22 - 139 - SYSTEMIC NECROTIZING ARTERITIS Flashcards
Behcet disease is markedly more common in these countries, except one:
A. Eastern Mediterranean
B. Northern Europe
C. Korea
D. Japan
B
Infection-related vasculitis: _____ in many cases of polyarteritis nodosa
A. Hepatitis B virus
B. Hepatitis C virus
C. EBV
D. Parvovirus
A
HLA locus in giant cell arteritis
A. Class I
B. Class II
C. Class III
D. Class IV
B
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
C
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
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
D
Cutaneous leukocytoclastic angiitis: _____% of cases follow a wide range of infections
A. 10
B. 20
C. 30
D. 40
B
Typical presentation of Takayasu arteritis
A. Pulselessness
B.Ulcer
C.claudication
D.Purpura
C
Mainstay of therapy for vasculitis
A. Glucocorticoids
B. Methotrexate
C. Glucocorticoid + Methotrexate
D. Glucocorticoid + Cyclophosphamide
A
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
D
Examples of small vessel vasculitis
Immune complex mediated:
* Cryoglobulinemia
* Henoch–Schönlein purpura (HSP)
ANCA-associated:
* microscopic polyangiitis (MPA)
* Churg–Strauss syndrome (CSS)
* granulomatosis with polyangiitis (Wegener)
examples of medium vessel vasculitis
Kawasaki Disease
Polyarteritis nodosa
examples of large vessel vasculitis
- Giant cell arteritis
- Takayasu arteritis
- Isolated aortitis
vasculitis with no predominant size
Behcet
Primary CNS
Relapsing Polychondritis
Cogan
Ulceration and digit ischemia are more readily produced in vasculitis of what size?
medium vessel
classic presentation of small-vessel vasculitis
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
infection associated with polyarteritis nodosa
hepatitis B virus
infection associated with cryoglobulinemic vasculitis
hepatitis C virus
ANCA are directed against what neutrophil proteins?
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.
single most important component of the early evaluation of a patient suspected of having vasculitis.
full review of systems with assessment of the overall severity of illness
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?
**6 to 12 months **since the effect of some mediations may persist after usage ends.
It is generally recommended to biopsy a skin lesion that has been clinically apparent for less than how many hours?
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.
the most important laboratory exam to order in evaluating a patient suspected of having vasculitis
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
blood exam abnormality found in most untreated patients with eosinophilic granulomatosis with polyangiitis (Churg–Strauss)
elevated absolute eosinophil
count greater than 1000 cells/µL helps differentiate this disease from asthma and atopy
what are the 3 ACA-associated vasculitis
- Microscopic polyangiitis
- Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome)
- Granulomatosis with polyangiitis (Wegener)
Total hemolytic complement is measured using what assay?
CH50
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.
test with high specificity for ANCA-associated vasculitis
anti-PR3 and anti-MPO antibodies
positive tests for ANCA by ELISA are essential to consider ANCA testing positive for purposes of diagnosing vasculitis
these illicit drugs have been associated with cutaneous vasculitis and/or arterial vasospasm,
cocaine and methamphetamines
- 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
Microscopic polyangiitis (MPA)
Wegener granulomatosis is also known as
Granulomatosis with polyangiitis
Churg-Strauss Syndrome is also known as
Eosinophilic granulomatosis with polyangiitis
- 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
Granulomatosis with polyangiitis (Wegener Syndrome)
- 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.
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome)
Cutaneous disease is more common in eosinophilic granulomatosis with polyangiitis than in systemic granulomatous vasculitis or microscopic polyangiitis.
The most common presentation of** severe vasculitis in eosinophilic granulomatosis with polyangiitis**
acute peripheral neuropathy with involvement of the heart, GI tract, brain, and eyes less commonly
histologic term defined by the absence of evidence of systemic disease
Cutaneous leukocytoclastic angiitis
medications that can cause drug-induced ANCA-associated vasculitis
propylthiouracil and hydralazine
- 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,
POLYARTERITIS NODOSA
The most common cutaneous features of polyarteritis nodosa
livedo reticularis/racemosa, painful cutaneous nodules or ulcers and digital ischemia
These manifestations reflect vasculitis of “medium-sized” arteries in the subcutis
livedo reticularis/racemosa, painful cutaneous nodules or ulcers and digital ischemia
- 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
Giant cell arteritis (temporal arteritis)
present in 30% to 40% of cases and is** the only cutaneous manifestation of giant cell arteritis apparent on examination**
Palpable nodularity of the temporal artery
Takayasu arteritis is a rare form of vasculitis (prevalence <1:100,000) involving what blood vessel?
aorta and its major branches.
The typical presentation of Takayasu
limb claudication.
ancillary to diagnose Takayasu arteritis
Angiography
2 phases of treatment of vasculitis
**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)
medications that have roles in remission induction for ANCA-associated vasculitis
cyclophosphamide, rituximab, and methotrexate
when is watchful waiting a reasonable approach in patients with vasculitis?
(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.
- 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
Glucocorticoids
non-cyclophosphamide drugs that can be given for remission maintenance of vasculitis
methotrexate, azathioprine, mycophenolate, cyclosporine
This drug has now been demonstrated to have efficacy in the treatment of mucocutaneous manifestations of Behçet disease
Apremilast
biologics that can be given in treating vasculitis
- 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
This monoclonal antibody to** anti-interleukin 5,** has been demonstrated to have efficacy in the treatment of eosinophilic granulomatosis with polyangiitis
Mepolizumab
monoclonal antibody to interleukin 6, for the treatment of giant cell arteritis
tocilizumab
CTLA-4 immunoglobin, to treat giant cell arteritis
abatacept
Medical Emergencies Related to the Primary Vasculitides
