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Inclusion body myositis: from immunopathology and degenerative mechanisms to treatment perspectives.
[inclusion body myositis]
Inclusion
body
myositis
is
the
most
common
inflammatory
myopathy
above
the
age
of
50
.
It
becomes
clinically
apparent
around
the
fourth
decade
and
leads
to
a
slowly
,
but
relentlessly
progressive
decline
in
muscular
wasting
and
weakness
.
The
pathology
consists
of
a
complex
network
of
inflammatory
and
degenerative
mechanisms
,
which
lead
to
an
attack
of
muscle
fibers
by
auto-reactive
T
cells
and
possibly
antibodies
.
At
the
same
time
,
various
aberrant
proteins
accumulate
within
the
muscle
fibers
,
including
β-amyloid
,
tau
and
α-synuclein
.
Several
key
components
of
proinflammatory
cell
stress
mechanisms
such
as
nitric
oxide
production
and
macroautophagic
processing
contribute
to
the
muscle
fiber
damage
.
So
far
,
none
of
the
anti-
inflammatory
or
immunomodulatory
treatment
efforts
have
been
able
to
halt
the
disease
progression
and
help
the
patients
.
In
this
summary
,
the
current
concept
of
the
complex
disease
pathology
of
IBM
is
reviewed
with
a
focus
on
recent
findings
as
well
as
future
treatment
perspectives
.
