Echocardiographic screening of valvular heart disease in granulomatosis with polyangiitis: do we need it?
Abstract
Systemic vasculitides are rare autoimmune-mediated disorders affecting various organs. Their clinical presentation is usually heterogeneous, with a long and difficult period until a diagnosis is finally established. They are classified according to the type of affected vessels, pathogenesis, demographics and clinical characteristics. Granulomatosis with polyangiitis (GPA), formerly Wegener’s granulomatosis, is included in the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides and is characterized by necrotizing vasculitis of small and medium-sized vessels. Ear, nose and throat, lungs, and kidneys are mainly affected, and the disease occurs more frequently in patients in the fifth decade of life.
Full text
Re is a
Po uguesa
de
Ca diologia
43
(2024)
105---106
www. e po ca diol.o g
Re is a
Po uguesa
de
Ca diologia
Po uguese
Jou nal
o
Ca diology
EDITORIAL
COMMENT
Echoca diog aphic
sc eening
o
al ula
hea
disease
in
g anuloma osis
wi h
polyangii is:
Do
we
need
i ?
Ras eio
ecoca diog áfico
de
al ulopa ia
na
g anuloma ose
com
poliangi e:
p ecisamos
dele?
Ca a ina
de
Sousaa,b,c
aDepa amen o
Co ac¸ão
e
Vasos,
Cen o
Hospi ala
Uni e si á io
Lisboa
No e,
Lisboa,
Po ugal
bCen o
Ca dio ascula
Uni e sidade
de
Lisboa
CCUL-RISE,
Faculdade
de
Medicina
Uni e sidade
de
Lisboa,
Lisboa,
Po ugal
cLusíadas
Knowledge
Cen e ,
Lisboa,
Po ugal
A ailable
online
23
Decembe
2023
Sys emic
asculi ides
a e
a e
au oimmune-media ed
dis-
o de s
a ec ing
a ious
o gans.
Thei
clinical
p esen a ion
is
usually
he e ogeneous,
wi h
a
long
and
di ficul
pe iod
un il
a
diagnosis
is
finally
es ablished.
They
a e
classified
acco ding
o
he
ype
o
a ec ed
essels,
pa hogenesis,
demog aphics
and
clinical
cha ac e is ics.1G anuloma osis
wi h
polyangii is
(GPA),
o me ly
Wegene ’s
g anuloma o-
sis,
is
included
in
he
an ineu ophil
cy oplasmic
an ibody
(ANCA)-associa ed
asculi ides
and
is
cha ac e ized
by
nec o izing
asculi is
o
small
and
medium-sized
essels.
Ea ,
nose
and
h oa ,
lungs,
and
kidneys
a e
mainly
a ec ed,
and
he
disease
occu s
mo e
equen ly
in
pa ien s
in
he
fi h
decade
o
li e.2
Ca dio ascula
mani es a ions
a e
a
common
cause
o
mo bidi y
and
mo ali y
among
pa ien s
wi h
sys emic
asculi is.
Long-s anding
ch onic
inflamma ion
leads
o
sig-
nifican
co ona y,
al ula
and/o
pe ica dial
disease
as
well
as
myoca di is
o
ao ic
in ol emen .
Among
pa ien s
wi h
GPA,
ca dio ascula
in ol emen
is
none heless
a e
(3.3%
in
a
coho
o
517
pa ien s
published
on
behal
o
he
Vas-
E-mail
add ess:
[email p o ec ed]
culi is
Clinical
Resea ch
Conso ium3)
and
he e ogeneous.
Pe ica di is
and
pe ica dial
e usion
a e
he
mos
equen
ca diac
complica ions
seen
in
GPA.4The
fi s
cases
o
ao ic
al ula
we e
desc ibed
in
he
la e
1980s.5Since
hen,
small
e ospec i e
coho
s udies
and
case
epo s
cons i u e
he
only
a ailable
e idence
ha
d i es
clinical
p ac ice.
As
in
all
a e
sys emic
diseases,
bu
especially
in
GPA,
obus
long- e m
p ospec i e
clinical
e idence
on
he
impac
on
he
ca dio ascula
sys em
is
lacking.
In
addi ion,
u he
e idence
is
needed
o
decide
on
he
pe iodici y
o
ca diac
clinical
su eillance.
In
he
cu en
issue
o
he
Jou nal,
Bo owiec
e
al.6
p esen
a
p ospec i e
obse a ional
single-cen e
s udy
on
pa ien s
ini ially
hospi alized
wi h
GPA
and
egula ly
ol-
lowed
in
a
uni e si y
hospi al
in
he
Medical
Uni e si y
o
Wa saw,
Poland,
be ween
Feb ua y
2010
and
No em-
be
2020.
This
is
a
case---con ol
s udy
o
105
pa ien s
analyzed
a
baseline
and
wi h
a
mean
ollow-up
o
up
o
6.2
yea s.
Va iables
analyzed
included
medical
his o y,
physical
examina ion,
labo a o y
s udies
and
echoca dio-
g aphic
examina ion
(mainly
wo-dimensional,
flow
and
issue
Dopple ).
h ps://doi.o g/10.1016/j. epc.2023.12.003
0870-2551/©
2023
Published
by
Else ie
Espa˜
na,
S.L.U.
on
behal
o
Sociedade
Po uguesa
de
Ca diologia.
This
is
an
open
access
a icle
unde
he
CC
BY-NC-ND
license
(h p://c ea i ecommons.o g/licenses/by-nc-nd/4.0/).
C.
de
Sousa
The
included
pa ien s
we e
mos ly
emale
(64%)
and
in
he
fi h
decade
o
li e.
The e
was
a
high
p e alence
o
ca -
dio ascula
isk
ac o s
in
his
popula ion,
wi h
hype ension
and
dyslipidemia
in
66%
and
70%
o
he
s udy
popula ion,
espec i ely.
Mos
pa ien s
we e
on
egula
glucoco icoids
(84%).
The
mos
equen
finding
a
baseline
echoca diog aphy
was
ao ic
egu gi a ion
(43%);
his
was
he
only
al ula
dys unc ion
ha
inc eased
significan ly
du ing
ollow-up,
o
51%
o
he
GPA
g oup.
O
hese
pa ien s,
only
wo
unde wen
ca diac
al e
su ge y.
O he
less
equen
ca diac
findings
we e
ao ic
s enosis
and
mi al
o
icuspid
egu gi a ion,
he
a es
o
which
we e
s able
in
ollow-up.
Ao ic
egu gi a ion
was
also
he
dominan
al ula
find-
ing
in
an
obse a ional
s udy
by
he
same
clinical
g oup,7
in
which
88
pa ien s
wi h
GPA
we e
compa ed
wi h
a
con-
ol
g oup
o
40
age-
and
gende -ma ched
pa ien s
(28%
s.
7.5%;
p=0.03).
Vege a ions,
leafle
hickening,
al ula
pe -
o a ion
and
endoca dial
masses
a e
he
main
mechanisms
in
hea
al e
lesions8desc ibed
in
ANCA-associa ed
sys emic
asculi is,
pa icula ly
GPA.
In
his
long- e m
p ospec i e
s udy6 he
au ho s
did
no
add ess
he
se e i y
o
ao ic
egu gi a ion
o
i s
p og es-
sion
in
non-significan
cases
a
baseline,
which
cons i u es
a
subs an ial
limi a ion
o
comp ehending
he
ull
impac
o
GPA
in
ao ic
al e
disease.
Addi ionally,
he
au ho s
iden ified
D-dime
le els
as
he
only
p edic o
o
ao ic
egu gi a ion
p og ession
in
his
g oup
o
pa ien s.
The
associa ion
o
ele a ed
D-dime s
wi h
inflamma ion
and
disease
ac i i y
may
explain
his
finding.
S ill,
pa ien s
wi h
ao ic
al e
disease
we e
olde ,
and
an
inpu
o
he
mul i a ia e
analysis
o
ca dio ascula
isk
p o-
file
would
help
o
u he
unde s and
he
di e ences
ound,9
which
I
belie e
is
an
impo an
limi a ion
o
he
p esen
analysis.
Finally,
only
ou
(3.8%)
o
he
included
pa ien s
equi ed
ao ic
al e
su ge y
du ing
ollow-up.
Compa ed
o
he
pe cen age
o
cases
o
symp oma ic
and
significan
hea
al e
disease
iden ified
in
he
sys ema ic
e iew
pub-
lished
in
2011,8i
migh
be
a gued
ha
he
long- e m
cou se
may
a e
all
be
less
malignan
han
p e iously
hough .
In
his
s udy,
a
small
s ep
was
defini ely
aken
o
u -
he
unde s and
he
long- e m
impac
o
his
a e
sys emic
asculi is
on
he
ca dio ascula
sys em,
specifically
in
cases
o
al e
in ol emen .
Su eillance
is
manda o y,
bu
he
cu en
e idence
aises
se e al
ques ions
ha
equi e
long-
e m
p ospec i e
s udies
wi h
he
in ol emen
o
mul iple
high- olume
cen e s
o
add ess
hese
issues
and
s eng hen
he
e idence
ha
ul ima ely
guides
us
in
clinical
p ac ice.
Funding
No
ex e nal
o
in e nal
unding
was
p o ided.
Conflic s
o
in e es
The
au ho
has
no
conflic s
o
in e es
o
decla e.
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