139 - Systemic Necrotizing Arteritis Flashcards by dvojnd rivera

Predominantly small vessel vasculitis

Immune complex mediated:

Cryoglobulinemia
HSP

Limited skin

ANCA-associated:

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Predominantly medium vessel

Kawasaki

PAN

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Predominantly large vessel

GCA
TAK
Isolated aortitis

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No predominant size

Behcet
Primary CNS
Relapsing polychondritis
Cogan

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Takayasu arteritis is substantially more common in

Women

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Kawasaki disease is almost exclusively a disease of

Young children

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Giant cell arteritis is limited to

Older adults

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Granulomatosis with polyangitis (Wegener) mostly occurs in

Whites

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Behcet disease is markedly more common in countries in the

Eastern Mediterranean
Japan
Korea

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

Hepatitis B virus

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Infection-related vasculitis: _____ in most cases of cryoglobulinemic vasculitis

Hepatitis C virus

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Small-vessel vasculitis follow one of 2 pathways:

Immune complex deposition

2. Non-immune complex mediated pathology that likely involves ANCA

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ANCA are directed against the neutrophil proteins

Myeloperoxidase (MPO)

Proteinase-3 (PR3)

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Large-vessel vasculitis are thought to be medicated by

T cells activating macrophages

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HLA locus in GCA

Class II

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HLA locus in Takayasu arteritis

Class I

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Single most important component of early evaluation of a patient suspected of having vasculitis

Full review of systems with assessment of overall severity of illness

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The diseases that cause cutaneous vasculitis can affect all organ systems, and in most cases that involvement will cause symptoms, _____ being a prominent exception

Renal disease

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

6 to 12 months

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It is generally recommended to biopsy a skin lesion that has been clinically apparent for

Less than 48 hours

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Histopathology: extravascular granulomas with geographic necrosis

Granulomatosis with polyangitis

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Histopathology: eosinophil-rich extravascular granulomas

Eosinophilic granulomatosis with polyangitis

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Y/N: Kidney or lung biopsies are less likely than skin biopsies to show pathology diagnostic of a particular disease

No - more likely

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

Renal disease

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

An elevated absolute eosinophil is found in most untreated patients with eosinophilic granulomatosis with polyangitis (Churg-Strauss), and a count greater than _____ helps differentiated this disease from asthma and atopy

1000 cells/microL

Y/N: The levels of ESR and CRP do not correlate well with stage or severity of disease

Yes

Autoimmune serologies: ANCA-associated vasculitis and lupus can present with vasculitis of the skin, but _____ does not

Anti-glomerular basement membrane (GBM) disease

90% of patients with _____ will test positive for ANCA

Microscopic polyangiitis

75% of patients with _____ will test positive for ANCA

Granulomatosis with polyangiitis

40% of patients with _____ will test positive for ANCA

Eosinophilic granulomatosis with polyangiitis

Pauci-immune glomerulonephritis, pulmonary hemorrhage, peripheral and cranial neuropathy, musculoskeletal, constitutional symptoms Most patients are positive for ANCA, usually with specificity for antibodies to myeloperoxidase

Microscopic polyangiitis

Encompasses all 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, middle ear), cavitary pulmonary nodules, orbital pseudotumor, subglottic stenosis, vasculitis of the eye Neutrophilic and granulomatous dermatitis with papules (particularly on the extensor surfaces of the elbows), subctaneous nodules and ulcers Majority ANCA type is c-ANCA/anti-PR3

Granulomatosis with polyangiitis

Unique features: history of asthma (often severe or poorly controlled), blood eosinophilia Nasal polyps, constitutional symptoms, rashes, all typical of atopy Pulmonary infiltrates on chest imaging (eosinophilic pneumonia)

Eosinophilic granulomatosis with polyangiitis

Most common presentation of severe vasculitis in eosinophilic granulomatosis with polyangiitis

Acute peripheral neuropathy with involvement of the heart, GI tract, brain, eyes

Cutaneous disease is more common in (MPA, GPA, EGPA)

Eosinophilic granulomatosis with polyangiitis

Histologic term defined by the absence of evidence of systemic disease

Cutaneous leukocytoclastic angiitis

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

Cutaneous leukocytoclastic angiitis: _____% of cases are associated with drug exposure

Skin disease, myalgia, hypertension (from renal artery involvement), abdominal pain, neuropathy and/or testicular pain

"Classic" polyarteritis nodosa

Most common cutaneous features of polyarteritis nodosa

Livedo reticularis/racemosa

Disease of adults older than 50 years Mostly a disease of people of Northern European ancestry Cranial arteritis is a common feature, polymyalgia rheumatica, fever, malaise, weight loss

Giant cell arteritis

Diagnosis of GCA is usually confirmed by

Temporal artery biopsy

Only cutaneous manifestation of GCA apparent on examination | Present in 30% to 40% of cases

Palpable nodularity of the temporal artery

Rare form of vasculitis involving the aorta and its major branches Many patients are diagnosed as young adults, 90% are female

Takayasu arteritis

Typical presentation of Takayasu arteritis

Limb claudication

Diagnosis of Takayasu arteritis is made by

Angiography

Mortality in the first 3 to 6 months of severe vasculitis affecting vital organs is around

10%

Other than advanced age, the predictors of higher risk of mortality reflect prior organ damage: _____ damage in the case of granulomatosis with polyangiits and microscopic polyangiitis, and _____ damage in the case of eosinophilic granulomatosis with polyangiitis

Kidney | Cardiac

For vasculitides expected to have extended courses and/or to include severe manifestations, the general approach is to plan for 2 phases of treatment:

Remission induction | Remission maintenance

Mainstay of therapy for vasculitis

Glucocorticoids

Proven effective for ANCA-associated vasculitides and, to a lesser extent, other forms of vasculitis

Cyclophosphamide

Serious toxicities of cyclophosphamide which are related to total cumulative dose

Female and male infertility | Bladder cancer

Apremilast has now been demonstrated to have efficacy in the treatment of

Mucocutaneous manifestations of Behcet disease

Biologic demonstrated to be as effective as cyclophosphamide for induction of remission of AAV

Rituximab

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

Mepolizumab

Monoclonal antibody to interleukin-6 | Demonstrated efficacy for the treatment of giant cell arteritis

Tocilizumab

CTLA-4 immunoglobulin | Promising data on its use for giant cell arteritis

Abatacept