Pathoma - Vasculitis - Fundamentals Of Pathology

Front 1

Vasculitis is defined as ________________.

Back 1

Inflammation of the blood vessel wall (arterial or veins)

Front 2

The wall of a blood vessel is composed of what layers?

Back 2

Tunica intima (endothelial cells sitting on basement membrane)
Tunica media (smooth muscle)
Tunica adventitia (connective tissue layer)

Front 3

Most cases of vasculitidies are NOT ____________.

Back 3

infectious

Front 4

Etiology of vasculitis is usually __________.

Back 4

unknown

Front 5

Clinically we can get two types of symptoms when patients present with a vasculitis. Explain.

Back 5

(1) Nonspecific symptoms
- Inflammation --> fever, weight loss, fatigue, myalgia etc. (seen in most vasculitides)
(2) Symptoms of organ ischemia
- Damage of wall --> Thrombus. After damage, healing results in fibrosis, narrowing wall with ischemia.

Front 6

Vasculitides are divided into what categories?

Back 6

Large-, medium- small-vessel vasculitis

Front 7

Large vessel vasculitis involves ___________.

Back 7

the aorta or its branches

Front 8

This is the MC form of vasculitis in older individuals. It usually affects ___________ (males/females)

Back 8

Temporal (giant cell) arteritis.

Usually affects females

Front 9

Temporal (Giant cell) arteritis is classically seen in older adults age ________.

Back 9

> 50 yo.

Front 10

What arteries are classically involved in temporal arteritis?

Back 10

Branches of carotid artery

Front 11

Describe clinical symptoms in context of vessel involved in temporal arteritis. Also mention other symptoms.

Back 11

(1) Headache (temporal)
(2) Visual disturbances (ophthalmic)
(3) Arteries that feed the jaw or mm. of jaw giving us jaw claudication.

Other: flu-like symptoms with joint and muscle pain.

Front 12

What is the disease called when a patient with temporal arteritis has joint and muscle pain with flu symptoms?

Back 12

Polymyalgia Rheumatica. Seen in a subset of patients with temporal arteritis.

Front 13

What lab parameter is elevated in temporal arteritis?

Back 13

ESR (greater than 100)

Front 14

What is seen on biopsy of an artery affected by temporal arteritis?

Back 14

Inflamed vessel wall with giant cells (granulomatous vasculitis) and intimal fibrosis.

Front 15

The lesions in temporal arteritis are ______________ (continuous/segmental).

Back 15

segmental

Front 16

What is something important to remember when performing biopsy of an artery in a patient with temporal arteritis?

Back 16

Take a very long piece of the vessel, because lesions are segmental. Check each segment.

Front 17

Temporal arteritis: You receive a negative biopsy. Does this exclude disease?

Back 17

No. You've taken a piece of the vessel where disease is not present.

Front 18

What is this?

Back 18

Temporal arteritis. Look at all the fibrosis (F) separating intima (I) from the media (M). Lumen is narrowed. Also note inflammation and giant cells.

Front 19

Treatment of temporal arteritis?

Back 19

Corticosteroids.

Front 20

Why should you give treatment as soon as you suspect (even if you don't have confirmation on biopsy) temporal arteritis.

Back 20

There is a high risk of blindness without treatment.

Front 21

Temporal (giant cell) arteritis is a _____________ (large/medium vessel) vasculitis.

Back 21

Large vessel

Front 22

Takayasu arteritis is a _____________ (large/medium vessel) vasculitis.

Back 22

large

Front 23

Takayasu arteritis is the same disease as temporal arteritis (it's a spectrum), however, there are some exceptions

Back 23

(1) In temporal arteritis we see it in patients greater than 50, in takayasu we see it in patients less than 50 yo.

(2) Takayasu is more proximal than temporal. Involves the aortic arch at branch points.

Front 24

What type of inflammation do we have in takayasu?

Back 24

Same as temporal; granulomatous inflammation.

Front 25

How is takayasu and temporal arteritis similar?

Back 25

(1) Granulomatous inflammation
(2) Large vessel vasculitis
(3) ESR elevated
(4) Treatment is corticosteroids

Front 26

Regarding Takayasu arteritis, involvement of the ____________ at _______ points results in ________ and _________ symptoms.

Back 26

aortic arch; branch; visual; neurologic

Front 27

What arteries are involved in Takayasu arteritis?

Back 27

Aortic arch at branch points.

Front 28

What does involvement of the aortic arch at branch points in Takayasu arteritis lead to?

Back 28

(1) Visual and neurologic symptoms
(2) Weak or absent pulse in an upper extremity ('pulseless disease')

Front 29

Medium-vessel vasculitides involve what?

Back 29

Muscular arteries that supply organs.

Front 30

What defines a medium-sized vessel?

Back 30

Muscular arteries (e.g. renal artery)

Front 31

What "type" of vasculitis occurs in polyarteritis nodosa?

Back 31

Necrotizing vasculitis

Front 32

What organ(s) are involved in polyarteritis nodosa?

Back 32

Most organs (polyarteritis = many arteries)

Front 33

What organ is spared in polyarteritis nodosa?

Back 33

Lungs

Front 34

Polyarteritis nodosa is a ______________ (large/medium/small sized vessel) vasculitis.

Back 34

medium

Front 35

Polyarteritis nodosa typically presents in ___________.

Back 35

young adults

Front 36

Polyarteritis nodosa affecting the renal artery would present as _________.

Back 36

HTN

Front 37

Polyarteritis nodosa affecting the mesenteric artery would present as _________.

Back 37

Abdominal pain with melena

Front 38

What are classic findings in Polyarteritis nodosa?

Back 38

(1) HTN
(2) Abdominal pain with melena
(3) Neurologic disturbances
(4) Skin lesions

Front 39

PN is associated with what serum molecule?

Back 39

HBsAg

Front 40

An early lesion in PN is?

Back 40

Transmural inflammation with fibrinoid necrosis of a vessel

Front 41

Why is the disease called polyarteritis NODOSA?

Back 41

When lesions heal after inflammation, massive fibrosis in that particular segment makes the artery seem hard (like a node).

Front 42

True or false: In PN all the lesions are of the same stage.

Back 42

False, lesions of varying stage are present.

Front 43

What is a consequence of lesions of varying stage in PN in clinical diagnosis?

Back 43

'String-of-pearl' appearance on imaging.

In areas where inflammation is present, we may weaken the wall and get an aneurysm. Aneurysm-fibrosis-aneurysm-fibrosis may form this appearance.

Front 44

What is seen?

Back 44

Example of fibrinoid necrosis.

Front 45

Treatment of PN?

Back 45

(1) Corticosteroids
(2) Cyclophosphamide

Front 46

Kawasakis disease is a _____________ (medium/small vessel) vasculitis.

Back 46

medium

Front 47

Kawasaki's disease classically affects ___________ children _____ years old.

Back 47

asian; < 4 yo

Front 48

How do children with Kawasaki's disease present?

Back 48

Non-specific vague symptoms.

(1) Fever
(2) Conjunctivitis
(3) Erythematous rash on palms and soles
(4) Enlarged cervical lymph nodes

Front 49

The preferential artery that is involved in Kawasaki's is the _________.

Back 49

coronary artery (it is common)

Front 50

What is a dreaded complication(s) of Kawasaki's?

Back 50

(1) Thrombosis with MI.
(2) Aneurysm with rupture.

Front 51

Treatment of Kawasaki's disease?

Back 51

(1) Aspirin (prevent thrombosis)
(2) IV IG

Front 52

Kawasaki's disease is ______________ (pervasive/self-limited).

Back 52

self-limited

Front 53

Memory help: How could you think about Kawasaki's disease?

Back 53

Picture a 4-yo on a Kawasaki motorcycle. They would be using their palms and soles to drive (rash). Heart rate goes up when you drive a motorcycle (MI).

Front 54

Buerger's disease is a ______________ (small/medium/large vessel) vasculitis.

Back 54

medium

Front 55

What is Buerger's disease (short)?

Back 55

Necrotizing vasculitis involving the digits.

Front 56

How does Buerger's disease present?

Back 56

Presents with ulceration, gangrene, and autoamputation of fingers and toes.

Front 57

This vasculitis is highly associated with smoking.

Back 57

Buerger's disease.

Front 58

Treatment of Buerger's disease?

Back 58

Smoking cessation

Front 59

A girl experiences pale digits some time after she first started smoking. What is it called? What is it associated with?

Back 59

Raynaud's syndrome.

Associated with Burger's.

Front 60

What is the color changes seen in Raynaud's phenomenon?

Back 60

White (compromised blood flow) to blue (cyanosis) to red (reperfusion)

Front 61

Small vessel vasculitis involves ________, ________ and _________.

Back 61

arterioles; capillaries; venules

Front 62

Wegener's granulomatosis is a _______________ (small/medium/large vessel) vasculitis.

Back 62

small

Front 63

What is Wegener's granulomatosis?

Back 63

Necrotizing granulomatous vasculitis involving the nasopharynx, lungs, kidneys.

Front 64

Mnemonic version of the name of Wegener's granulomatosis?

Back 64

weCener's granulomatosis
("The C disease")

(1) C-ANCA
(2) C-distribution
(3) Cyclophosphamide

Front 65

Wegener's granulomatosis classically presents in what subgroup of patients?

Back 65

Middle-aged males

Front 66

Wegener's granulomatosis classically presents as?

Back 66

(1) Sinusitis or nasopharyngeal ulceration
(2) Hemoptysis with bilateral nodular lung infiltrates
(3) Hematuria due to RPGN

Front 67

The hematuria that may be present in Wegener's granulomatosis is due to what pattern of disease in the kidney?

Back 67

RPGN

Front 68

What molecule could tell you something about Wegener's granulomatosis?

Back 68

C-ANCA. Levels correlate with disease activity.

Front 69

On biopsy in a patient with Wegener's granulomatosis you would see what?

Back 69

Large necrotizing granulomas with adjacent necrotizing vasculitis.

Front 70

Key treatment in Wegener's granulomatosis?

Back 70

Cyclophosphamide and corticosteroids

Front 71

After treatment, Wegener's usually ____________ (remain in remission/relapse).

Back 71

Relapse (relapses are common)

Front 72

Microscopic polyangiitis is a ____________ (small/medium/large vessel) vasculitis.

Back 72

small (microscopic)

Front 73

What is microscopic polyangiitis?

Back 73

Necrotizing vasculitis involving multiple organs, especially lung and kidney.

Front 74

What is the difference in the pattern of involvement of organs in microscopic polyangiitis and wegener's?

Back 74

Wegener's: Nasopharynx, lung, kidney
Microscopic: Especially lung and kidney

Front 75

Microscopic polyangiitis is similar to Wegener's, however, two things are absent in this disease. What is it?

Back 75

(1) Nasopharyngeal involvement is absent
(2) Granulomas are absent
(3) P-ANCA instead of C-ANCA (Wegener's)

Front 76

What molecule could tell you something about microscopic polyangiitis?

Back 76

Serum P-ANCA levels correlate with disease severity.

Front 77

She has a vasculitis. What could it be?

Back 77

Saddle nose deformity in Wegener's (Another C!)

Front 78

Treatment of Microscopic polyangiitis?

Back 78

Similar to Wegener's. Cyclophosphamide and corticosteroids.

Front 79

What is a common occurrence after treatment of Microscopic polyangiitis.

Back 79

Same as for Wegener's, recurrence is common.

Front 80

Churg-Strauss syndrome is a ____________ (small/medium/large vessel) vasculitis.

Back 80

small

Front 81

What is Churg-Strauss syndrome?

Back 81

Necrotizing granulomatous vasculitis with eosinophils.

Front 82

Churg-strauss syndrome involves what organs?

Back 82

Multiple organs, especially lungs and heart.

Front 83

A patient has a vasculitis. You observe a P-ANCA in her blood. What vasculitides could this be? How to differentiate?

Back 83

Churg-strauss or Microscopig polyangiitis.

Differentiation:
- CS has granulomas, MP not.
- CS usually has asthma and peripheral eosinophilia

Front 84

What is HSP?

Back 84

Henoch-Schönlein purpura.

Vasculitis due to IgA IC deposition.

Front 85

HSP is the MC vasculitis in what age group?

Back 85

Children

Front 86

Classical presentation of HSP?

Back 86

(1) Palpable purpura on buttocks and legs
(2) GI pain and bleeds
(3) Hematuria (IgA nephropathy)

Front 87

In HSP, Ig__ deposits in the ___________ of the kidneys.

Back 87

IgA; mesangium

Front 88

HSP usually follows what?

Back 88

URT infection

Front 89

HSP is ___________ (pervasive/self-limiting).

Back 89

self-limiting

Front 90

A boy has HSP that abates. Can it recur?

Back 90

It may recur

Front 91

HSP is treated with?

Back 91

Steroids if severe, but it is self-limited.