Chapter 139 - Systemic Necrotizing Arteritis Flashcards
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
Vasculitis becomes palpable when it reaches ___mm in diameter
2mm
It is generally recommended biopsy a skin lesion that has been clinically apparent for less than ____ hours
48
True or False
Normal liver function tests do NOT rule out infectious hepatitis.
True
The most common cutaneous features of polyarteritis nodosa
Livedo reticularis/racemosa
All patients with cutaneous vasculitis need to be evaluated for systemic vasculitis.
True or False
True
___ is nearly always essential to establish a diagnosis of vasculitis and determine types of vasculitis.
Skin biopsy
Mimics of idiopathic vasculitis
Infection
Malignancy
Thrombosis
Embolic disease
Essential aspect of management
Comprehensive clinical follow up of all patients
Major pathologic process of vasculitis
Inflammation of the blood vessels
Skin disease is a frequent manifestation in many forms of vasculitis, esp. ____ arteritis
Small and medium vessel arteritis
The most widely used criteria for vasculitis are
American College of Rheumatology classification criteria
Chapel Hill Consensus Conference disease definitions
More readily pronounced in medium vessel vasculitis than in small vessel vasculitis
Ulceration
Digit ischemia
Classic presentation of small vasculitis
Palpable purpura
2 pathways of small vessel vasculitis
- Immune complex deposition
2. Non-immune complex mediated
ANCA directed against the neutrophil proteins
- Myeloperoxidase
2. Proteinase -3
Large vessel vasculitides are mediated by T cells activating ___ in walls of large arteries
Macrophages
In giant cell arteritis, both __ &; ___ are prominent.
IL17 secreting TH17
IFN gamma secreting TH1
Presents with jaw claudication &;shoulder, hip girdle pain
Giant cell arteritis
Presents with angina
Takayasu arteritis
Similarities of Giant cell arteritis and Takayasu arteritis (5)
Amaurosis fugax Congestive heart failure (aortic valve insufficiency) Limb claudication Headaches/ scalp tenderness Lightheadedness/ Syncope
Gross hematuria due to renal infarction is often seen in
Polyarteritis nodosa
Presents with asthma, nasal polyps, &;muscle weakness
Churg Strauss syndrome
Presents with proptosis and gingivitis
Granulomatous polyangiitis
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