Significant reduction of
hepatitis B virus infection has also reduced the frequency of PAN in the world
in parallel. In addition, some patients, previously classified as PAN, are now
classified as separate diagnoses (such as MPA).
The diagnosis is mostly
made at middle and advanced ages and the incidence increases with age. It makes
its peak in the 6th decade of life.
It's a little more in men
(1,5: 1).
Many cases are idiopathic,
the pathogenesis of some patients is associated with HBV, HCV and hairy cell
leukemia.
HBV-associated PAN usually
develops within 4 months after HBV.
Regardless
of the underlying cause, PAN is characterized by segmental transmural
inflammation of musculature arteries.
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PAN
does not hold venues.
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The
cellular infiltrate consists of polymorphonuclear leukocytes and mononuclear
cells.
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It
may be leukocytoclastosis
(fragmentation of white blood cells).
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Necrosis
of the arterial wall leads to a homogeneous eosinophilic appearance known as
fibrinoid necrosis.
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Damage
to the internal and external elastic lamina may cause aneurysmal dilatation.
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In
the same sample, lesions at various stages can be found.
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Granulomatous
inflammation leads to another diagnosis. Not on the PAN.
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PAN’lı hastalar tipik
olarak sistemik semptom ve bulgularla gelirler.
On the skin; Purpura, tender
erythematous nodules, ulcers, and bullous or vesicular eruptions may be
present.
Skin lesions are more
frequent in lower extremity and extremity edema is frequent. Skin lesions can
be serious enough to go to gangrene.
Kidney; The most commonly
affected organ in autopsy series. Renal insufficiency, hypertension, perirenal
hematomas (rupture of renal artery aneurysms), multiple renal infarcts may also
be present.
Incomplete narrowing of
the inflamed arteries does not cause inflammation or necrosis, although it
causes glomerular ischaemia. Therefore, urine examination shows sub-nephrotic
proteinuria, often with minimal proteinuria and possibly moderate hematuria. However,
erythrocytic eruptions, which are representative of glomerular inflammation,
are not usually seen. SLE or ANCA (+) vasculitis should be considered if seen. As
a result of renal ischemia, RAAS activation is considered as the cause of
hypertension.
Neurological; 70% of
patients have a mononeuritis multiplex. Over time, the number of affected
nerves increases, so the first asymmetric involvement may return to distal
symmetric polyneuropathy.
SSS involvement occurs in
5-10% patients.
GIS; The involvement of
the mesenteric artery may be recognized early by abdominal pain. Because the
patient is afraid of eating or because of malabsorption, weight loss may occur.
Nausea-vomiting, melena,
bloody / bloodless diarrhea and GI bleeding can also be seen.
Intestinal infarction and
perforation may also develop.
Splenic infarct
Patients with PAN have
increased risk of perforation during colonoscopy.
Although AMI is not a very
common occurrence, PAN can cause coronary ischemia.
Rarely, cholecystitis,
appendicitis, segmental pancreatic infarction can occur.
Myalgia and muscle
weakness are frequently observed.
Orchitis (10%)
Eye findings; Ischemic
retinopathy, retinal detachment, optic neuropathy
Breast and uterine
involvement
Bronchial artery
involvement has been described in PAN, but other diseases should be considered
if there are other parenchymal involvements due to capilleritis or vasculitis.
Diagnosis
PAN diagnosis is
considered by the patient's clinic and radiological imaging, but is confirmed
with biopsies from the affected organs.
The renal biopsy to be
performed in the PAN can give us the pathognomonic inflammation in the
middle-sized arteries. Because of the multiplicity of microaneurysms, there may
sometimes be an increased risk of bleeding. Some advocate renal biopsy only if
arteriography is negative.
Conventional mesenteric or
renal arteriography is an alternative to biopsy.
Less invasive techniques
such as CT and MRI can be used.
The
PAN classification of ACR in 1990 can determine the disease with 82%
sensitivity and 87% specificity.
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≥ 4kg weight
loss without any other explanation
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Livedo
reticularis
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Testicular pain
or tenderness
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Miyaji (except
shoulder and hip zone), muscle weakness, limb tenderness or polyneuropathy
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Mononeuropathy
or polyneuropathy
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Diastolic blood
pressure greater than 90 mmHg (new onset)
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Elevation of
serum BUN (> 40 mg / dL) or creatinine (> 1,5 mg / dL) levels.
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Evidence of HBV
infection
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Characteristic
angiographic abnormalities (other than those resulting in noninflammatory
disease processes)
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