Chapter 139 - Systemic Necrotizing Arteritis Flashcards by Beatrice Maxine Chan

Matching type

Henoch Schonlein purpura
Kawasaki disease
Giant cell arteritis
Behcet
Polyarteritis nodosa
Churg Strauss Syndrome
Takayasu arteritis
Microscopic polyangiitis

A. Small vessel
B. Medium vessel
C. Large vessel
D. None of the above

Small vessel: HSP, Cryoglobulinemia, MPA, CSS, GPA

Medium vessel: Kawasaki, PAN

Large vessel: GCA, TAK, isolated aortitis

No predominant size: Behcet, primary CNS, Relapsing polychondritis, Cogan

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Vasculitis becomes palpable when it reaches ___mm in diameter

2mm

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It is generally recommended biopsy a skin lesion that has been clinically apparent for less than ____ hours

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True or False

Normal liver function tests do NOT rule out infectious hepatitis.

True

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The most common cutaneous features of polyarteritis nodosa

Livedo reticularis/racemosa

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All patients with cutaneous vasculitis need to be evaluated for systemic vasculitis.
True or False

True

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___ is nearly always essential to establish a diagnosis of vasculitis and determine types of vasculitis.

Skin biopsy

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Mimics of idiopathic vasculitis

Infection
Malignancy
Thrombosis
Embolic disease

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Essential aspect of management

Comprehensive clinical follow up of all patients

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Major pathologic process of vasculitis

Inflammation of the blood vessels

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Skin disease is a frequent manifestation in many forms of vasculitis, esp. ____ arteritis

Small and medium vessel arteritis

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The most widely used criteria for vasculitis are

American College of Rheumatology classification criteria

Chapel Hill Consensus Conference disease definitions

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More readily pronounced in medium vessel vasculitis than in small vessel vasculitis

Ulceration

Digit ischemia

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Classic presentation of small vasculitis

Palpable purpura

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2 pathways of small vessel vasculitis

Immune complex deposition

2. Non-immune complex mediated

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ANCA directed against the neutrophil proteins

Myeloperoxidase

2. Proteinase -3

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Large vessel vasculitides are mediated by T cells activating ___ in walls of large arteries

Macrophages

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In giant cell arteritis, both __ &; ___ are prominent.

IL17 secreting TH17

IFN gamma secreting TH1

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Presents with jaw claudication &;shoulder, hip girdle pain

Giant cell arteritis

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Presents with angina

Takayasu arteritis

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Similarities of Giant cell arteritis and Takayasu arteritis (5)

Amaurosis fugax
Congestive heart failure (aortic valve insufficiency)
Limb claudication
Headaches/ scalp tenderness
Lightheadedness/ Syncope

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Gross hematuria due to renal infarction is often seen in

Polyarteritis nodosa

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Presents with asthma, nasal polyps, &;muscle weakness

Churg Strauss syndrome

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Presents with proptosis and gingivitis

Granulomatous polyangiitis

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Similarities of GPA and EGPA (8)

``` Tearing Ear pain and fullness Epistaxis, nasal crusting, and discharge, anosmia Facial pain/ tooth pain Pulmonary nodules —> hemoptysis Bronchitis/ large airway collapse —> dyspnea, cough Myocarditis Superficial granulomas ```

All small vessel vasculitis presents with (6)

``` Sensorineural hearing loss Pulmonary emboli Glomerulo nephritis Sensory/motor dysfunction Purpura Peripheral edema ```

Presentation of medium to large artery stenosis

Digital ischemia/gangrene

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

Full ROS

Skin lesions, always purpura, are the most common manifestation of drug-induced vasculitis. True or False

False, usually purpura

Normal phyical examination r/o pathology. | True or False

False, do not | Eg. Normal BP —> severe glomerulonephritis

Helpful measures to screen for large vessel vasculitis: (3)

Absent pulses Asymmetric blood pressure Bruits

Typical features of acute neutrophilic vasculitis

``` Fibrinoid necrosis Extravasation of RBCs Extravasation of neutrophils Leukocytoclasia (nuclear debris) Immune deposits ```

Extravascular granulomas with geographic necrosis is strongly suggestive of

Granulomatosis polyangiitis

Predominance of IgA over IgG is diagnostic of IgA vasculitis. True or False

False, suggestive only

Most important to order in patient suspected of having vasculitis

Tests for renal disease Urinalysis Serum creatinine

__ vessel vasculitis is expected to produce hematuria, accompanied by RBC casts, and proteinuria; on the other hand, ___ vessel vasculitis is expected to produce isolated hematuria only

Small; medium

Normal liver function tests cannot rule out infectious hepatitis. True or False

True

___ should be ordered on all patients suspected of having vasculitis

CBC

An elevated eosinophil count greater than ___ cells/ul is found in most untreated patients with Churg Strauss

1000

Levels of ESR/CRP do not correlate with severity of disease. | True or False

True May have normal values during active vasculitis May remain in remission despite elevation of markers

Approximately __% of patients with MPA | __% of patients with GPA, and __% of patients with EGPA will test positive for ANCA

90% 75% 40%

ANA is extremely specific for SLE (> 95%). | True or False

False, sensitive

Only __ % of Sjogren syndrome patients tests positive for RF, anti-Ro, anti-La.

80%

At least __% of RA tests positive for RF; and at least __% of rheumatoid vasculitis

70% | 95%

In cryoglobulinemic vasculitis, __ levels are usually depleted; and ___ less depleted or even normal

C4; C3

Low complement helps to raise suspicion for lupus but not specific for vasculitis in SLE. True or False

True

Drugs of abuse associated with cutaneous vasculits and/or arterial vasospasm

Cocaine | Amphetamines

Levamisole can lead to characteristic gangrenous lesions as well as positive tests for the ff antibodies

Anti-PR3 | Anti-MPO

Appropriate screening test for vasculitis

Chest Xray

For patient with vasculitis and pulmonary symptoms, __ is usually indicated

CT scan

For small vessel vasculitis, a screening __ is indicated for staging

CT scan

Sinus involvement is extremely common in

EGPA | GPA

Nasal inflammation is better assessed with a CT scan. | True or False

False, by PE

Central role in the diagnosis and management of large and medium vessel vasculitis

Angiography

Nerve conduction testing is recommended for screening asymptomatic patients and to distinguish between compressive and noncompressive neuropathy True or False

False, not recommended

__ is a commonly missed manifestation of small vessel vasculitis

Hearing loss

Baseline __ is recommended for all patients with established diagnosis of ANCA-associated vasculitis

Audiogram

Medical emergency of rapid onset cardiomyopathy is related to

EGPA

Angina or myocardial infarction is an expected emergency in

Large vessel vasculitis

Pulmonary embolus is an expected medical emergency of

Small vessel vasculitis