22 - 139 - SYSTEMIC NECROTIZING ARTERITIS

Question 1

Behcet disease is markedly more common in these countries, except one:

A. Eastern Mediterranean

B. Northern Europe

C. Korea

D. Japan

Answer 1

B

Question 2

Infection-related vasculitis: _____ in many cases of polyarteritis nodosa

A. Hepatitis B virus

B. Hepatitis C virus

C. EBV

D. Parvovirus

Answer 2

A

Question 3

HLA locus in giant cell arteritis

A. Class I

B. Class II

C. Class III

D. Class IV

Answer 3

B

Question 4

For drug-induced vasculitis, it is useful to ask about prescription, nonprescription, and “alternative” or herbal medication use in the prior

A. 1 to 3 months

B. 3 to 6 months

C. 6 to 12 months

D. >12 months

Answer 4

C

Question 5

Tests for _____ are the most important to order in evaluating a patient suspected of having vasculitis

A. Cardiac disease

B. Hematologic disease

C. Liver disease

D. Renal disease

Answer 5

D

Question 6

90% of patients with _____ will test positive for ANCA

A. Granulomatosis with polyangiitis

B. Eosinophilic granulomatosis with polyangiitis

C. Granulomatous with polyangiitis

D. Microscopic polyangiitis

Answer 6

D

Question 7

Cutaneous leukocytoclastic angiitis: _____% of cases follow a wide range of infections

A. 10

B. 20

C. 30

D. 40

Answer 7

B

Question 8

Typical presentation of Takayasu arteritis

A. Pulselessness

B.Ulcer

C.claudication

D.Purpura

Answer 8

C

Question 9

Mainstay of therapy for vasculitis

A. Glucocorticoids

B. Methotrexate

C. Glucocorticoid + Methotrexate

D. Glucocorticoid + Cyclophosphamide

Answer 9

A

Question 10

Monoclonal antibody to anti-interleukin 5 Demonstrated to have efficacy in the treatment of eosinophilic granulomatosis with polyangiitis

A. Tocilizumab

B. Rituximab

C. Dupilumab

D. Mepolizumab

Answer 10

D

Question 11

Examples of small vessel vasculitis

Answer 11

Immune complex mediated:
* Cryoglobulinemia
* Henoch–Schönlein purpura (HSP)

ANCA-associated:
* microscopic polyangiitis (MPA)
* Churg–Strauss syndrome (CSS)
* granulomatosis with polyangiitis (Wegener)

Question 12

examples of medium vessel vasculitis

Answer 12

Kawasaki Disease
Polyarteritis nodosa

Question 13

examples of large vessel vasculitis

Answer 13

• Giant cell arteritis
• Takayasu arteritis
• Isolated aortitis

Question 14

vasculitis with no predominant size

Answer 14

Behcet
Primary CNS
Relapsing Polychondritis
Cogan

Question 15

Ulceration and digit ischemia are more readily produced in vasculitis of what size?

Answer 15

medium vessel

Question 15

classic presentation of small-vessel vasculitis

Answer 15

palpable purpura

• but other presentations are common.
• These include nonpalpable purpura that can be either round or have angular borders (retiform purpura), papules, urticarial lesions, nodules of different sizes and at different depths, bullous lesions, and ulcers

Question 16

infection associated with polyarteritis nodosa

Answer 16

hepatitis B virus

Question 17

infection associated with cryoglobulinemic vasculitis

Answer 17

hepatitis C virus

Question 18

ANCA are directed against what neutrophil proteins?

Answer 18

myeloperoxidase (MPO) or proteinase-3 (PR3)

• they are primarily contained in intracellular granules but are also translocated to the cell surface or released via degranulation or excretion of neutrophil extracellular traps (NETs), allowing access to ANCA, activation of neutrophils, involvement of complement components, and necrotizing vasculitis.

Question 19

single most important component of the early evaluation of a patient suspected of having vasculitis.

Answer 19

full review of systems with assessment of the overall severity of illness

Question 20

In cases of drug-induced vasculitis, it is useful to ask about prescription, nonprescription, and “alternative” or herbal mediation use in the prior to how many months?

Answer 20

**6 to 12 months **since the effect of some mediations may persist after usage ends.

Question 21

It is generally recommended to biopsy a skin lesion that has been clinically apparent for less than how many hours?

Answer 21

less than 48 hours

to maximize the chance of finding the typical features of acute neutrophilic vasculitis, including fibrinoid necrosis, extravasation of erythrocytes, extravasation of neutrophils with release of nuclear debris (leukocytoclasia), and the presence of immune deposits.

Question 22

the most important laboratory exam to order in evaluating a patient suspected of having vasculitis

Answer 22

test for renal disease

because renal disease is common in many vasculitides and is rarely accompanied by signs or symptoms until end-stage renal failure occurs

Question 23

blood exam abnormality found in most untreated patients with eosinophilic granulomatosis with polyangiitis (Churg–Strauss)

Answer 23

elevated absolute eosinophil

count greater than 1000 cells/µL helps differentiate this disease from asthma and atopy

Question 24

what are the 3 ACA-associated vasculitis

Answer 24

1. Microscopic polyangiitis 2. Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) 3. Granulomatosis with polyangiitis (Wegener)

Question 24

Total hemolytic complement is measured using what assay?

Answer 24

CH50 ## Footnote but since this assay is cumbersome and suffers from variability between laboratories, measurement in serum of the complement proteins C3 and C4 is usually sufficient, and is useful for assessing patients with cutaneous vasculitis in several settings.

Question 24

test with high specificity for ANCA-associated vasculitis

Answer 24

anti-PR3 and anti-MPO antibodies ## Footnote positive tests for ANCA by ELISA are essential to consider ANCA testing positive for purposes of diagnosing vasculitis

Question 25

these illicit drugs have been associated with cutaneous vasculitis and/or arterial vasospasm,

Answer 25

cocaine and methamphetamines

Question 26

* multisystem vasculitis of **small** and also sometimes **medium**-sized vessels * Pauci-immune **glomerulonephritis** develops in the majority of patients, and **pulmonary** hemorrhage, **peripheral** and **cranial** **neuropathy**, **musculoskeletal**, and constitutional symptoms are also common * most are (+) for ANCA, usually with specificity for antibodies to myeloperoxidase (MPO) * most common cutaneous lesion is palpable purpura and the pathology is indistinguishable from other types of leukocytoclastic vasculitis * most common cutaneous lesion is **palpable purpura** and the pathology is indistinguishable from other types of leukocytoclastic vasculitis

Answer 26

Microscopic polyangiitis (MPA)

Question 27

Wegener granulomatosis is also known as

Answer 27

Granulomatosis with polyangiitis

Question 28

Churg-Strauss Syndrome is also known as

Answer 28

Eosinophilic granulomatosis with polyangiitis

Question 29

* encompasses **all the features of microscopic polyangiitis **but also many additional manifestations caused by **necrotizing granulomatous inflammation** * Chronic **inflammation of the upper airway** (nasal cavity, sinuses, auditory tube, and middle ear) is present in about 90% of patients * Vasculitis of the **eye** (scleritis and episcleritis) * majority ANCA type in this disease is** C-ANCA/antiPR3; **however, P-ANCA/anti-MPO is also not uncommon. * palpable purpura, neutrophilic and granulomatous dermatitis with **papules** (particularly on the extensor surfaces of the elbows), subcutaneous **nodules**, and **ulcers**

Answer 29

Granulomatosis with polyangiitis (Wegener Syndrome)

Question 30

* history of **asthma** (often severe or poorly controlled), and **blood eosinophilia** * Nasal **polyps**, constitutional symptoms, and **rashes**, all typical of atopy, are also common * The presence of **pulmonary infiltrates on chest imaging **(eosinophilic pneumonia) provides an important distinction from asthma * Palpable and nonpalpable **purpura**, reflecting vasculitis histologically, comprise about 50% of cutaneous lesions *** Eosinophilic dermatitis and granulomatous dermatitis **rich in both neutrophils and eosinophils are also common and produce erythematous macules, papules, and nodules.

Answer 30

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome) ## Footnote Cutaneous disease is more common in eosinophilic granulomatosis with polyangiitis than in systemic granulomatous vasculitis or microscopic polyangiitis.

Question 31

The most common presentation of** severe vasculitis **in e**osinophilic granulomatosis with polyangiitis**

Answer 31

**acute peripheral neuropathy** with involvement of the heart, GI tract, brain, and eyes less commonly

Question 32

histologic term defined by the absence of evidence of systemic disease

Answer 32

Cutaneous leukocytoclastic angiitis

Question 33

medications that can cause drug-induced ANCA-associated vasculitis

Answer 33

propylthiouracil and hydralazine

Question 34

* Idiopathic vasculitis of **medium**-sized vessels * * involves multiple organ systems and presents with some combination of skin disease, myalgia, hypertension (from renal artery involvement), abdominal pain, neuropathy, and/or testicular pain * many cases were associated with chronic infection with **hepatitis B,**

Answer 34

POLYARTERITIS NODOSA

Question 35

The most common cutaneous features of polyarteritis nodosa

Answer 35

livedo reticularis/racemosa, painful cutaneous nodules or ulcers and digital ischemia

Question 36

These manifestations reflect vasculitis of “medium-sized” arteries in the subcutis

Answer 36

livedo reticularis/racemosa, painful cutaneous nodules or ulcers and digital ischemia

Question 37

* currently considered strictly a disease of adults **older than 50 years **that is markedly more common with advancing age * mostly a disease of people of **Northern European** ancestry * **Cranial arteritis** is a common feature with this term indicating stenosis or occlusion of 1 or more branches of the **carotid artery **to produce headache (70% to 80%), jaw claudication (50%), and monocular (and rarely binocular) blindness (15%) *** Polymyalgia rheumatica**, which includes pain and stiffness of the shoulder and hip girdles, is seen in at least 30% to 40% * Constitutional symptoms are common, including fever, malaise, and weight loss * Diagnosis is usually confirmed by temporal artery biopsy

Answer 37

Giant cell arteritis (temporal arteritis)

Question 38

present in 30% to 40% of cases and is** the only cutaneous manifestation of giant cell arteritis apparent on examination**

Answer 38

Palpable nodularity of the temporal artery

Question 39

Takayasu arteritis is a rare form of vasculitis (prevalence <1:100,000) involving what blood vessel?

Answer 39

aorta and its major branches.

Question 40

The typical presentation of Takayasu

Answer 40

limb claudication.

Question 41

ancillary to diagnose Takayasu arteritis

Answer 41

Angiography

Question 42

2 phases of treatment of vasculitis

Answer 42

**1. Remission Induction ** * high-dose glucocorticoids with steady dose taper combined with a short course (3-6 months) of a fairly rapid-acting and potent immunosuppressive agent * **cyclophosphamide, rituximab, and methotrexate** - all have roles in remission induction for ANCAassociated vasculitis **2. Remission Maintenance** * prolonged use of a non–cyclophosphamidebased regimen to allow for glucocorticoids to be either fully discontinued or maintained at a low dose (eg, ≤10 mg prednisone daily)

Question 43

medications that have roles in remission induction for ANCA-associated vasculitis

Answer 43

cyclophosphamide, rituximab, and methotrexate

Question 44

when is watchful waiting a reasonable approach in patients with vasculitis?

Answer 44

(1) a diagnosis is not clear and no major organ system appears threatened; (2) there is an obvious cause or etiology for the vasculitis that is either reversible (toxin/drug) or self-limited (some infections); (3) uncertainly exists as to whether the patient’s symptoms are due to a flare of disease or the result of either chronic damage or another process.

Question 45

* mainstay of therapy for vasculitis given the rapidity of action, reliability of response, and physicians’ familiarity with dosing and side effects * are usually the initial drug used to treat vasculitis and may be the only agent used for some forms of the disease

Answer 45

Glucocorticoids

Question 46

non-cyclophosphamide drugs that can be given for remission maintenance of vasculitis

Answer 46

methotrexate, azathioprine, mycophenolate, cyclosporine

Question 47

This drug has now been demonstrated to have efficacy in the treatment of **mucocutaneous manifestations of Behçet disease**

Answer 47

Apremilast

Question 48

biologics that can be given in treating vasculitis

Answer 48

* **rituximab** * **Mepolizumab** - monoclonal antibody to anti-interleukin 5; for eosinophilic granulomatosis with polyangiitis * **tocilizumab** - monoclonal antibody to interleukin 6; for giant cell arteritis * **abatacept** - CTLA-4 immunoglobin, to treat giant cell arteritis

Question 49

This monoclonal antibody to** anti-interleukin 5,** has been demonstrated to have efficacy in the treatment of **eosinophilic granulomatosis with polyangiitis**

Answer 49

Mepolizumab

Question 50

monoclonal antibody to interleukin 6, for the treatment of giant cell arteritis

Answer 50

tocilizumab

Question 51

CTLA-4 immunoglobin, to treat giant cell arteritis

Answer 51

abatacept

Question 52

Medical Emergencies Related to the Primary Vasculitides

Answer 52