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EGPA

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg–Strauss syndrome, is a rare form of systemic vasculitis that primarily affects small- to medium-sized vessels. It is classified among the ANCA-associated vasculitides and is characterized by eosinophil-rich granulomatous inflammation and necrotizing vasculitis. The illness often progresses through phases, commonly starting with allergic manifestations, followed by marked eosinophilia and tissue infiltration, and then systemic vasculitis.

Most patients present with a history of asthma and notable eosinophilia, along with allergic rhinitis or sinusitis.

Diagnosis relies on a combination of clinical presentation, laboratory findings, imaging, and histology. The classic approach

Treatment centers on suppressing the inflammatory response. Induction therapy typically starts with corticosteroids; organ-threatening disease or

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Organ
involvement
can
include
the
lungs
(pulmonary
infiltrates
or
eosinophilic
pneumonia),
heart
(myocarditis
or
cardiomyopathy),
peripheral
nerves
(mononeuritis
multiplex),
skin
(purpura
or
nodules),
and
gastrointestinal
tract.
Cardiac
and
renal
involvement
are
among
the
more
serious
complications
and
can
drive
prognosis.
Eosinophil
counts
and
ANCA
status
vary;
MPO-ANCA/p-ANCA
is
positive
in
a
substantial
minority
of
cases,
particularly
those
with
vasculitic
features.
uses
clinical
features
such
as
asthma,
eosinophilia,
neuropathy,
pulmonary
infiltrates,
sinus
abnormalities,
and
biopsy
showing
extravascular
eosinophils
with
vasculitis.
Differential
diagnoses
include
other
forms
of
vasculitis
and
eosinophilic
disorders.
poor
prognostic
features
may
require
additional
immunosuppressants
such
as
cyclophosphamide
or
rituximab.
Maintenance
therapy
can
involve
azathioprine,
methotrexate,
or
mycophenolate.
Targeted
therapy
with
interleukin-5
inhibitors
(for
example,
mepolizumab)
has
been
used
in
selected
cases.
With
modern
treatment,
many
patients
achieve
remission,
though
relapses
occur
and
long-term
follow-up
is
important.