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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.

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.

Oligoarticular onset juvenile idiopathic arthritis (oJIA) is characterised by a prevalent lower limb involvement, antinuclear antibodies (ANA) positivity and high risk of anterior uveitis. As we observed that oJIA patients frequently present with joint hypermobility (JH), we investigated whether there was a relationship between oJIA and JH.Our series consisted of children with oJIA, as defined by the International League of Associations for Rheumatology criteria, for whom complete clinical data of at least 2 years' duration were available. Clinical and laboratory data, collected at disease onset and at the last follow-up, included: sex, age, presence of JH according to the Beighton score, disease activity, presence of uveitis, ANA, treatment and outcome.A total of 274 oligoarticular JIA patients (224 female, 50 male; mean age: 11.5) followed on average for 6.6 years, entered the study. The mean age at disease onset was 4.9 years, ANA were positive in 83.9% and uveitis occurred in 20.8%. JH was present in 70.8% of cases at onset, in 44.5% at the last evaluation. JH was more frequent in females (73.7%) than in males (58.0%) (P = 0.028). Uveitis was less frequent in hypermobile children both at diagnosis (17.5 vs. 28.7%, P = 0.037) and during overall disease course (23.7 vs. 36.3%, P = 0.034). Of 163 subjects with at least 5-year follow-up, the full clinical remission rate was significantly higher in JH patients (50.5%) than in those without JH (42.3%; P = 0.042).In patients with oligoarticular JIA, JH is more frequent than in healthy subjects, uveitis less frequent and the long-term outcome better.

Tonsillectomy has recently been suggested as an effective treatment for PFAPA syndrome but little is known about its long-term efficacy. We compared the clinical features and the long-term outcome of a large cohort of patients with PFAPA syndrome treated with tonsillectomy or with standard medical treatment.We conducted a retrospective study on patients with PFAPA syndrome followed at a tertiary care centre from January 1993 to August 2010. Clinical characteristics and laboratory parameters were evaluated at onset and during the follow-up. Disease outcomes of patients who underwent tonsillectomy and of those treated with medical therapy (NSAIDs, prednisone) were compared. Clinical remission on medication (CRM) was considered the persistence of fever attacks which were well controlled by medical therapy, clinical remission (CR) was defined as the absence of fever attacks, without any treatment, for more than 12 months.275 patients with PFAPA syndrome, 59.6% males, aged 27.9 months at onset and followed for mean 54.5 months, entered the study. CR was reported in 59.6% of the patients and was significantly less frequent in those with positive family history for PFAPA (46.4% vs. 66.1%, p=0.003). 27/41 patients (65.9%), responded to tonsillectomy and this result was comparable with that observed in those treated with medical therapy (59.1%, p=0.51). Disease duration, age at remission or presence of associated symptoms were not significantly different in both groups. No predictors of tonsillectomy failure were found.In a large cohort of patients with PFAPA syndrome, tonsillectomy efficacy was comparable to the standard medical treatment.

To analyse the use of complementary and alternative medicine (CAM) in children with rheumatic diseases, treated at a paediatric rheumatology centre in Italy.Parents of children with different kinds of chronic rheumatic diseases anonymously completed a questionnaire about their children's past or current use of CAM. Two groups of patients were analysed: Group A consisted of children who were still attending the centre; Group B consisted of children who had not attended the clinic for more than one year.150 completed surveys were analysed: 22 paediatric patients (14.7%), 10/100 in group A and 12/50 in group B, used CAM to treat their diseases. The therapies used the most were homeopathy, herbal remedies, vitamins and minerals. We observed a significantly greater use of CAM among patients who had not attended the clinic for more than one year (24%) as compared to those who were regularly checked (10%) (p=0.02). Parents' use of CAM was significantly related to its use for their children (p=0.001). A poor outcome, probably related to the exclusive use of alternative treatments, was observed in three out of six patients who had completely stopped using traditional immunosuppressive drugs.Physicians should be aware of the use of CAM particularly in patients who skip their regular check-ups. The use of CAM to treat childhood rheumatic conditions in Italy seems to be less frequent than in North America.

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.

Objective: Polyarteritis nodosa (PAN) is a rare vasculitis in childhood and poor information is known about its long-term outcome. Our aim was to describe the clinical features, at onset and during the disease course, of childhood- onset PAN and identify a potential correlation with persistent organ damage and worse outcome in a cohort of paediatric patients with a confirmed diagnosis of PAN. Methods: A retrospective collection of demographic and clinical data of 52 Caucasian children diagnosed with PAN, fulfilling the EULAR/PRES diagnostic criteria, recruited from eight paediatric rheumatologic centres and one transition unit, was performed. A statistical correlation was made between clinical involvement at onset or during the overall disease course and patients' final outcome. Results: Data from 52 patients (31 males, 21 females) were collected: their mean age at onset was 7.9 years (median 6.3) and mean follow-up period was 6.2 years (median 5.4). At the last follow-up visit, 27 patients (51.9%) were off therapy in clinical remission, 17 (32.7%) were in clinical remission while on medication, and 6 (11.6%) had a persistent or relapsing disease course. Two patients (3.8%) deceased because of severe cerebral involvement. Cranial nerve palsy during the disease course was significantly correlated with a worse prognosis (p=0.011). The presence of nephrogenic hypertension at onset and seizures during the disease course were significantly associated with the development of irreversible organ damage (p= 0.040 and 0.011, respectively). Conclusion: Childhood PAN is a severe disease with substantial risk of long-term morbidities. In our cohort of patients the worst outcome was significantly correlated with renal and neurological involvement. © Clinical and Experimental Rrheumatology 2014.

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.