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Manfredi, et al: Capillaroscopy in SSc and DM
Personal non-commercial use only. The Journal of Rheumatology Copyright © 2016. All rights reserved.
Nailfold Videocapillaroscopy Alterations in
Dermatomyositis and Systemic Sclerosis:
Toward Identification of a Specific Pattern
Andreina Manfredi, Marco Sebastiani, Federica Campomori, Nicolò Pipitone, Dilia Giuggioli,
Michele Colaci, Emanuela Praino, and Clodoveo Ferri
ABSTRACT. Objective. The term scleroderma pattern typically defines capillary abnormalities of scleroderma
spectrum disorders, mainly systemic sclerosis (SSc) and dermatomyositis (DM). Our study aimed to
investigate differences in nailfold videocapillaroscopy (NVC) between DM and SSc, with a
cross-sectional and longitudinal evaluation.
Methods. NVC features of 29 consecutive patients with DM were compared with 90 patients with
SSc categorized into the 3 subsets of scleroderma pattern: early, active, and late. Twenty patients with
DM and all with SSc were also longitudinally reevaluated after 30 months of followup.
Results. At baseline, all SSc groups showed giant capillaries, with significant differences with DM
only for early and active pattern. Ramified capillaries were significantly more frequent and severe in
DM than in early and active patterns, while DM showed an opposite trend compared with late pattern.
Capillary loss was lower in early pattern and higher in active and late, compared with DM. Finally,
giant-ramified capillaries were almost exclusive of DM. During followup, NVC showed a different
evolution in DM and SSc. In DM we recorded a reduction of giant capillaries, while ramified capil-
laries increased both in DM and in early and active SSc pattern. The number of capillaries recovered
in DM; conversely, capillary loss slightly worsened in all SSc patterns. Giant-ramified capillaries
significantly decreased in patients with DM, remaining rare in patients with SSc.
Conclusion. Our study strengthens the specificity of DM and SSc microangiopathy and points out
the need for large prospective studies to confirm our results and possibly to revise current terminology
by distinguishing between “scleroderma” and “dermatomyositis” patterns. (First Release June 15
2016; J Rheumatol 2016;43:1575–80; doi:10.3899/jrheum.160122)
Key Indexing Terms:
SYSTEMIC SCLEROSIS DERMATOMYOSITIS MICROANGIOPATHY
VIDEOCAPILLAROSCOPY SCLERODERMA PATTERN
From the Rheumatology Unit, University of Modena and Reggio Emilia,
Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena;
Rheumatology Unit, Arcispedale Santa Maria Nuova, Reggio Emilia;
Rheumatology Unit, University of Bari, Bari, Italy.
A. Manfredi, MD, Rheumatology Unit, University of Modena and Reggio
Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena;
M. Sebastiani, MD, Rheumatology Unit, University of Modena and Reggio
Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena;
F. Campomori, MD, Rheumatology Unit, University of Modena and
Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena;
N. Pipitone, MD, Rheumatology Unit, Arcispedale Santa Maria Nuova,
Reggio Emilia; D. Giuggioli, MD, Rheumatology Unit, University of
Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria
Policlinico di Modena; M. Colaci, MD, Rheumatology Unit, University of
Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria
Policlinico di Modena; E. Praino, MD, Rheumatology Unit, University of
Bari; C. Ferri, MD, Professor, Rheumatology Unit, University of Modena
and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di
Modena.
Dr. Manfredi and Dr. Sebastiani contributed equally to this article.
Address correspondence to Dr. A. Manfredi, Rheumatology Unit,
University of Modena and Reggio Emilia, Via del Pozzo 71, 41121
Modena, Italy. E-mail: andreina.manfredi@gmail.com
Accepted for publication April 19, 2016.
The term scleroderma pattern was first used by Brown and
O’Leary in 1925 to define capillary abnormalities in the
course of systemic sclerosis (SSc)
1
. It is represented by the
association of typical capillaroscopic microvascular alter-
ations, namely giant capillaries, microhemorrhages, ramified
capillaries, microvascular array disorganization, and capillary
loss or avascular areas
2,3,4
. It has since been observed that
scleroderma pattern is not specific to SSc, but potentially
associated with scleroderma spectrum disorders, including
(with some variations according to different authors) SSc,
myositis, systemic lupus erythematosus, mixed connective
tissue disease, and undifferentiated connective disease
5
. SSc
and dermatomyositis (DM) are more frequently associated
with this typical microvascular derangement
6
.
In the course of SSc, the vascular morphological changes
in the nailfold occur very early and they have been widely
investigated
5
. In 2000, Cutolo, et al proposed a reclassifi-
cation of scleroderma pattern into 3 different subgroups:
early, active, and late pattern, characterized by prevalent
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