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Criteria for the
Classification of Systemic Sclerosis
(Scleroderma)
1980 Criteria
for the Classification of
Systemic Sclerosis
The American
College of Rheumatology (former
American Rheumatism Association
- ARA) has defined criteria,
that are 97 % sensitive and 98 %
specific for systemic sclerosis
(SSc) as follows:
Major
criterion:
Minor
criteria:
-
Sclerodactyly (only fingers
and/or toes)
-
Digital
pitting scars or loss of
substance of the digital
finger pads (pulp loss)
-
Bilateral
basilar pulmonary fibrosis
The patient
should fulfill the major
criterion or two of the three
minor criteria. Raynaud's
phenomenon is observed in 90-98
% of SSc patients.
Subsets of
Systemic Sclerosis
|
|
Diffuse |
Limited* |
|
Skin
involvement |
Distal
and proximal
extremities, face, trunk |
Distal to
elbows, face |
|
Raynaud’s
phenomenon |
Onset
within 1 year or at time of
skin changes |
May
precede skin disease by
years |
|
Organ
involvement |
Pulmonary
(interstitial fibrosis);
renal (renovascular
hypertensive crisis);
gastrointestinal; cardiac |
Gastrointestinal; pulmonary
arterial hypertension after
10-15 years of disease in
<10% of patients; biliary
cirrhosis |
|
Nail fold
capillaries |
Dilatation and dropout |
Dilatation without
significant dropout |
|
Antinuclear antibodies |
Anti-topoisomerase
1 |
Anticentromere |
* Also
referred to as CREST( calcinosis,
Raynaud’s, esophageal
dysmotility, sclerodactyly,
telangiectasia).
ABCDCREST
Criteria for
the Classification of Systemic
Sclerosis
-
Autoantibodies: autoantibodies
to centromere proteins (CENPs)
detected by indirect
immunofluorescence;
anti-Scl-70 (topoisomerase I)
detected by double
immunodiffusion; anti-fibrillarin
(U3-RNP) detected by
immunoprecipitation
-
Bibasilar
pulmonary fibrosis detected by
chest radiograph: linear
shadows or “honey-comb”
reticular appearance most
expressed at the periphery of
the lungs and at the bases
-
Contracture
of the joints defined as
permanent limitation of joint
motion. The prayer sign is
detected when a patient
opposed the palmar surfaces of
both hands with extended
wrists. The sign is positive
when the patient is unable to
oppose the palms. This
suggests joint or skin
pathology, or shortening of
the forearm flexors
-
Dermal
thickening can be defined by
the modified Rodnan skin
score, which employs clinical
palpation of the skin as
described
-
Calcinosis
cutis, most often located on
the fingers, is intra and/or
subcutaneous deposits of
hydroxyapatite that can
ulcerate the skin; it can be
detected by radiography,
crystallographic or chemical
analysis
-
Raynaud’s
phenomenon is a sudden pallor
of an acral structure (e.g.,
fingers, whole hand, toes, tip
of nose, earlobe, or tongue).
The involved area may
subsequently develop cyanosis
and, with re-warming, become
erythematous. Determination is
by patient’s history or
physician’s observation
-
Esophageal
distal hypomotility can be
detected by cine/video barium
esophagram, performed in the
upright and supine position.
Reflux-esophagitis can be
detected by
esophagogastroduodenoscopy in
the forms of erosive
esophagitis or Barret’s
esophagus
-
Sclerodactyly is symmetric
thickening and tightening of
the skin on the digits. Before
sclerodactyly develops there
could be a phase of
non-pitting digital edema of
varying duration. It is
defined as non-pitting
increase in soft tissue mass
of the digits that extends
beyond the normal confines of
the joint capsules
-
Teleangiectasias are visible
macular dilatations of
superficial cutaneous blood
vessels that collapse upon
pressure and fill slowly when
pressure is released. Common
locations are the digits,
face, lips, tongue
A
classification of definite SSc
requires three or more criteria.
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