?

A

Granulomatous inflammation with giant cells (type IV hypersensitivity)

154
Q

Granulomatous inflammation with giant cells (type IV hypersensitivity)

Elevated ESR
Jaw claudication
Headache

A

Giant cell (temporal) arteritis

155
Q

What’s the sequelae of giant cell arteritis? What do we administer to avoid it?

A

Retinopathy (blindness) treat with corticosteroids right way

156
Q

Giant cell granulomatous inflammation within the branches of the aortic arch (esp, subclavian arteries)

A

Takayasu

157
Q

Negative ANCA
Distal segmental thrombosing vasculitis
Ulcers/necrotic digits

A

Bueger’s (Thromboangitis Obliterans)

Common in smokers

158
Q

Segmental transmural inflammation of the BV wall with fibrinoid necrosis (type III)

Never involves the lungs & is associated with which infection?

A

Polyarteritis nodosa associated with HBV (HBsAg)

Abdominal pain
Melena
Hypertension
String of pears app
Skin/neuro symptoms

159
Q

Allergic type vasculitis associated with asthma

Necrotizing granulomatous angiitis

P-ANCA +ve

A

Churg-Strauss (lungs & renal system involvement)

160
Q

Primarily affects the coronary artery in children under 4yrs old

What do you treat it with & what substance secrete by platelet cells does the treatment inhibit

A

Kawasaki

Rx with Ig’s IV aspirin to inhibit thromboxane A2 (COX2/TXA2) from platelets

Acute MI
Conjunctivitis
Strawberry tongue
Desquamation
Erythematous rash hand/feet
Edema
Cervical lymphadenopathy

161
Q

C-ANCA +ve

Necrotizing granulomatous lesions of the nose, sinus, kidney, lungs, (nodular infiltrates & cavitary lesions)

Post UTR/LRT infection

A

Wegeners granulomatosis

Hematuria (glomerulonephritis)
Gi pain
**Sinusitis
Hemoptysis

Rx Cyclophosphamide (side effects can be hemorrhagic cystitis or transitional cell cancer)

162
Q

P-ANCA +ve

Necrotizing vasculitis in organs like the lungs & kidneys

A

Micropoly angiitis

163
Q
A

Wegener’s granulomatosis

164
Q
A

Giant cell granuloma

165
Q
A
166
Q
A
167
Q

Key clinical features of Giant cell temporal arteritis:

A
  • Temporal/unilateral headaches (near the TA)
  • Painful/Palpably enlarged temporal artery
  • Generalized muscle ache/stiffness
  • Temporary/permanent blindness (Opthalmic artery)
  • Jaw Claudication
168
Q

Describe the findings in the histo slide & the likely diagnosis

A

The H&E stain shows a section of a temporal artery with:
- Giant cells
- Degenerated internal elastic membrane

Likely diagnosis: Temporal (giant cell) arteritis

USMLE Pathoma (20 decks)

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