Reumatizam, Vol. 62 No. suppl. 1, 2015.
Review article
EARLY SCLERODERMA
Silvia Bellando Randone
Marco Matucci Cerinic
Abstract
Systemic sclerosis (SSc) is a chronic autoimmune disease,
characterized by a high level of clinical heterogeneity and
associated with a high morbidity along with the highest
disease-specific mortality of all autoimmune connective
tissue diseases. SSc is quite easy to diagnose in the advanced
phase, i.e., when it has already evolved to obliterative
vasculopathy and skin and internal organ fibrosis, but
it is difficult to establish a diagnosis in the early phase.
This limits the possibility to start early treatment, as well
as the potential for prevention of disease evolution and
tissue damage. Raynaud’s phenomenon (RP) has been
proposed as one of the defining clinical features for the
diagnosis of “early” SSc, so that particular attention should
be paid to this symptom, even in the absence of other
signs of the disease. Based on previous diagnostic criteria,
the diagnosis of SSc can be delayed for several years
after the onset of RP, and even after the onset of the first
non-RP symptom.
This time gap between symptoms and diagnosis, mainly
based on dermal or internal organ fibrosis, is a ‘‘window of opportunity’’ that could represent a chance to intervene
earlier in the disease course, thus potentially preventing
organ damage.
The definition of Very early SSc has been proposed as a
state characterized by RP, puffy fingers, disease-specific
autoantibodies, and pathognomonic microvascular alterations
at capillaroscopy, without skin and internal organ
involvement. In patients with the above symptoms further
investigations such as esophageal manometry, B-mode echocardiography,
and lung function tests are recommended
to detect preclinical alterations of internal organs in SSc.
Recently, new classification criteria have been proposed
with the goal to identify patients in the earliest phase of
the disease. However, as predictors of the future course of
the disease are still unknown, patients must be followed up
regularly, even though the ideal frequency of visits has not
yet been established.
Keywords
systemic sclerosis; early diagnosis; treatment; classification criteria; early systemic sclerosis
Hrčak ID:
182777
URI
Publication date:
10.10.2015.
Visits: 2.282 *