. 2015;2(3):46-49.
Blood perfusion
Systemic Sclerosis (SSc) is a connective
tissue disease with multifactorial
aetiology and autoimmune pathogenesis.
SSc is characterized by structural and
functional alterations of microcirculation,
with important clinical implications, such
as Raynaud Phenomenon (RP) and digital
ulcers1,2. For these reason, morphological
and functional assessment of the peripheral
microvasculature is a must for diagnosis,
prognosis and therapy in SSc patients 2.
Nailfold videocapillaroscopy
Nailfold videocapillaroscopy (NVC) is
the best safe and non-invasive method
to detect morphological microvascular
abnormalities. NVC allows to distinguish
secondary RP from both primary RP and
healthy subjects, identify morphological
patterns that are specific to various SSc
stages (‘Early’, ‘Active’ and ‘Late’ patterns
of microvascular damage) and calculate
the Microangiopathy Evolution Score
(MES) to follow disease evolution3,4.
The video-capillaroscope makes
use of a magnification system (from 50x
up to 500x magnification), and it has an
optical/digital probe which can be moved
over the surface of the finger nails from
the 2nd to the 5th finger of both hands2.
The normal NVC image is characterized
by normal skin transparency, morphology
of the capillary to “U” or “hairpin shape”,
morphological/structural homogeneity,
10-12 capillaries / linear millimetre, one
capillary inside dermal papilla, diameters
of capillary branches <20 μm, and lack of
morphological atypia2. Nailfold capillaries
are frequently normal in primary RP,
but it is possible to observe capillaries
with efferent branch enlargement or
tortuous capillaries. Therefore in normal
conditions, or in the presence of primary
RP, the NVC examination is characterized
by a regular array of capillary loops
along the nailfold bed, without abnormal
Different techniques to assess microvascular damage in
systemic sclerosis
Ruaro B1, Sulli A1, Smith V2, Paolino S1, Pizzorni C1, Cutolo M1
enlargements nor capillary loss2.
Conversely, secondary RP is characterized
by the morphological signs that represent
the microvascular damage: these include
giant capillaries, microhaemorrhages,
capillary loss, presence of avascular
areas and angiogenesis. These sequential
capillaroscopic changes are typical of the
microvascular involvement observed in
more than 95% of SSc patients and are
described by the term “SSc pattern”2,3.
NVC technique identifies
morphological patterns specific to
various stages of SSc (‘Early’, ‘Active’
and ‘Late’ patterns)3,4. The ‘Early’
SSc pattern is characterized by few
enlarged/giant capillaries, few capillary
microhaemorrhages, no evident capillary
loss and a relatively well preserved
capillary distribution. The ‘Active’ SSc
pattern, a marker of disease progression, is
characterized by frequent giant capillaries
(more than 66%), frequent capillary
microhaemorrhages, moderate (up to 33%)
capillary loss, absent or mild ramified
capillaries and a mild disorganization of
the capillary architecture. In the ‘Late’
SSc pattern there is irregular enlargement
of the capillaries, severe (>66%) capillary
loss with evident avascular areas,
ramified or bushy capillaries and a severe
disorganization of the normal capillary
array, although giant capillaries and
microhaemorrhages are almost absent3,4
(Figure 1). NVC is also used to make a
quantitative assessment (i.e. quantify
certain characteristics and make semiquantitative
scoring) of the microvascular
damage. The usual capillaroscopic
parameters (diagnostic parameters,
such as irregularly enlarged capillaries,
giant capillaries, microhaemorrhages;
and progression parameters, such as
reduced capillary number, capillary
ramifications and capillary architectural
disorganization) are evaluated by a semiquantitative
scale. Score 0-3 has been
adopted for all these parameters3
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