Clinical data
In all patients we collected clinical, anthropometric and laboratory
features, including disease duration, onset of Raynaud’s phenomenon,
skin involvement according to the modified Rodnan Skin Score (mRSS) (12)
and cutaneous subset according to LeRoy criteria (13). Moreover, the
presence/absence of interstitial lung disease (ILD) defined by chest
HR-CT scan, pulmonary function test (PFT) with FVC, FEV1/FVC, DLCO and
RV estimation, the 6-minute walking distance test (6mWDT), the
presence/absence of pulmonary arterial hypertension (PAH) diagnosed by
right heart catheterization, the presence/absence of esophagopathy
evaluated at esophageal scintigraphy or esophageal manometry or chest
HR-CT scan, the presence of digital pitting-scars and/or digital ulcers
(past or active), the nailfold capillaroscopic pattern ”Early”, ”Active”
and ”Late” (14), the disease activity according to the ESSG (European
Scleroderma Study Group) disease activity index (15), were recorded.
Drugs investigated were: peripheral vasodilators, iloprost,
immunosuppressive drugs, glucocorticoids, antihypertensive and
lipid-lowering agents, targeted therapy for the treatment of PAH
(bosentan, sildenafil, tadalafil, ambrisentan), oxygen therapy. The BMI,
waist circumference (Waist C) and Waist-to-Hip Ratio (WHR),
cardiovascular risk index, acute phase proteins, cholesterol and
triglycerides serum levels, previous smoking status, comorbidity (such
as arterial hypertension, diabetes mellitus and hyperlipidemia) were
also assessed. The BMI was calculated as weight in kilograms divided by
the height in square meters and in accordance with the WHO BMI category
classification, patients were considered underweight (BMI
<18.5), normal-weight (BMI= 18.5-24.99), overweight (BMI=
25-29.99) and obese (≥30). The cardiovascular (CV) risk was estimated as
the ratio of total cholesterol/high-density-lipoprotein (HDL), or
applying the Framingham Risk Score (FRS).