Subclinical atherosclerosis in systemic sclerosis: Different risk profiles among patients according to clinical manifestations.

Introduction: Like other autoimmune diseases, systemic sclerosis (SSc) has been described to be associated with accelerated atherosclerosis (ATS). Before clinical manifestations of cardiovascular disease (CVD) occur, subclinical ATS can be investigated in different ways.

Aim: To evaluate the presence of subclinical ATS in a group of patients with SSc, and to identify different risk profiles among patients.

Anti-CXCL4 Antibody Reactivity Is Present in Systemic Sclerosis (SSc) and Correlates with the SSc Type I Interferon Signature.

Systemic sclerosis (SSc) is characterized by skin/internal organ fibrosis, vasculopathy and autoimmunity. Chemokine (C-X-C motif) ligand 4 (CXCL4) is an SSc biomarker, predicting unfavorable prognosis and lung fibrosis. CXCL4 binds DNA/RNA and favors interferon (IFN)-α production by plasmacytoid dendritic cells (pDCs), contributing to the type I IFN (IFN-I) signature in SSc patients.

CXCL4 assembles DNA into liquid crystalline complexes to amplify TLR9-mediated interferon-α production in systemic sclerosis.

Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by fibrosis and vasculopathy. CXCL4 represents an early serum biomarker of severe SSc and likely contributes to inflammation via chemokine signaling pathways, but the exact role of CXCL4 in SSc pathogenesis is unclear. Here, we elucidate an unanticipated mechanism for CXCL4-mediated immune amplification in SSc, in which CXCL4 organizes "self" and microbial DNA into liquid crystalline immune complexes that amplify TLR9-mediated plasmacytoid dendritic cell (pDC)-hyperactivation and interferon-α production.

From VEDOSS to established systemic sclerosis diagnosis according to ACR/EULAR 2013 classification criteria: a French-Italian capillaroscopic survey.

Objectives: Nailfold capillaroscopy (NC) shows microcirculatory abnormalities in systemic sclerosis (SSc). The inclusion of NC specific abnormalities increases the sensitivity of both 2013 ACR/EULAR and VEDOSS (Very Early Diagnosis of Systemic Sclerosis) classification criteria. We aimed to detect NC features able to predict progression toward established SSc in VEDOSS patients.

Occupational therapy integrated with a self-administered stretching program on systemic sclerosis patients with hand involvement.

Objectives: To evaluate the effect of occupational therapy (OT) intervention, integrated with a self-administered stretching program on the hands of patients with SSc, after one and three months of treatment.

Methods: We enrolled 31 patients with SSc, randomly allocated to the occupational group (15 patients) or to the control group (16 patients). Each patient received specific outcome measures: Canadian Occupational Performance Measure (COPM), HAQ, Short-Form Health Survey (SF-36), Duruoz Hand Index (DHI), reassessed after 1 (T1) and three months (T2).

Pulmonary hypertension in systemic sclerosis: prevalence, incidence and predictive factors in a large multicentric Italian cohort.

Objectives: This paper aims to investigate the prevalence, the incidence of pulmonary hypertension (PH) and its subtypes in Italian patients with systemic sclerosis (SSc) and to characterise features associated with and predictive of development of PH.

Methods: Eight-hundred and sixty-seven consecutive SSc patients recruited at 4 Italian centres were enrolled. At admission, all patients underwent a careful history, physical examination, EKG, lung high resolution computed tomography (HRCT), pulmonary function tests, B-mode echocardiography and right heart catheterisation (RHC), if indicated.

Systemic sclerosis patients with and without pulmonary arterial hypertension: a nailfold capillaroscopy study.

Objective: Pulmonary arterial hypertension (PAH) is a complication of SSc due to increased vascular resistance, and abnormal vascularity is a well-known feature of the disease as shown by nailfold videocapillaroscopy (NVC). This study investigated for specific NVC changes in SSc patients with and without PAH to assess any useful difference.

Methods: Twenty-four SSc patients, 12 with PAH and 12 without, entered the study.