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Purpose Evidence of an increased diagnostic pressure on thyroid has emerged over the past decades. This study aimed to provide estimates of a wide spectrum of surveillance indicators for thyroid dysfunctions and diseases in Italy. Methods A population-based study was conducted in North-eastern Italy, including 11.7 million residents (20% of the total Italian population). Prescriptions for TSH testing, neck ultrasound or thyroid fine needle aspiration (FNA), surgical procedures, and drugs for hypo- or hyperthyroidism were extracted from regional health databases. Proportions and rates of selected examinations were calculated from 2010 to 2017, overall and by sex, calendar years, age, and region. Results Between 2010 and 2017 in North-eastern Italy, 24.5% of women and 9.8% of men received at least one TSH test yearly. In 2017, 7.1% of women and 1.5% of men were prescribed drugs for thyroid dysfunction, 94.6% of whom for hypothyroidism. Neck ultrasound examinations were performed yearly in 6.9% of women and 4.6% of men, with a nearly two-fold variation between areas. Thyroid FNA and thyroidectomies were three-fold more frequent in women (394 and 85 per 100,000) than in men (128 and 29 per 100,000) with a marked variation between areas. Both procedures decreased consistently after 2013. Conclusions The results of this population-based study describe recent variations over time and between surrounding areas of indicators of ‘diagnostic pressure’ on thyroid in North-eastern Italy. These results emphasize the need to harmonize practices and to reduce some procedures (e.g., neck ultrasound and total thyroidectomies) in certain areas.

Gastrointestinal stromal tumors (GISTs), tumors characterized by c-KIT mutations, are the most frequent mesenchymal tumors of the digestive tract. The stomach is the most commonly involved site. Localization, size and mitotic rate are reliable predictors of survival and the two milestones of GISTs treatment are surgery and imatinib. This article is aimed to report the data of an audit, carried out on the morphological and clinical aspects of the disease and to review the present knowledge on GISTs. A total of 172 patients with GISTs (M : F=1 : 1; mean age 65 years) were recruited. The stomach was the most frequently involved site. In 50% of the cases the tumor was smaller than 5 cm, whereas major symptoms were observed in 43% of the cases. Predictors of progressive disease were present only in a small percentage of cases but the disease was in the metastatic phase in over 25% of the cases at diagnosis. Familial aggregation was rare but a consistent share of the patients (21%) had other synchronous or metachronous cancers. The most frequent mutations were in-frame deletions and point mutations of c-KIT exon 11. This report confirms in part the available data on GIST in a consecutive series of patients recruited in Italy and shows that only large collaborative multicenter studies provide data sound enough to enable making reasonable clinical and therapeutic choices, and suggests that, as a measure of secondary prevention, a diagnostic definition should be obtained in all submucosal lesions of the GI tract and that GIST patients should be screened for second tumors.

Objective Juvenile localized scleroderma is a chronic progressive fibrotic disorder of the skin that causes permanent disability and aesthetic damage. This study was undertaken to assess the safety and efficacy of methotrexate (MTX) in the treatment of juvenile localized scleroderma. Methods In this double-blind study, patients with active juvenile localized scleroderma were randomized (2:1) to receive oral MTX (15 mg/m 2, maximum 20 mg) or placebo once weekly, for 12 months or until treatment failure. Both groups received oral prednisone (1 mg/kg/day, maximum 50 mg) for the first 3 months. A target lesion was evaluated clinically, with infrared thermography and using a computerized scoring system with skin score rate (SSR) evaluation. Response to treatment was defined as the absence of new lesions, SSR â1, and a decrease in lesion temperature of at least 10% compared to baseline. Treatment failure was defined as the occurrence of new lesions, SSR >1, or increased lesion temperature. All analyses were done on the intent-to-treat population. Results Of the 85 patients screened, 70 (ages 6-17 years) were randomized (46 to the MTX group, 24 to the placebo group). The mean disease duration was 2.3 years. After an initial response in all patients, disease relapsed in 15 MTX-treated patients (32.6%) and 17 placebo-treated patients (70.8%) (P ;lt& 0.005). New lesions appeared in 3 MTX-treated patients (6.5%) versus 4 placebo-treated patients (16.7%). The mean SSR decreased from 1 to 0.79 in the MTX group and increased from 1 to 1.1 in the placebo group, and the mean target lesion temperature decreased by 44.4% in the MTX group versus 12.1% in the placebo group. Twenty-six patients in the MTX group (56.5%) and 11 patients in the placebo group (45.8%) developed mild side effects related to treatment. None of the side effects were severe enough to necessitate treatment discontinuation. Conclusion Our findings indicate that MTX is efficacious in the treatment of juvenile localized scleroderma and is well tolerated. Copyright © 2011 by the American College of Rheumatology.

Objectives. To determine whether demographic, clinical and immunological features may predict the outcome in juvenile SSc (JSSc).Methods. Clinical and laboratory characteristics of patients with JSSc collected from paediatric rheumatology centres worldwide were analysed. First, univariate tests identified those features significantly related with fatal outcome, and then multivariate logistic regression analysis was applied to determine the predictors of mortality.Results. One hundred and thirty-four patients from 40 centres were eligible for the analysis. Sixteen patients died and a rapidly fatal course was observed in most of them: 4/16 died within 1 yr after diagnosis and 10/16 within 5 yrs. At the moment of diagnosis, patients with poor outcome showed a significantly higher frequency of internal organ involvement, particularly cardiac, respiratory and gastrointestinal systems. No significant difference emerged for entity of skin, vascular and musculo-skeletal involvement, nor for auto-antibodies profile and laboratory tests. Multivariate analysis showed the following factors to be significant predictors of mortality: fibrosis on chest X-rays [odds ratio (OR) 11.2], raised creatinine levels (OR 22.7) and pericarditis (OR 41.3), while a short disease duration at diagnosis conferred protection (OR 0.3).Conclusions. All patients with JSSc and fatal outcome were affected by the diffuse form of the disease, and most of them showed a very rapid progression and early signs of internal organ involvement. This suggests that, in children, SSc may have two possible courses: a rapid development of internal organ failure leading to severe disability and eventually to death, or a slow course of the disease with lower mortality. Univ Padua, Dept Paediat, I-35128 Padua, Italy Cerrahpasa Tip Fak, Dept Pediat, Istanbul, Turkey Fdn IRCCS Policlin S Matteo, Dept Pediat, Pavia, Italy Clin Pediat II Marchi Milan, Milan, Italy Inst Nacl Rehabil Serv Reumatol, Mexico City, DF, Mexico First Fac Med, Prague, Czech Republic Gen Fac Hosp, Prague, Czech Republic Universidade Federal de São Paulo, Dept Pediat, São Paulo, Brazil Unidad Reumatol Hosp Sor Maria Ludov, La Plata, Argentina Univ Copenhagen Hosp, DK-2100 Copenhagen, Denmark Universidade Federal de São Paulo, Dept Pediat, São Paulo, Brazil Web of Science

Objective. Up to now, no validated tools are in use for the assessment of the skin lesions in localized scleroderma (LS). The aim of this study is to evaluate the performance of a new computerized skin score (CSS) method for the measurement of circumscribed lesions in LS. Methods. The study consisted of three phases: set up of the CSS technique, measurement of target lesions of LS patients, assessment of intra- and inter-rater reliability. The CSS technique consists in delimitating the indurate lesion on an adhesive transparent film, transferring it over a cardboard and then calculating the affected area with a specifically created software. The technique was explained to a panel of 10 physicians with different expertise in LS (three paediatric rheumatologists, two dermatologists, five paediatric residents). All participants, singularly and blindly to the others, examined 10 consecutive patients twice after a time interval of at least 6h. The intra-observer variability was evaluated by the repeatability coefficient and the inter-rater reliability by the intra-class correlation coefficient (ICC). Results. The repeatability coefficients were good, ranging between 1.90 and 7.03. The mean values of skin scores were not significantly different among the examiners. The ICC for indurate area calculation were high in both the experts (0.97) and the residents (0.91–0.94). Conclusions. CSS has shown to be a reliable method to assess the skin lesions in patients with LS. It is reproducible, easy to use and, with the support of the CSS software, applicable worldwide.

Background Recent studies report that methotrexate (MTX) is beneficial in the treatment of juvenile localized scleroderma (JLS) but little is known about its long-term effectiveness. Objective We assessed the therapeutic role of MTX in children with JLS who were followed up for a prolonged period. Methods A cohort of patients with JLS, previously enrolled in a double-blind, randomized controlled trial and treated with oral MTX (15 mg/m 2 /wk) and prednisone (1 mg/kg/d, maximum 50 mg) for the first 3 months, were prospectively followed up. Lesions were evaluated clinically, with infrared thermography, and by a computerized skin score. Response to treatment was defined as: (1) no new lesions; (2) skin score rate less than 1; and (3) decrease in lesion temperature by at least 10% compared with baseline. Clinical remission (CR) on medication was defined when response was maintained, on treatment, for at least 6 months, and complete CR when response was maintained, without treatment, for at least 6 months. Results Of 65 patients treated with MTX, 48 (73.8%) were responders, 10 (15.4%) relapsed by 24 months since MTX start, and 7 (10.8%) were lost to follow-up. Among the responders, 35 (72.9%) maintained CR for a mean of 25 months and 13 (27.1%) were in CR on medication. Adverse effects seen in 28 patients (48.3%) were generally mild and never required treatment discontinuation. Limitations The use of objective measures not widely available, such as infrared thermography and computerized skin score, makes it difficult to compare data from previous studies. Conclusions Long-term MTX therapy is beneficial and well tolerated for JLS.

Objective.To evaluate safety and efficacy of adalimumab (ADA) and infliximab (IFX) for the treatment of juvenile idiopathic arthritis-related anterior uveitis (JIA-AU).Methods.Starting January 2007, patients with JIA-AU treated with IFX and ADA were managed by a standard protocol and data were entered into the National Italian Registry (NIR). At baseline, all patients were refractory to standard immunosuppressive treatment and/or were corticosteroid-dependent. Data recorded every 3 months included uveitis course, number/type of ocular complications, drug-related adverse events (AE), treatment change or withdrawal, and laboratory measures. Data of patients treated for at least 1 year were retrieved from the NIR and analyzed using descriptive statistics. Treatment efficacy was based on change in uveitis course and in number of ocular complications.Results.Up to December 2009, data for 108 patients with JIA-AU treated with anti-tumor necrosis factor-α agents were recorded in the NIR and data from 91, with at least 12 months’ followup, were included in the study. Forty-eight patients were treated with IFX, 43 with ADA. Forty-seven patients (55.3%) achieved remission of AU, 28 (32.9%) had recurrent AU, and 10 (11.8%) maintained a chronic course. A higher remission rate was observed with ADA (67.4% vs 42.8% with IFX; p = 0.025). Ocular complications decreased from 0.47 to 0.32 per subject. Five patients experienced resolution of structural complications. No patient reported serious AE; 8 (8.8%) experienced 11 minor AE (9 with IFX, 2 with ADA).Conclusion.IFX and ADA appear to be effective and safe for treatment of refractory JIA-related uveitis, with a better performance of ADA in the medium-term period.

Purpose: Aim of the present study was to validate a statistical model to predict a severe course of anterior uveitis (AU) in patients with juvenile idiopathic arthritis (JIA). Methods: Consecutive patients with newly diagnosed uveitis have been followed for at least 1 year with a standardized protocol. For each patient, demographic, clinical and laboratory characteristics, including time interval between arthritis and uveitis onset, α2-globulins level at arthritis onset, number of uveitis relapses/year, ocular complications and therapy and visual acuity, have reported. The validation procedure included the assessment of sensitivity, specificity and efficiency of previously published statistical model (Zulian et al. J Rheumatol 2002; 29: 2446–2453) in a new inception cohort of patients during a short length follow-up. Results: Sixty patients with JIA, followed at 14 paediatric rheumatology–ophthalmology centres in Italy, entered the study. The mean age at arthritis onset was 4.4 years (range 1.2–15.8 years), and the mean interval time between arthritis and uveitis onset was 1.8 years (range: 0.0–14.2 years). After the first AU, patients, followed for a mean of 3.2 years, had a mean of 2.9 uveitis relapses. Twenty-two patients (36.7%) presented at least one complication. Using a probability cut-off value = 0.7, the statistical model revealed 80% sensitivity, 58% specificity and 65% efficiency. Conclusion: The time interval between arthritis and uveitis onset resulted as the main predictor of severe course uveitis in JIA. The statistical model was able to predict the development of a severe course in 8 of 10 patients.