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VASCULITE I
VASCULOPATII
Schonlein
n 1852 Rokitansky descrie
trsturile clinice
caracteristice ale poliarteritei
nodoase.
Karel Cercetri aprofundate n
Rokitanski y aceast maladie le aparin lui
Kussmaul i Maier n 1966.
Marea majoritate a
vasculitelor au fost
identificate ncepnd cu
secolul XX.
Adolf Kussmaul (1822-1902)
1908 Takayasu descrie
modificrile
ischemice, rezultat al
arteritei care-i poart
numele.
Mikito Takayasu
Descrierile vasculitelor
continu cu
granulomatoza Wegener,
boala Behcet, sindromul
Churg Strauss (1951),
vasculitele prin
hipersensibilizare (1984),
Epidemiologie
Datele epidemiologice referitor
la vasculite nu snt numeroase.
Se estimeaz orientativ o
inciden a vasculitelor
sistemice n general ntre 0,4 i
14 cazuri la 100 000 populaie.
Arterita Takayasu
Arterit frecvent granulomtoas, ce afecteaz predominant
aorta i / sau ramurile ei majore. Debuteaz de regul la
pacieni mai tineri de 50 de ani.
Arterita cu celule
gigante Arterit frecvent granulomatoas, ce afecteaz de regul
aorta i /sau ramurile ei principale, cu predilecie pentru
ramurile arterelor carotide i celor vertebrale. Frecvent
implic arterele temporale. Debuteaz de obicei la pacienii
cu vrsta peste 50 ani i frecvent este asociat cu
polimialgia reumatic.
(2)
Vasculite Vasculitele ce afecteaz predominant
arterele de calibru mediu, definite ca
ale vaselor principalele artere viscerale, i ramificrile
medii lor. Pot fi afectate i artere de orice alte
dimensiuni. Sunt comune aneurismele
inflamatorii i stenozele.
Poliarterita Arterit necrotizant a vaselor medii sau de calibru mic, fr
glomerulonefrit sau vasculit la nivel de arteriole, capilare sau
nodoas venule, i neasociat cu anticorpi ctre citoplasma neutrofilic
(ANCA).
- formarea de
autoanticorpi cum ar fi :
Ac. anticelul endotelial
i ANCA (Ac ctre
citoplasma neutrofilic
i/sau lezarea celulelor
endoteliale mediat de
neutrofile )
- lezarea celulelor
endoteliale mediat de
limfocitele T
Rspuns imun celular i
molecular , inclusiv
secreia de citokine i a
moleculelor de adghezie.
Prezentarea clinic
Cele mai frecvente simptome
constituionale snt fatigabilitatea,
slbiciunea general, febr i
atralgiile
Metodele de diagnostic
Cteva semneal vasculitelor
clinice sugereaz
sistemice prezena vasculitei
Purpura sau alt gen de erupii
Leziuni cutanate ce nu dispar la
digitopresiune, din cauza hemoragiilor
intracutanate.
Multiplexul mononeuritic
Se ntlnete cnd snt lezai doi sau
mai muli nervi din pri separate ale
corpului
Implicarea pulmonar
Hemoragia alveolar din
capilarit poate cauza hemoptizie.
Hemoptizia poate deasemenea fi
asociat cu o vasculit de calibru
mediu din cauza rupturii unui
aneurism a unei arterii bronice.
Semne clinice n vasculite
Calibru mare Deficit de puls
Sufluri
Calibru mediu Noduli cutanai
Livedo reticularis
Infarct digital
Calibru mic Purpur palpabil
Ulceraii superficiale
Multiplex mononeuritic
Leziuni papulonecrotice
Datele de laborator:
Favorizeaz elaborarea
diagnosticului pozitiv i diferenial,
aprecierea activitii vasculitelor,
eficiena tratamentului.
Analiza general a sngelui poate
evidenia: anemie, trombocitopenie,
leucocitoz, creterea VSH-lui,
eozinofilie (Churg-Strauss).
Histologic AT
Este o arterita granulomatoasa sclerozanta.
Intima prezinta striuri longitudinale, datorate
contraciei fibrotice a tunicii medii i externe,
cu proliferarea in placa a celei interne.
Infiltratul inflamator celular este compus din
macrofage i mai ales din limfocite T
citotoxice, care ajung in aorta prin vasa
vasorum aortice, recunosc proteinele de oc
termic exprimate in cantitate mare de peretele
vascular i secret perforina. In vecinatatea
mediei, sunt prezente uneori celule gigante
multinucleate, care au fagocitat elastina.
Tardiv apar fibroza, tromboze i calcifieri, cu
stenozarea lumenului arterial i dilataii
anevrismale.
Photomicrograph of the
aorta from a patient with
Takayasu's arteritis
demonstrates marked
thickening of the intimal
layer and inflammatory
infiltrates in the media
and laminar necrosis
Tabloul clinic
Majoritatea simptomelor AT aparin
sindromului de insuficien circulatorie
la nivelul trunchiurilor arteriale
afectate. Uneori, se realizeaza
fenomene de furt vascular, cu ischemia
unor regiuni proximale.
Simptomatologia depinde de
etapa evolutiva a afeciunii.
In faza preocluziva iniiala, care
dureaza luni sau ani, apar
manifestari constituionale i
musculo-scheletale nespecifice
uneori serozite (pleurezie,
pericardita), manifestari cutanate
(eritem nodos, ulcere gambiere) i
oculare (episclerita, irita etc).
Faza ocluziva se caracterizeaza
prin claudicaie, diminuarea
amplitudinii pulsului la diferite
sedii i o diferen a presiunii
arteriale sistolice de peste 10
mmHg intre brae, sufluri arteriale
sau aortice, hipertensiune
arteriala renovasculara,
insuficien cardiaca congestiva,
tulburari de vedere.
Distribuia macroscopica a leziunilor a
condus la clasificarea AT in patru tipuri, funcie
de sediul leziunilor:
arcul aortic i ramurile sale (tipul I),
aorta toracica, descendenta i
abdominala (tipul II),
teritoriile tipurilor precedente,
impreuna (tipul III) i
arterele pulmonare (tipul IV).
In tipul I (ascendent) cea mai comuna
anomalie
este suflul carotidian.
Claudica.ia i diminuarea pulsaiilor arteriale sunt mai
frecvente la membrele superioare decat la cele inferioare.
In sindromul arcului aortic primele manifestari sunt
cele neurosenzoriale: de la cefalee, vertij, hipoacuzie,
simptome oculare (claudica.ia vizuala a lui Frovig
amauroza i scotoame posturale sau de efort) pana la
cataracta i glaucom. Acestea pot fi insoite de manifestari
neurologice de focar (hemianopsie, paralizia privirii
conjugate superioare, afazie, mono- sau hemipareza,
convulsii etc).
Ischemia in teritoriul carotidian determina claudicaie
maseterina sau brahiala, hipotrofie musculara,
sindrom Raynaud i ischemia extremitailor. Pulsul
arterial este diminuat sau disparut (boala fara puls)
la toate sediile cefalice i brahiale,
brahiale iar manifestarile
cardiace sunt frecvente.
Tipul II (descendent )
corespunde coarctaiei aortice
inversata sau atipica. Simptomele
sunt minore, datorita circulaiei
colaterale bune. Pulsaiile arteriale
ale membrelor inferioare sunt
reduse, cu discrepana tensionala
intre membrele superioare i
inferioare, exista sufluri aortice
abdominale, claudicaie viscerala,
sindrom Leriche i hipertensiune
arteriala fixa, reno-vasculara.
Tipul III, mixt sau
extensiv , cel mai frecvent, reunete
afectarea arcului aortic i a aortei
abdominale, ceea ce realizeaza
manifestari mixte.
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t
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Cranial Nerve Palsy . The Internet Journal of Rheumatology.
2005 Volume 2 Number 1
Keywords: polyarteritis nodosa | vasculitis | juvenile | cranial
nerves
Table of Contents
y
Introduction
Case Report
Discussion
Abstract
t
Polyartheritis Nodosa PAN is not common in adolescents. Here,
we report a case of a 13 year old girl with PAN who presented
with recurrent abdominal pains followed by cutaneous and
peripheral nerves involvements during three years with a
relapsing-remitting course. This is a classic case of PAN in an
h
adolescent who diagnosed late in the course of her disease
because its monosymptomatic and unusual presentation. Also
there are some reports about cranial nerves involvements with
separate mechanism. In our case oculomotor and
vestibuloacoustic nerves were seen in the absence of signs of
e
brainstem disease.
Introduction
Kussmaul and Maier first described polyarteritis nodosa (PAN)
in 1866 after observing areas of focal inflammatory exudations
m
that gave rise to palpable nodules in a patient with advanced
disease (1 ). PAN, like other vasculitides, is generally
multisystem and most commonly affects skin, joints, peripheral
nerves, the gut, and the kidney vasculature. In 1948, Davson et al
described a microscopic PAN characterized by a diffuse
a
necrotizing glomerulonephritis with similar clinical features to
classic PAN but with the additional manifestations of small
vessel involvement resulting in rapidly progressive
glomerulonephritis (RPGN) and pulmonary capillaritis (2 ).
Polyarteritis nodosa (PAN) is a vasculitis of medium-sized or
t
small vessels. The vasculitis of PAN has a predilection for
medium-sized arteries of the skin, peripheral nerves, the gut, and
the kidney vasculature. PAN is predominantly observed in
patients aged 40-60 years, but can occur in all age groups.
When PAN presents in an atypical location and atypical age
o
group, the diagnosis becomes less straightforward.
PAN is not common in adolescents. Here, we report a case of a
13 year old girl with PAN who presented with recurrent
abdominal pains followed by cutaneous and peripheral nerves
involvements during three years with a relapsing-remitting
u
course. This is a classic case of PAN in an adolescent who
diagnosed late in the course of her disease because its
monosymptomatic and unusual presentation.
Case Report
In November 2004, a 15-year old girl was admitted to our hospital
s
because of foot drop and abdominal pain.
The patient had been well until three years before admission,
when recurrent abdominal pains developed. The location of
abdominal pains was periumbelical and there was not
accompanying nausea and/or womiting, night sweats,
n
intermittent diarrhea and fever. Two years before admission, she
noted erythematous vesicles and papules over the both shins with
next central ulceration. During last year, she had high blood
pressures in her visits in the range of 140/90 to 160/110 mmHg.
Some laboratory and radiologic tests were done for evaluation of
o
abdominal pains and hypertention. Immunologic tests for
rheumatoid factor, antinuclear antibodies, anti dsDNA
antibodies, cANCA, anticadiolipin antibodies, C3, C4, CH50
levels and hepatitis B virus surface antigen and HCV Ab were
negative. Erythrocyte sedimentation rate was 8 mm/h and C-
d
reactive protein was 3+ and all other hematological and
biochemical tests were normal. Ultrasound examination of
abdomen and pelvis, upper GI series, endoscope study of
esophagus, stomach and duodenum showed no abnormality.
Abdominal and pelvis CT with contrast were normal. Gastric
u
biopsy samples showed chronic inflammatory gastritis.
Four month before admission she complained headache, vertig o
and diplopia. Third cranial nerve palsywith petosis was
diagnosed. Hearing loss in left ear was developed in next days.
Magnetic resonance imag ing (MRI) was normal. Audiometry
l
showed sensory hearing loss in left ear. Two month before, she
had an exacerbation of abdominal pain and erythematous nodules
on anterior surface of her legs. So she admitted in other hospital.
A skin-biopsy specimen disclosed leukocytoclastic vasculitis and
the patient were treated with oral prednisone at a dose of 40 mg
e
daily.
Five days before admission, numbness developed in the left toes
and progressed to foot drop.
On examination, the patient was cachectic (34 Kg) and had a
weight loss of about 8 kg during last month due to her anorexia.
s
There was patchy, brownish pigmentation, with depressed,
hyperpigmented areas, 2 to 5 cm in diameter, over both shins (fig
1). No peripheral edema was found. The temperature was
36.8C, the pulse was 86, and the respirations were 14. The
o
blood pressure was 150/90 mm Hg. The thyroid gland was not
enlarged and tender. The lungs were clear and cardiovascular
examination was normal. There was hypogasteric and
periumblical tenderness in abdominal palpation. There was no
organomegaly and joints were not swollen and tender. The urine
n
was neg ative for protein; the sediment contained 1 to 2 red cells,
2 to 3 white cells, and 0 to 2 hyaline, granular, and white-cell
casts and a few bacteria per high-power field. Echocardiography
was normal.
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