International Journal of Molecular Sciences,
Journal Year:
2024,
Volume and Issue:
25(14), P. 7933 - 7933
Published: July 20, 2024
Type
I
interferon
(IFN-I)
signaling
has
been
shown
to
be
upregulated
in
systemic
sclerosis
(SSc).
Dysregulated
B-cell
functions,
including
antigen
presentation,
as
well
antibody
and
cytokine
production,
all
of
which
may
affected
by
IFN-I
signaling,
play
an
important
role
the
pathogenesis
disease.
We
investigated
signature
71
patients
with
more
severe
form
disease,
diffuse
cutaneous
SSc
(dcSSc),
33
healthy
controls
(HCs).
Activation
via
Toll-like
receptors
(TLRs)
can
influence
cascade;
thus,
we
analyzed
effects
TLR
homologue
CD180
ligation
on
B
cells.
stimulation
augmented
phosphorylation
signal
transducer
activator
transcription
1
(STAT1)
dcSSc
cells
(
Frontiers in Immunology,
Journal Year:
2023,
Volume and Issue:
14
Published: Oct. 4, 2023
Autoimmune
diseases
(AIDs)
are
immune
disorders
whose
incidence
and
prevalence
increasing
year
by
year.
AIDs
produced
the
system’s
misidentification
of
self-antigens,
seemingly
caused
excessive
function,
but
in
fact
they
result
reduced
accuracy
due
to
decline
system
which
cannot
clearly
identify
foreign
invaders
thus
issuing
false
attacks,
eventually
leading
disease.
The
occurrence
is
often
accompanied
emergence
inflammation,
inflammatory
mediators
(inflammatory
factors,
inflammasomes)
play
an
important
role
pathogenesis
AIDs,
mediate
process
affecting
innate
cells
(such
as
macrophages)
adaptive
T
B
cells),
ultimately
promote
autoimmune
responses,
so
targeting
mediators/pathways
one
emerging
treatment
strategies
AIDs.
This
review
will
briefly
describe
inflammation
different
give
a
rough
introduction
inhibitors
hoping
have
reference
significance
for
subsequent
options
European Journal of Cancer,
Journal Year:
2024,
Volume and Issue:
204, P. 114071 - 114071
Published: April 22, 2024
Systemic
sclerosis,
a
severe
inflammatory
autoimmune
disease,
shares
common
thread
with
cancer
through
the
underlying
mechanism
of
inflammation.
This
milieu
not
only
drives
immune
dysregulation
characteristic
diseases
but
also
plays
pivotal
role
in
pathogenesis
cancer.
Among
cellular
components
involved,
B
cells
have
emerged
as
key
players
hematologic
tumor
and
contributing
to
persistent
tissue
fibrosis
systemic
well
progression
evasion
Consequently,
novel
therapeutic
strategies
targeting
hold
promise
both
conditions.
Recent
exploration
CD19
CAR
T
sclerosis
patients
has
shown
great
potential,
introduced
possible
risks
drawbacks
associated
viral
vectors,
prolonged
cell
persistence,
lengthy
production
timelines,
high
costs,
necessity
conditioning
organotoxic
fertility-damaging
chemotherapy.
Given
these
challenges,
alternative
CD19-depleting
approaches
are
interest
for
managing
diseases.
Here,
we
present
pioneering
use
blinatumomab,
bispecific
anti-CD3/anti-CD19
engager
patient
progressive,
offering
promising
such
challenging
cases.
Seminars in Respiratory and Critical Care Medicine,
Journal Year:
2024,
Volume and Issue:
45(03), P. 342 - 364
Published: May 7, 2024
Abstract
Systemic
sclerosis
(SSc)
is
a
rare
autoimmune
disease
characterized
by
tripod
combining
vasculopathy,
fibrosis,
and
immune-mediated
inflammatory
processes.
The
prevalence
of
interstitial
lung
(ILD)
in
SSc
varies
according
to
the
methods
used
detect
it,
ranging
from
25
95%.
fibrotic
vascular
pulmonary
manifestations
SSc,
particularly
ILD,
are
main
causes
morbidity
mortality,
contributing
35%
deaths.
Although
early
trials
were
conducted
with
cyclophosphamide,
more
recent
randomized
controlled
have
been
performed
assess
efficacy
tolerability
several
medications,
mostly
mycophenolate,
rituximab,
tocilizumab,
nintedanib.
many
uncertainties
remain,
expert
consensus
emerging
optimize
therapeutic
management
provide
clinicians
evidence-based
clinical
practice
guidelines
for
patients
SSc-ILD.
This
article
provides
an
overview,
light
latest
advances,
available
evidence
diagnosis
Biology,
Journal Year:
2023,
Volume and Issue:
12(2), P. 285 - 285
Published: Feb. 10, 2023
Systemic
sclerosis
(SSc)
is
a
debilitating
autoimmune
disease
that
affects
multiple
systems.
It
characterized
by
immunological
deregulation,
functional
and
structural
abnormalities
of
small
blood
vessels,
fibrosis
the
skin,
and,
in
some
cases,
internal
organs.
Fibrosis
has
devastating
impact
on
patient’s
life
lung
associated
with
high
morbimortality.
Several
immune
populations
contribute
to
progression
SSc,
plasmacytoid
dendritic
cells
(pDCs)
have
been
identified
as
crucial
mediators
fibrosis.
Research
murine
models
skin
shown
pDCs
are
essential
development
fibrosis,
removing
improves
subset
(DCs)
specialized
anti-viral
responses
also
involved
diseases,
such
systemic
lupus
erythematosus
(SLE)
psoriasis,
mostly
due
their
capacity
produce
type
I
interferon
(IFN).
A
IFN
signature
levels
CXCL4,
both
derived
from
pDCs,
poor
prognosis
patients
SSc
correlated
This
review
will
examine
recent
research
molecular
mechanisms
through
which
SSc.
Frontiers in Immunology,
Journal Year:
2023,
Volume and Issue:
14
Published: May 12, 2023
Systemic
sclerosis
(SSc)
is
an
intricate
systemic
autoimmune
disease
with
pathological
features
such
as
vascular
injury,
immune
dysregulation,
and
extensive
fibrosis
of
the
skin
multiple
organs.
Treatment
options
are
limited;
however,
recently,
mesenchymal
stem
cell-derived
extracellular
vesicles
(MSC-EVs)
have
been
acknowledged
in
preclinical
clinical
trials
being
useful
treating
diseases
likely
superior
to
MSCs
alone.
Recent
research
has
also
shown
that
MSC-EVs
can
ameliorate
SSc
changes
vasculopathy,
dysfunction,
fibrosis.
This
review
summarizes
therapeutic
effects
on
mechanisms
discovered
provide
a
theoretical
basis
for
future
studies
role
SSc.
Lara D. Veeken,
Journal Year:
2023,
Volume and Issue:
63(6), P. 1534 - 1538
Published: Sept. 5, 2023
Abstract
Objectives
Autologous
hematopoietic
stem
cell
transplantation
(AHSCT)
has
been
shown
to
improve
long-term
survival
for
early
diffuse
progressive
SSc
compared
with
CYC.
CYC,
however,
does
not
provide
a
benefit
in
SSc.
The
combination
of
MMF
and
rituximab
is
potent
alternative
regimen.
We
aimed
retrospectively
compare
the
outcomes
patients
who
underwent
AHSCT
met
eligibility
criteria
but
received
upfront
therapy
rituximab.
Methods
Repeated
assessments
modified
Rodnan
Skin
Score
(mRSS),
forced
vital
capacity
(FVC),
diffusing
(DLCO)
values
were
conducted.
Clinical
improvement
was
defined
as
an
mRSS
decrease
>25%
or
FVC
increase
>10%.
Event-free
(EFS)
absence
persistent
major
organ
failure
death.
Results
Twenty-one
group
16
group.
Age,
sex
disease
duration
similar
between
two
groups.
at
12
months
seen
18
(86%)
13
(81%)
(P
=
0.7).
hazard
ratio
EFS
24
favoured
(HR
0.09,
P
0.04).
During
follow-up,
both
groups
exhibited
significant
comparable
reduction
each
time
interval
up
months.
Conclusion
eligible
resulted
skin
lung
clinical
better
safety
profile
Autoimmunity Reviews,
Journal Year:
2024,
Volume and Issue:
23(6), P. 103574 - 103574
Published: May 21, 2024
Large-vessel
vasculitides
(LVV)
comprise
a
group
of
chronic
inflammatory
diseases
the
aorta
and
its
major
branches.
The
most
common
forms
LVV
are
giant
cell
arteritis
(GCA)
Takayasu
(TAK).
Both
GCA
TAK
characterized
by
granulomatous
inflammation
vessel
wall
accompanied
maladaptive
immune
vascular
response
that
promotes
damage
remodeling.
process
in
starts
adventitia
where
fibroblasts
constitute
dominant
population.
Fibroblasts
traditionally
recognized
for
synthesizing
renewing
extracellular
matrix
thereby
being
players
maintenance
normal
tissue
architecture
repair.
More
recently,
have
emerged
as
highly
plastic
population
exerting
various
functions,
including
regulation
local
processes
organization
cells
at
site
through
production
cytokines,
chemokines
growth
factors
well
cell-cell
interaction.
In
this
review,
we
summarize
discuss
current
knowledge
on
LVV.
Furthermore,
identify
key
questions
need
to
be
addressed
fully
understand
role
pathogenesis
