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Source: www.scielo.br |
Definition
Vasculitis is an inflammation of vessels, characterized by infiltration
of the vessel wall with leukocytes due to an unknown mechanism. This
infiltration of the wall leads to the destruction of the wall, with complications
such as bleeding and stenosis with an ischemic disease as a result.
Vessels that are affected differently in size and location according to the
underlying vasculitis type.
Vasculitis can be primary or secondary to another condition.
Clinical manifestation
There are no specific or sensitive diagnostic symptoms.
However, the presence of systemic symptoms with single/multiple organ dysfunction,
should lead to thinking about vasculitis.
:Some symptoms
- Fatigue
- Abdominal pain
- Arthralgia
- Fever
- Arterial hypertension
- Renal insufficiency
- Neurological symptoms
- Purpura
: suggestive symptoms of some vasculitis
Mononeuritis multiplex is suggestive for polyarteritis nodosa
Palpable purpura is suggestive for cutaneous vasculitis of
hypersensitivity typ. Purpura with another organ dysfunction is suggestive for
IgA vasculitis or microscopic polyangiitis
Pulmonary with renal involvement is suggestive for wegner’s vasculitis
or anti-GBM antibodies.
Diagnosis
: History
Important aspects of history:
Medicine: some medication cause hypersensitivity vasculitis
Disease history: hepatitis c: may cause mixed cryoglobulinemia and some
cases of polyarteritis
Systemic lupus erythematosus SLE: secondary vasculitis
Age of patients: age-specific distribution of different vasculitides
Physical examination
Palpable purpura?
Mononeuritis multiplex?
Laboratory tests
- ANA: may suggest connective tissue disorder such SLE
- Complement level: may be low in mixed cryoglobulinemia und SEL BUT not in most other vasculitides
- ANCA:
- cANCA (against protease 3): strongly suggests a diagnosis of granulomatosis with polyangiitis (Wegener’s)
- pANCA (against myeloperoxidase): suggests a diagnosis of microscopic polyangiitis
Nonspecific tests: to asset the function/ association of other organs:
creatinine
Liver enzyme tests
Hepatitis serology
CK
ESR
Urine analysis
Blood/Tissue culture
Electromyography:
by neuromuscular symptoms and suspected systemic
vasculitis
Tissue biopsy:
essential for establishing the diagnosis.
Angiography:
helps by vasculitis of large/medium vessels, less sensitive are
Angio-CT/ Angio-MRT/ Ultrasound
Classification of vasculitis
- Large vessel vasculitis
- Medium vessel vasculitis
- Small vessel vasculitis
- Secondary vasculitis:
- Viral infection
- Connective tissue disorders
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Source: www.sciencedirect.com |
ACR Criteria (American college of rheumatology): These criteria try to differ diagnosed vasculitis from each other: the diagnosis is always established histologic:
Large vessel vasculitis
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Takayasu arteritis
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Age of 40 years or younger
at disease onset
Claudication of the
extremities
Decreased pulsation of one
or both brachial arteries
Difference of at least 10
mm Hg in systolic blood pressure between arms
Bruit over one or both
subclavian arteries or the abdominal aorta
Arteriographic narrowing
or occlusion of the entire aorta, its primary branches, or large arteries in
the upper or lower extremities that is not due to arteriosclerosis,
fibromuscular dysplasia, or other causes
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ACR Criteria (American
college of rheumatology):
The presence of any 3 or
more criteria yield a sensitivity of 90.5% and a specificity of 97.8%
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Giant cell arteritis
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Age ≥50 years at time of
disease onset
Localized headache of new
onset
Tenderness or decreased
pulse of the temporal artery
Erythrocyte sedimentation
rate (ESR) greater than 50 mm/h (Westergren)
Biopsy that includes an
artery and reveals a necrotizing arteritis with a predominance of mononuclear
cells or a granulomatous process with multinucleated giant cells
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ACR criteria
The presence of 3 or more
criteria yields a diagnostic sensitivity of 93.5% and specificity of 91.2%.
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Medium vessel vasculitis
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Polyarteritis nodosa
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Otherwise unexplained
weight loss >4 kg
Livedo reticularis
Testicular pain or
tenderness
Myalgias (excluding that
of the shoulder and hip girdle), weakness, or polyneuropathy
Mononeuropathy or
polyneuropathy
New onset diastolic blood
pressure >90 mmHg
Elevated levels of serum
blood urea nitrogen (>40 mg/dL or 14.3 mmol/L) or
creatinine (>1.5 mg/dL or 132 mcmol/L)
Evidence of hepatitis B
virus infection via serum antibody or antigen serology
Characteristic
arteriographic abnormalities not resulting from noninflammatory disease
processes
A biopsy of medium- or
small-sized artery containing polymorphonuclear cells
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ACR criteria
The presence of 3 or more
criteria yields a diagnostic sensitivity of 82% and specificity of 87%.
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Kawasaki disease
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Diagnosis requires the
presence of fever lasting five days or more without any other explanation,
combined with at least four of the five following physical findings:
Bilateral conjunctival
injection
Oral mucous membrane
changes, including injected or fissured lips, injected pharynx, or strawberry
tongue
Peripheral extremity
changes, including erythema of palms or soles or edema of hands or feet
(acute phase), as well as periungual desquamation (convalescent phase).
Polymorphous rash
Cervical lymphadenopathy
(at least one lymph node >1.5 cm in diameter)
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Criteria: Established by
Tomisaku Kawasaki in 1967
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Primary CNS vasculitis
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Rare case, in patient with
CNS symptoms and signs in combination with evidence of a cerebral vasculitis
by angiography and leptomeningeal biopsy, no established criteria
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Small vessel vasculitis
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Eosinophilic
granulomatosis with polyangiitis (Churg-Strauss)
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Asthma (a history of
wheezing or the finding of diffuse high pitched wheezes on expiration)
Eosinophilia of >10
percent on differential white blood cell count
Mononeuropathy (including
multiplex) or polyneuropathy
Migratory or transient
pulmonary opacities detected radiographically
Paranasal sinus
abnormality
Biopsy containing a blood
vessel showing the accumulation of eosinophils in extravascular areas
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The presence of four or
more of these criteria yields a sensitivity of 85 % and a specificity of 99.7
%
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Granulomatosis with
polyangiitis (Wegener’s)
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Nasal or oral inflammation
(painful or painless oral ulcers or purulent or bloody nasal discharge)
Abnormal chest radiograph
showing nodules, fixed infiltrates, or cavities
Abnormal urinary sediment
(microscopic hematuria or red cell casts)
Granulomatous inflammation
on biopsy of an artery or perivascular area
NOTE: these criteria was
made before ANCA discovery
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he presence of two or more
of these four criteria yielded a sensitivity of 88 percent and a specificity
of 92 percent
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Hypersensitivity
vasculitis
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Age >16
Use of a possible
offending drug in temporal relation to the symptoms
Palpable purpura.
Maculopapular rash
Biopsy of a skin lesion
showing neutrophils around an arteriole or venule
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The presence of three or
more of these criteria had a sensitivity of 71 % and specificity of 84 %.
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Henoch-Schönlein purpura
(IgA vasculitis) HSP
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Palpable purpura
Bowel angina
Gastrointestinal bleeding
Hematuria
Age at onset ≤20 years
lesion
No new medications
Note: the same as above,
with IgA deposition on skin and kidney
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The presence of three or
more of the six criteria yielded a correct classification of HSP in 87
percent
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Cryoglobulinemic
vasculitis
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No criteria established;
Diagnosis usually made by:
History
skin purpura
low complement levels
circulating cryoglobulins
histology showing small
vessel inflammation with immune deposits
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Differential diagnosis
- most common is systemic rheumatic diseases, such as systemic lupus erythematosus.
- Fibromuscular dysplasia
- Bacteremia
- Amyloidosis
- Atherosclerosis
- Vasospasm
- Malignancies
- Ergotism
Therapy
Therapy depends upon the nature and severity of the vasculitis. Below is
the treatment concept in general, each typ of vasculitis should be treated due
to its guidelines.
Hypersensitivity vasculitis:
Discontinuing the offending drug may be adequate
antihistamine for itching
short course of corticosteroids for more severe cases.
simple observation may be adequate for transitory vasculitis confined to
the skin, as with mild cases of Henoch-Schönlein purpura (IgA vasculitis).
Systemic vasculitis:
glucocorticoid therapy.
patients with rapidly progressive vasculitic diseases, such as
granulomatosis with polyangiitis (Wegener’s) or polyarteritis nodosa, require
combination therapy consisting of a cytotoxic drug (usually cyclophosphamide) and
steroids for 1-2 months.
Then the steroid dose may be reduced; therapy is usually continued for 6
to 12.
Azathioprine and methotrexate have been used in less
severe forms of vasculitis and as maintenance therapy after remission has been
induced by cyclophosphamide.
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