Is there a role for laser speckle contrast analysis (LASCA) in predicting the outcome of digital ulcers in patients with systemic sclerosis?

Objective: Digital ulcers (DUs) represent one major burden for patients with systemic sclerosis (SSc). The objectives of our study were to evaluate blood flow in SSc-DUs with laser speckle contrast analysis (LASCA) and to correlate the skin perfusion to clinical and laboratory data.

Methods: Forty DUs in 31 consecutive patients with SSc according to 2013 ACR/EULAR criteria (20 with limited cutaneous disease, 3 males) were prospectively examined with LASCA.

Iloprost use and medical management of systemic sclerosis-related vasculopathy in Italian tertiary referral centers: results from the PROSIT study.

Vasculopathy is a crucial feature of systemic sclerosis (SSc), and Raynaud's phenomenon (RP) and digital ulcers (DU) have a deep impact on the quality of patients' life. The management of vascular disease can be challenging for the clinician because of the suboptimal tolerability of the treatments and lack of consensus on the best therapeutic approach. Intravenous iloprost, a synthetic analogue of prostacyclin, is broadly used for the treatment of RP and ischemic ulcers secondary to SSc.

Muscular vasculitis confined to lower limbs: description of two case reports and a review of the literature.

Muscular involvement is common during systemic vasculitides, such as polyarteritis nodosa. However, in rare cases, muscular involvement can be the only clinically evident feature of the disease. The clinical pattern of isolated muscular vasculitis may mimic several other inflammatory muscle disorders, such as idiopathic inflammatory myositis, and may represent a challenge in differential diagnosis.

One year in review 2016: systemic sclerosis.

Systemic sclerosis is a rare acquired systemic disease characterised by a complex pathogenesis and multi organ involvement. Every year, novel insights into the pathogenesis, diagnosis and treatment of this severe disease are published. Herewith, we provide an overview of the most significant literature contributions published over the last year.

Thigh magnetic resonance imaging for the evaluation of disease activity in patients with idiopathic inflammatory myopathies followed in a single center.

Introduction: In patients with idiopathic inflammatory myopathies (IIM), magnetic resonance imaging (MRI) has been proposed as a useful tool for diagnosis and follow-up. It may identify muscle inflammation (edema) and fatty infiltration for evaluation of disease activity and damage. Little information is available on the role of MRI in assessment of large cohorts of adult patients with IIM.

Adult-onset Still's disease: an Italian multicentre retrospective observational study of manifestations and treatments in 245 patients.

Adult-onset Still's disease (AOSD) is a systemic inflammatory condition of unknown aetiology characterized by typical episodes of spiking fever, evanescent rash, arthralgia, leukocytosis and hyperferritinemia. Given the lack of data in Italian series, we promote a multicentric data collection to characterize the clinical phenotype of Italian patients with AOSD. Data from 245 subjects diagnosed with AOSD were collected by 15 centres between March and May 2013.

Development of de novo major involvement during follow-up in Behçet's syndrome.

The primary aim of the study was to evaluate the incidence of de novo major involvement during follow-up in a cohort of patients with Behçet's syndrome (BS); the secondary aim was to analyse the epidemiological profile and the long-term outcome of those patients who developed new major involvement. Among our cohort of 120 BS patients, we evaluated all subjects who had no major organ involvement during the early years of their disease; specifically, at disease onset, the 52% of the cohort presented a prevalent mucocutaneous involvement. The primary outcomes were represented by the following: Hatemi et al.

Epidemiology and management of neuropsychiatric disorders in Behçet's syndrome.

Behçet's syndrome (BS) is a systemic, chronic, relapsing vasculitis, typically characterized by recurrent orogenital ulcers, ocular inflammation and skin manifestations; articular, vascular, gastroenteric and neurological involvement may also occur. Besides the other clinical features of BS, it seems relatively frequent that patients with BS develop a neurobehavioural syndrome, characterized by euphoria, bipolar disorders and paranoid attitudes, loss of insight/disinhibition, and indifference to their disease, defined as 'neuro-psycho-BS'. To date, the pathogenetic mechanism underlying neuro-psycho-BS has not been determined.

Prognostic value of flow-mediated dilation in patients with systemic lupus erythematosus: a pilot prospective cohort study.

This pilot study evaluated the predictive value of flow-mediated dilation (FMD) for damage accrual in a cohort of SLE patients. Thirty-eight female SLE patients without cardiovascular involvement were enrolled. Clinical history, traditional cardiovascular risk factors, laboratory parameters, disease activity and damage and brachial artery FMD were collected at study entry and after a mean follow-up of 4.

Clinically amyopathic dermatomyositis: analysis of a monocentric cohort.

Objective: Clinically amyopathic dermatomyositis (CADM) is characterized by the presence of specific cutaneous manifestations of dermatomyositis (DM) without clinical signs of muscular involvement. The aim of this study was to examine the prevalence, clinical characteristics, and outcome of patients with CADM followed at our Rheumatology Unit.

Methods: Clinical charts of patients diagnosed as DM were retrospectively examined.

Isolated aortitis versus giant cell arteritis: are they really two sides of the same coin?

Objectives: The aim of the study was to compare epidemiological data, clinical findings and results of investigations in patients with isolated aortitis and those with giant cell arteritis (GCA) to establish whether patients with isolated aortitis differ from those with GCA.

Methods: We reviewed the medical notes of all patients consecutively seen in two Rheumatology centres in the last two decades with a suspicion of GCA, searching for cases characterised by abnormal [18F] fluorodeoxyglucose (FDG) PET uptake of the aorta. 'Isolated aortitis' was defined as increased FDG uptake in the aorta not explained by atherosclerosis in the absence of FDG uptake in other large vessels.

Cancer-associated myositis: a 35-year retrospective study of a monocentric cohort.

This study is aimed at retrospectively studying cancer-associated inflammatory myopathies (CAM) in a cohort of patients with inflammatory myopathies. CAM were diagnosed if the tumor was diagnosed 2 years before or after disease onset. One hundred and sixty-two patients were included, 27 (17 %) had CAM.

Improved survival in limited scleroderma-related pulmonary artery hypertension.

Reportedly, patients with scleroderma-related pulmonary hypertension (SSc-PAH) respond poorly to new vasoactive drugs (NVD). Forty-nine SSc-PAH patients underwent right heart catheterization (RHC) and, according to NVD availability, divided as follows: Group 1 (n = 23, from 1999 to 2004, poor availability), and Group 2 (n = 26, from 2005 to 2010, good availability). Before diagnostic RHC, NVD had been given to 30 % of the patients in Group 1, and 58 % of those in Group 2 (p = 0.

Alteration of microcirculation is a hallmark of very early systemic sclerosis patients: a laser speckle contrast analysis.

Objectives: To investigate blood flow and microvascular reactivity by laser speckle perfusion imager (Perimed, Jarfalla) in consecutive patients affected by Raynaud's phenomenon at baseline and following dynamic stimulations.

Methods: Skin blood flow in the dorsum of the hand was measured at baseline and after cold test and post-occlusive hyperemia test in 56 consecutive subjects affected by Raynaud's phenomenon (RP), 20 primary (PRP) and 36 secondary to systemic sclerosis (SSc). Twenty healthy subjects (HS) were studied as controls.

When the heart is burning: amino-terminal pro-brain natriuretic peptide as an early marker of cardiac involvement in active autoimmune rheumatic disease.

Background: We evaluated the influence of inflammation on cardiac endocrine function in autoimmune rheumatic disease (RD) patients with preserved left ventricular systolic function.

Methods: 160 consecutive RD patients (29 males, age 55 ± 14 years, left ventricular ejection fraction, LVEF, 63 ± 5%: inflammatory polyarthritis: 13%, systemic sclerosis: 25%, connective tissue diseases: 39%, systemic vasculitides: 23%) and 120 healthy controls (24 males, 55 ± 10 years) underwent clinical, echocardiographic evaluation and blood sampling for erythrocyte sedimentation rate, C-reactive protein (CRP), fibrinogen and plasma NT-proBNP.

Results: A significant correlation was found between plasma NT-proBNP and inflammatory markers (all p<0.

Thyroid cancer in systemic lupus erythematosus: a case-control study.

Context: Although advances in treatment have permitted patients with systemic lupus erythematosus (SLE) to live longer, the rates of several types of cancers in these patients appear to be increasing.

Objective: We used a prospective study to investigate the prevalence and features of thyroid cancer in SLE patients.

Design And Patients: The prevalence of thyroid cancer in 153 unselected SLE patients was compared with that in two population-based, gender- and age-matched control groups: 1) 459 subjects from an iodine-deficient area (iodine-deficient control) and 2) 459 subjects from an iodine-sufficient area (iodine-sufficient control).

Vascular reactivity in patients with undifferentiated connective tissue diseases.

Introduction: Endothelial dysfunction is an early event in the development of atherosclerotic disease. We investigated endothelial function in the peripheral circulation of patients with undifferentiated connective tissue diseases (UCTDs), in comparison to healthy controls.

Methods: In 15 young UCTD patients (mean age 39 years) with inactive disease and without cardiovascular risk factors and in 15 age-matched controls we evaluated endothelial function in the brachial conduit artery and in the forearm microcirculation.

Occurrence of organ-specific and systemic autoimmune diseases among the first- and second-degree relatives of Caucasian patients with connective tissue diseases: report of data obtained through direct patient interviews.

Studies have demonstrated a familial aggregation of systemic and organ-specific autoimmune diseases. The aim of the present survey was to obtain, by patient interviews, a preliminary estimate of the prevalence of systemic and organ-specific autoimmune diseases among the first- and second-degree relatives of Caucasian patients with connective tissue diseases (CTD) or inflammatory arthritis followed at our unit. Between June 2007 and January 2008, 626 patients and 85 controls (patients with osteoarthritis, osteoporosis, or fibromyalgia) were interviewed.