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23 INNOSC Theranostics and Pharmacological Sciences, 2023, Vol. 6, No. 1 Kivrak, et al.
on different clinical features as initial symptoms components, causing skin hardening. Scleroderma-
in its diagnosis, such as the presence of Raynaud like syndrome, especially diabetic sclerodactyly
phenomenon, the nail bed capillary pattern, as the most common skin manifestation of type 1
proximal or distal cutaneous changes, and the DM, strongly correlates with the duration of the
presence of autoantibodies [5], SSc can be divided disease. In many cases, it may be challenging
into two types: SSc with limited skin involvement to distinguish the histopathological changes in
and diffuse skin involvement. By referring to the diabetic scleroderma-like syndrome from those in
European League Against Rheumatism (EULAR) the course of SSc [14].
records, Minier et al. stated that puffy fingers are the Cardiac involvement in SSc may lead to
main findings in the initial assessment of SSc [6]. high morbidity and mortality. Therefore, early
Hypertension (HT), diabetes mellitus (DM), diagnosis and treatment are required. For instance,
and renal diseases are the major systemic vascular the 10-year mortality of patients with cardiac
diseases in which differences can be observed involvement in SSc is around 20%. Both primary
in the disease progression, presentation, and and secondary cardiac involvement may be present
prognosis. The previous studies have shown that in SSc. While primary involvement occurs due to
the development of HT can be predicted in patients the direct effect of inflammation on cardiac tissue,
over 45 with high inflammatory markers and skin secondary involvement is secondary to pulmonary
involvement [7]. HT causes vascular changes hypertension.
similar to those observed in SSc [8]. Perivascular SSc may cause myocardial fibrosis and right and
inflammatory infiltrates, impaired angiogenesis, left ventricular systolic and diastolic dysfunction,
and endothelial apoptosis are all observed in the as well as pericardial and endocardial damages.
early stages of the disease. Data from animal Echocardiography is a contributory non-invasive
models have shown that prolonged, uncontrolled test used to evaluate cardiac involvement. Through
overexpression of vascular endothelial growth echocardiography, the left and right ventricular
factor may have paradoxical effects on the formation functions can be assessed, and pulmonary hypertension
of new vessels, leading to capillary changes similar can be detected with high sensitivity [15].
to those observed in SSc. In addition to impaired In this study, we aimed to investigate the clinical,
angiogenesis, defective vasculogenesis may laboratory, and echocardiographic features of SSc
contribute to the vascular symptoms in SSc [9]. patients in our center, evaluate the proportion of
Cardiovascular diseases are frequently observed patients with cardiovascular disease ratio, and
in patients with rheumatoid joint diseases and compare them with other population-based studies
individuals with SSc [10]. in the Turkish population.
Although the relationship between SSc and
pulmonary arterial hypertension (PAH) is known, 2. Methods
idiopathic PAH and PAH associated with SSc 2.1. Study participants
(SSc-PAH) are histologically different [11].
SSc-PAH is the most common form of PAH In this study, 150 patients who were previously
associated with connective tissue disease. SSc-PAH diagnosed and treated in the rheumatology
is essential to all cases of PAH [12]. Approximately clinic were included in the study. Their age, sex,
12%–15% of patients with SSc are estimated to clinical signs and symptoms, laboratory and
have a lifetime risk of developing PAH. Despite echocardiography findings, and concomitant
treatment with pulmonary vasodilators, patients diseases were evaluated. The determinants of
with SSc-PAH have a high mortality rate [13]. the study were signs of excessive fibrosis (skin
Although the etiopathogenesis of scleroderma- lesion and pulmonary fibrosis), vasculopathy
like conditions in DM is associated with (hypertension and proteinuria), and inflammation
non-enzymatic glycation of collagen, it has not (arthritis and elevated C-reactive protein [CRP],
been established. High blood glucose levels erythrocyte sedimentation rate [ESR], or
can stimulate the proliferation of fibroblasts rheumatoid factor [RF]). This study was approved
and the production of other extracellular matrix by the Local Ethics Committee.

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