Advances in Rheumatology,
Journal Year:
2024,
Volume and Issue:
64(1)
Published: Oct. 9, 2024
Systemic
vasculitis
is
a
group
of
rare
diseases
that
share
an
essential
characteristic:
inflammation
blood
vessel
walls.
This
injury
occurs
during
the
disease
course,
but
specific
features
vary
for
each
entity.
In
this
paper,
we
will
address
relevant
aspects
newest
monogenic
mutation
vasculitis,
such
as
deficiency
adenosine
deaminase
2
(ADA2)
and
VEXAS
syndrome
(UBA1),
other
Cogan
Susac
may
some
similarities
with
them.
ARP Rheumatology,
Journal Year:
2024,
Volume and Issue:
3(2), P. 151 - 156
Published: June 30, 2024
Vacuoles,
E1
enzyme,
X-linked,
autoinflammatory,
somatic
(VEXAS)
syndrome
is
an
emerging
adult-onset
systemic
autoinflammatory
disorder
affecting
multiple
organ
systems.
While
lung
involvement
common
in
this
syndrome,
literature
regarding
specific
patterns
sparse.
In
report,
we
present
a
case
description
of
patient
with
VEXAS
who
presented
at
the
emergency
department
on
two
separate
occasions
acute
interstitial
pneumonia
(AIP)
and
diffuse
alveolar
hemorrhage
(DAH).
A
review
comparison
our
observed
findings
to
general
AIP,
DAH
provided.
This
report
underscores
rarity
pulmonary
manifestations
associated
contributing
valuable
insight
limited
available
topic.
Aktuelle Rheumatologie,
Journal Year:
2024,
Volume and Issue:
49(05), P. 310 - 316
Published: Sept. 26, 2024
Zusammenfassung
Die
Behandlung
von
älteren
Patient:innen
bedarf
besonderer
Aufmerksamkeit
und
spezieller
Fachkenntnisse.
Altersbedingte
Veränderungen
des
Immunsystems,
multiple
Komorbiditäten
eine
altersabhängige
Pharmakokinetik
-dynamik
erfordern
individuelle
sorgfältige
Herangehensweise.
Ziel
dieses
Artikels
ist
es,
aktuelle
Erkenntnisse
praxisrelevante
Ansätze
zur
Diagnostik
Therapie
ausgewählter
rheumatologischer
Erkrankungen
im
höheren
Lebensalter,
wie
Polymyalgia
rheumatica
(PMR),
Riesenzellarteriitis
(RZA),
Schnitzler-Syndrom
VEXAS-Syndrom
zu
präsentieren.
Immuno,
Journal Year:
2024,
Volume and Issue:
4(3), P. 286 - 300
Published: Sept. 23, 2024
VEXAS
syndrome
is
a
new
disease
entity
with
symptoms
that
can
mimic
hematological,
rheumatic
and
dermatological
diseases.
It
important
to
take
multidisciplinary
approach
patient
care,
taking
into
account
genetic
testing,
in
which
the
presence
of
mutations
UBA1
gene
confirm
diagnosis.
mutation
has
been
shown
be
involved
induction
inflammatory
response
through
many
different
mechanisms.
NF-κB
TNF-α
pathways
appear
most
syndrome.
There
are
result
outcomes,
suggesting
it
possible
prognostic
factor.
Furthermore,
differ
how
they
impair
function.
Cytokines
have
significantly
altered
patients;
however,
their
exact
expression
importance
were
not
clearly
defined.
Interleukins,
such
as
interleukin
(IL)-6,
IL-1,
IL-2R
others,
reported
expressed
at
an
level,
similarly
other
cytokines,
IFN-γ
or
TNF-α.
worth
noting
certain
cytokines
vary
between
patients,
poses
therapeutic
difficulties
selecting
right
drug.
Therefore,
aim
this
review
was
describe
associate
mutation.
Advances in Rheumatology,
Journal Year:
2024,
Volume and Issue:
64(1)
Published: Oct. 9, 2024
Systemic
vasculitis
is
a
group
of
rare
diseases
that
share
an
essential
characteristic:
inflammation
blood
vessel
walls.
This
injury
occurs
during
the
disease
course,
but
specific
features
vary
for
each
entity.
In
this
paper,
we
will
address
relevant
aspects
newest
monogenic
mutation
vasculitis,
such
as
deficiency
adenosine
deaminase
2
(ADA2)
and
VEXAS
syndrome
(UBA1),
other
Cogan
Susac
may
some
similarities
with
them.
