JOURNAL

AUGUST 2016
Diagnosis and classification of
idiopathic inflammatory
myopathies
Andi Tenri Hardiyanti Atoga
10542 0270 11
Pembimbing : dr. Jimmy Gunardi Tanzil,
Sp.Pd

Bagian Ilmu Penyakit Dalam

Fakultas Kedokteran
Universitas Muhammadiyah Makassar
2016

DEFINITION
The idiopathic inflammatory myopathies
(IIMs), collectively termed myositis, are a
heterogeneous group of disorders that are
characterized clinically by chronic muscle
weakness and low muscle endurance and by
inflammatory cell infiltrates in muscle tissue.
Based on differences in clinical and
histopathological
features,
IIMs
been
subdivided
into
polymyositis
(PM),
dermatomyositis (DM) and inclusion body
myositis (IBM).

IIMs are not only muscle disorders as other organs

are often affected, such as the skin in DM and the
lungs, heart, joints and gastrointestinal tract in both
PM and DM.
IIMs are rare diseases with a prevalence of 10 in 100
000 individuals, an international collaboration was
essential, as was the interdisciplinary effort including
experts in adult and pediatric rheumatology,
neurology, dermatology and epidemiology. The new
criteria have been developed based on data from
more than 1500 patients from 47 centres worldwide
and are based on clinically easily available variables.

Diagnosis
The diagnosis of IIMs is based on clinical
symptoms such as subacute development
of symmetrical muscle weakness and
muscle fatigue, most prominent in proximal
muscles, and signs such as laboratory
results
supporting
skeletal
muscle
inflammation
and
muscle
fibre
degeneration and repair (regeneration)

Tools for diagnosis of idiopathic inflammatory

myopathies
Clinical history of muscle weakness or

muscle fatigue
Clinical examination: muscle atrophy,
weakness, skin rash
History of previous medication
Family history of rheumatic or muscle
disease
Muscle enzymes in serum (CK, LD, AST,
ALT and aldolase)
Muscle biopsy from affected skeletal
muscle

When myositis diagnosis has been

confirmed, other organ involvement
should be searched for:
HRCT of lungs
Pulmonary function tests and

diffusion capacity
ECG
Echocardiography

Pathognomonic rashes
of
dermatomyositis:
Gottrons papules, red
or
violet
papules
occurring over the
knuckles,
the
interphalangeal joints
and other extensor
surfaces

Gottrons sign, red

or violet macules
occurring over the
knuckles and
sometimes the
interphalangeal
joints in patients
with
dermatomyositis.

Heliotrope rash, red or violet

eruption over the upper eyelids and
sometimes extending around the
eye

Summary
The new myositis classification criteria are
based on patients and comparators from
many centres worldwide representing
different ethnicities. Thus, the patients
should be representative of various clinical
phenotypes of myositis. The variables in
the final version of the criteria are easily
available, clinically relevant and collected
in the routine diagnostic workup of patients
in most centres.

Elements of the classification criteria for idiopathic

inflammatory myopathies (except IBM) approved by the
Myositis Study Group and the 119th European
Neuromuscular Centre workshop.

Elements of the classification criteria for idiopathic

inflammatory myopathies (except IBM) approved by the
Myositis Study Group and the 119th European
Neuromuscular Centre workshop.

Elements of the classification criteria for idiopathic

inflammatory myopathies (except IBM) approved by the
Myositis Study Group and the 119th European
Neuromuscular Centre workshop.

for idiopathic inflammatory myopathies

(except IBM) to define different myositis
phenotypes
Definite polymyositis

Amyopathic dermatomyositis

TERIMA KASIH