Systemic sclerosis sine scleroderma in children is more aggressive than in adults.

OBJECTIVES: To compare the clinical and laboratory features of pediatric systemic sclerosis sine scleroderma (ssJSSc) with adult-onset ssSSc. METHODS: Demographic, clinical and laboratory data of ssJSSc, retrospectively retrieved from our hospital medical records, case reports from the literature and from the PRES JSSc registry, were compared with the Padua cohort of adult patients with ssSSc. Patients were defined as having ssSSc if they never had skin involvement but all the following features: (I) Raynaud's Phenomenon (RP) and/or digital vasculopathy, (II) positive antinuclear antibodies (ANA), (III) internal organs involvement typical of scleroderma, (IV) no other defined connective tissue diseases. RESULTS: Eighteen juvenile and 38 adult-onset ssSSc patients, mean disease duration 5.8 and 9.7 years, respectively, entered the study. The frequency of females affected was significantly lower in ssJSSc (38.9% vs 89.5%, p < 0.0001). When compared to adults, ssJSSc displayed less SSc-specific capillaroscopy abnormalities (68.8% vs 94.7%, p = 0.02) while significantly higher vascular (digital pitting scars, ulcers 35.3% vs 10.5%, p = 0.042), respiratory (50.0% vs 23.7%, p = 0.02) and cardiac involvement (50.0% vs 2.6%, p < 0.0001). The outcome was significantly worse in ssJSSc as six patients (33%) died (n = 3) or reached an end-stage organ failure (n = 3) in comparison to only two deaths (5.3%) in the adult cohort. Anti-centromere antibodies were significantly lower in children (20.0% vs 68.4%, p = 0.001) while no difference was noted for other SSc-specific autoantibodies. CONCLUSION: Compared to adults where ssSSc generally has an indolent course, children present with aggressive disease that heralds a worse prognosis characterized by high cardiorespiratory morbidity and mortality.Key Indexing Terms: scleroderma, juvenile systemic sclerosis, outcome, heart, pulmonary arterial.

Is artificial intelligence ready to replace specialist doctors entirely? ENT specialists vs ChatGPT: 1-0, ball at the center.

PURPOSE: The purpose of this study is to evaluate ChatGPT's responses to Ear, Nose and Throat (ENT) clinical cases and compare them with the responses of ENT specialists. METHODS: We have hypothesized 10 scenarios, based on ENT daily experience, with the same primary symptom. We have constructed 20 clinical cases, 2 for each scenario. We described them to 3 ENT specialists and ChatGPT. The difficulty of the clinical cases was assessed by the 5 ENT authors of this article. The responses of ChatGPT were evaluated by the 5 ENT authors of this article for correctness and consistency with the responses of the 3 ENT experts. To verify the stability of ChatGPT's responses, we conducted the searches, always from the same account, for 5 consecutive days. RESULTS: Among the 20 cases, 8 were rated as low complexity, 6 as moderate complexity and 6 as high complexity. The overall mean correctness and consistency score of ChatGPT responses was 3.80 (SD 1.02) and 2.89 (SD 1.24), respectively. We did not find a statistically significant difference in the average ChatGPT correctness and coherence score according to case complexity. The total intraclass correlation coefficient (ICC) for the stability of the correctness and consistency of ChatGPT was 0.763 (95% confidence interval [CI] 0.553-0.895) and 0.837 (95% CI 0.689-0.927), respectively. CONCLUSIONS: Our results revealed the potential usefulness of ChatGPT in ENT diagnosis. The instability in responses and the inability to recognise certain clinical elements are its main limitations.

Impact of the COVID-19 pandemic on head and neck cancer diagnosis: data from a single referral center, South Tyrol, northern Italy.

OBJECTIVE: The aim of the study was to evaluate the impact of the COVID-19 pandemic on new diagnoses of head and neck cancer (HNC) in South Tyrol, northern Italy in terms of the number of new diagnoses and worsening disease stage due to diagnostic delay. METHODS: Patients were divided into two groups: the control group with a first diagnosis of HNC in 10 months before the national lockdown (March 9th, 2020) and the study group with a first diagnosis of HNC in 10 months after lockdown. RESULTS: A total of 124 patients were included in the study. Before the spread of COVID-19, 79 new diagnoses of HNCs were registered, while in the period after the lockdown, 45 new cancers cases were diagnosed and the difference was statistically significant (p = 0.01278). Early clinical T-stage results showed 52 cases in the control group and 21 in the study group, again with a significant difference (p = 0.03711). Advanced T-stage results showed 27 cases in the control group and 24 in the study group. CONCLUSIONS: This study highlights the impact of the COVID-19 pandemic on HNCs, showing a statistically significant difference in the number of diagnoses before and after the lockdown which was related to the spread of the SARS-CoV-2 virus, and with a relevant decrease in early cT-staged HNCs.

Mycophenolate mofetil for methotrexate-resistant juvenile localized scleroderma.

OBJECTIVES: To investigate safety and efficacy of MMF in patients with severe or MTX-refractory juvenile localized scleroderma. METHODS: Consecutive juvenile localized scleroderma patients undergoing systemic treatment were included in a retrospective longitudinal study. Patients treated with MMF because they were refractory or intolerant to MTX (MMF-group) were compared with responders to MTX (MTX-group). Disease activity was assessed by Localized Scleroderma Cutaneous Assessment Tool and thermography. Disease course was established on the number of relapses and treatment changes. Relapse-free survival was examined by Kaplan-Meier analysis. RESULTS: MMF and MTX groups included 22 and 47 patients, respectively. No significant difference in demographics, follow-up duration and treatment before diagnosis was observed between groups. The most represented clinical subtypes in the MMF-group were pansclerotic morphea and mixed subtype (P = 0.008 and P = 0.029, respectively), and linear scleroderma of the face in the MTX-group (P = 0.048). MMF was started because of MTX resistance (18 patients), relapse during MTX tapering/withdrawal (3 patients) and anaphylaxis to MTX (1 patient). After mean 9.4 years of follow-up, 90.9% of patients on MMF and 100% of those on MTX had inactive disease. No significant difference in relapse-free survival between the groups was found (P = 0.066, log-rank test), although MMF likely induced more persistent remission. MMF was well tolerated and combination of MMF and MTX did not increase its efficacy. CONCLUSION: The present study adds strong evidence on the efficacy and tolerance of MMF in severe and/or MTX-refractory juvenile localized scleroderma. Further controlled studies are needed to prove its efficacy as first line treatment.

Osteoperiostitis in children: proposal for a diagnostic algorithm.

Juvenile osteoperiostites (JOP) are a group of inflammatory bone diseases whose differential diagnosis is often difficult. The main conditions are acute osteomyelitis (AOM), chronic non-bacterial osteomyelitis (CNO) and the Goldbloom syndrome (GS). The study was aimed to develop an algorithm to enable an early diagnosis of JOP. Clinical records of patients with AOM, CNO and GS, followed at our Center over the past 10 years, were reviewed. Twelve additional patients with GS were selected from PubMed/MEDLINE literature search. Data collected included demographics, clinical manifestations, laboratory and instrumental investigations at disease onset. The association between categorical variables was investigated, and the segmentation of patients with different diagnoses was analyzed through a classification tree model (CTREE package) in order to build up a diagnostic algorithm. Ninety-two patients (33 CNO, 44 AOM, 15 GS) entered the study. Among 30 variables considered at onset, nine (age at onset, fever, weight loss, symmetry, focality, functional limitation, anemia, elevated ESR, CRP) resulted statistically significant in differentiating the three clinical entities from each other and were chosen to build up a decisional tree. Three variables, symmetry of bone involvement, presence of fever and age at disease onset, resulted significant to discriminate each of the three diseases from the others. The performance of the diagnostic algorithm was validated by comparing the diagnoses provided by the model with the real diagnoses and showed 85.9% accuracy.Conclusion: We propose a diagnostic algorithm, based on simple clinical data, which can help guide a prompt and appropriate diagnosis of JOP. What is Known: • Juvenile osteoperiostitis (JOP) are a group of inflammatory bone diseases followed by various pediatric specialists. • The distinction between these conditions is not easy as clinical and laboratory features often overlap. What is New: • We propose a diagnostic algorithm, based on clinical data of real patients, with high degree accuracy. • This instrument can help guide the prompt and appropriate diagnosis of JOP.

Application of an adapted relationship scale for assessing the occurrence of six different relationships as perceived by seven health care professions in Northern Italy.

Interprofessional working relationships can influence the quality of collaborative practices, with consequences for patient safety outcomes. This article reports findings of an adapted relationship scale comprising six different relationship types, ranging from hostile to collegial, between seven health-care professions: physicians, nurses, dieticians, occupational therapists, physiotherapists, speech therapists, and psychologists. Survey data were gathered within amixed-method study aiming to explore the current status quo of interprofessional collaboration in aHealth Trust, located in Northern Italy. An online questionnaire was completed by 2,238 health professionals achieving aresponse rate of 44%. The working relationship element was answered by 1,897 respondents. The results of the survey are used as abasis for recommending strategies for advancing interprofessional collaboration in the Trust. Descriptive statistics were used to examine relationship-types, with frequency of occurrence considered. The non-parametric Mann Whitney and Kruskal Wallis tests were used to explore relationship differences among groups defined by sociodemographic variables. Participants reported overall positive relationships with other health professions. We noted variability in the occurrence of different relationship-types amongst the health professions. In particular, the six professions viewed their relationships with doctors more negatively than physicians who reported amore positive perception of their relationships with the six professions.

Perceptions of collaborative relationships between seven different health care professions in Northern Italy.

This article presents quantitative findings from a mixed method study that aimed to explore the status quo of interprofessional collaboration (IPC) in a Health Trust, located in a trilingual region in Northern Italy. The survey targeted seven health professions (physicians, nurses, dieticians, occupational therapists, physiotherapists, speech therapists, and psychologists). The survey was distributed online to more than 5,000 health professionals and completed by 2,238. This paper presents results on the frequency of collaboration as well as data from a multiple-group measurement scale for assessing IPC. Descriptive statistics were calculated for continuous variables while categorical data were analysed as counts and percentages. Pearson's Chi-square test and Fisher's exact test were calculated while Mann Whitney and Kruskal Wallis tests were applied to analyse statistical differences in IPC between groups according to sociodemographic variables. In general, our survey data showed that participants, perceived IPC in a positive way, even if the analysis indicated heterogeneity in the level of collaboration expressed. We also found that not all professions had an opportunity to collaborate with others. In addition, we found evidence to suggest that the way health care is organised impacted on perceptions of collaboration between the health professionals in this study. This study provides an initial insight into the perceived levels of IPC within a North Italian context. As such, it offers an account of the strengths and weaknesses of IPC from seven different professional groups based in this region of Europe.

[Types and frequencies of complications associated with midline catheters and PICCs in a South Tyrolean district hospital: a retrospective cohort study]. / Arten und Häufigkeiten von Komplikationen bei Midline-Kathetern und PICCs in einem Südtiroler Bezirkskrankenhaus ­ eine retrospektive Kohortenstudie.

Types and frequencies of complications associated with midline catheters and PICCs in a South Tyrolean district hospital: a retrospective cohort study Abstract. BACKGROUND: Specialized registered nurses play a key role in the insertion and management of peripherally inserted central catheters (PICCs) and midline catheters in Anglo-Saxon countries. From the German-speaking area no data on the use of PICCs and midline catheters are available. AIM: The aim of this study was to describe the types and frequencies of complications of PICCs and midline catheters which were inserted by specialized registered nurses in a South Tyrolean district hospital. METHODS: We performed a retrospective cohort study of PICCs and midline catheters inserted between 2013 and 2015 in one surgical unit in the district hospital Bruneck (South Tyrol / Italy). RESULTS: Data from 900 catheters (421 midline catheters and 479 PICCs) inserted in 686 adult patients (404 women, 282 men) were analysed. The cumulative incidence was 29.2 % complications for midline catheters (incidence rate: 13 complications / 1000 catheter days) and 16.0 % for PICCs (incidence rate: 3 complications / 1000 catheter days). The most frequent complication was the removal of the catheter by the patients (PICCs: 6.7 %, midline catheters: 15.7 %). Other less frequent complications were mechanical complications, occlusions, infections and thromboses. CONCLUSIONS: This study in one district hospital revealed similar types and frequencies of complications as previous international studies. Specialized and clinically competent nurses in German-speaking countries could develop advanced roles in the insertion and management of PICCs and midline catheters.

A multiple-group measurement scale for interprofessional collaboration: Adaptation and validation into Italian and German languages.

This article presents a study that aimed to validate a translation of a multiple-group measurement scale for interprofessional collaboration (IPC). We used survey data gathered over a three month period as part of a mixed methods study that explored the nature of IPC in Northern Italy. Following a translation from English into Italian and German the survey was distributed online to over 5,000 health professionals (dieticians, nurses, occupational therapists, physicians, physiotherapists, speech therapists and psychologists) based in one regional health trust. In total, 2,238 different health professions completed the survey. Based on the original scale, three principal components were extracted and confirmed as relevant factors for IPC (communication, accommodation and isolation). A confirmatory analysis (3-factor model) was applied to the data of physicians and nurses by language group. In conclusion, the validation of the German and Italian IPC scale has provided an instrument of acceptable reliability and validity for the assessment of IPC involving physicians and nurses.

Joint hypermobility and oligoarticular juvenile idiopathic arthritis: What relationship?

AIM: 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. METHODS: 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. RESULTS: 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). CONCLUSION: In patients with oligoarticular JIA, JH is more frequent than in healthy subjects, uveitis less frequent and the long-term outcome better.

Frequency, type and treatment of fungal pathogens in toenail onychomycosis in the central Alpine region of South Tyrol, northern Italy - a 10-year retrospective study from 2004 to 2013.

Onychomycosis is one of the most common dermatological diseases, primarily caused by dermatophytes, and sometimes, also by yeast and moulds. This study aims to clarify the frequency of isolated pathogens from mycotic toenails in the reference centre of the central Alpine mountain region of South Tyrol, northern Italy. Age and gender differences, types of pathogens and initial therapy used were also investigated. This is a retrospective study from 2004 to 2013 on the files of all patients with suspected onychomycosis in whom a cultural pathogen detection was performed. A total of 1565 patients were studied (943 women; 622 men), with a median age of 55 years. Fungal pathogens could be detected by culture in 49.5%. Significantly more pathogens could be grown in men (63.8%) than in women (39.9%, P < 0.0001). The most common pathogens grown were dermatophytes (53.3%), especially T. rubrum and T. interdigitale, followed by moulds (24.7%) and yeast (22%). Therapies prescribed were topical in 64.2% and systemic in 35.8% of the cases. Topical treatment was more frequent in older patients (P < 0.05). In northern Italy, dermatophytes are the most common causative agents for onychomycosis. Fungal pathogens were more commonly found in men than in women and topical treatment was more frequent in older patients.

The state of interprofessional collaboration in Northern Italy: a mixed methods study.

Health care systems are facing continual reorganizations in response to scientific and technological innovations as well as financial constraints. Simultaneously the rising prevalence of chronic diseases call for a kind of health care organization in which interprofessional collaboration (IPC) functions on a high level. This article describes a research project that aims to generate an empirical account of the current state of IPC in the South Tyrolean Health Trust, located in a bilingual region in northern Italy. The study will employ a sequential mixed methods' design. A survey will be followed by individual and focus group interviews. An innovative aspect of the study is that it will include participants from six different health professions (dieticians, nurses, occupational therapists, physicians, physiotherapists, and speech therapists). Qualitative data analysis will draw on the negotiated order/structural ordering approach and on the social world/arena perspective. The results are expected to generate specific recommendations to improve IPC in the South Tyrolean Health Trust and inform further research.

Tonsillectomy efficacy in children with PFAPA syndrome is comparable to the standard medical treatment: a long-term observational study.

OBJECTIVES: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: In a large cohort of patients with PFAPA syndrome, tonsillectomy efficacy was comparable to the standard medical treatment.

Joint hypermobility, growing pain and obesity are mutually exclusive as causes of musculoskeletal pain in schoolchildren.

OBJECTIVES: Chronic musculoskeletal pain (MSP) is common in children and can be due to several non-inflammatory conditions such as the benign joint hypermobility syndrome (BJHS), and growing pains (GP). We evaluated frequency, risk factors and causes of MSP in a large cohort of healthy schoolchildren. METHODS: We conducted a cross sectional study in a cohort of healthy schoolchildren, aged 8-13 years, by collecting information and performing a physical examination. The anamnesis was focused on family history for MSP, presence and sites of MSP interfering with the regular daily activities during the previous 6 months and presence of GP. Physical examination included body mass index, pubertal stage and musculoskeletal examination focused on the presence of hypermobility according to the Beighton criteria. RESULTS: Two hundred and eighty-nine schoolchildren, 143 females and 146 males, participated in the study. Chronic MSP occurred in 30.4% of subjects, BJHS occurred in 13.2%. GJH was more frequent in symptomatic subjects than in asymptomatic ones (p=0.054). Symptomatic subjects were more frequently pre-pubertal than pubertal (p=0.006). In general, GP, BJHS and obesity (OB) were mutually exclusive as causes of MSP as, among 88 symptomatic subjects, 52.3% had GP, 40.9% presented BJHS, 4.5% were OB and only two (2.3%) presented both BJHS and OB. After puberty, GP persisted in 66.7%, BJHS in 26.7% and in association with OB in 6.7%. CONCLUSIONS: Approximately one third of schoolchildren suffer from MSP. BJHS, GP and OB are mutually exclusive as causes of MSP in schoolchildren. Pubertal stage plays an important role in the physiopathology of this condition.

Clinical overview and outcome in a cohort of children with polyarteritis nodosa.

OBJECTIVES: 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). CONCLUSIONS: 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.

A preliminary disease severity score for juvenile systemic sclerosis.

OBJECTIVE: To develop a preliminary disease severity score for juvenile systemic sclerosis (SSc). METHODS: We conducted an evidence- and consensus-based study that included the following 5 phases: 1) prospective collection of data regarding the demographic and clinical characteristics of patients with diffuse juvenile SSc who were followed up for at least 4 years or until death; 2) blinded evaluation of the disease course profiles of these patients by experts in juvenile SSc, so that patient profiles with a defined clinical course could be used as the gold standard for the score validation phase; 3) definition of candidate severity indices to be included in potential scores; 4) selection of the pediatric severity score with the best statistical performance, as determined by its ability to classify individual patients as having improvement or worsening of disease compared with baseline values or the previous evaluation; 5) validation of the efficiency of the selected score in patients with a mild, moderate, or severe disease course and comparison with the Medsger severity score for adults with SSc. RESULTS: Thirty-five patients classified as having a mild (n = 17), moderate (n = 10), or severe (n = 8) disease course entered the study. The selected pediatric SSc score, defined as the Juvenile Systemic Sclerosis Severity Score (J4S), included indices of 9 organ systems each scored on a scale of 0-4. To weight the importance of the involvement of different organ systems, a coefficient of severity was introduced. Compared with the modified Medsger severity score, the J4S performed significantly better in detecting change in severity, both in patients with a moderate disease course (0.89 versus 0.52) and in patients with a severe disease course (0.82 versus 0.75). CONCLUSION: The J4S is a valid instrument to assess disease severity in juvenile SSc.

A Comprehensive Examination of Vegan Lifestyle in Italy.

The popularity of veganism and plant-based diets is rapidly increasing worldwide, including in Italy, where more individuals and families are adopting this lifestyle. However, medical and health professionals often lack the necessary knowledge and are skeptical about this diet despite the scientific evidence. It is important for them to provide support and expertise to those following this diet. The survey evaluated various aspects of the lifestyle of Italian vegans living in Italy and abroad, including food frequency, vitamin and mineral supplementation, relationship with medical and health professionals, and perceived difficulties in daily life. The emphasis was on potentially critical aspects for those following this dietary choice. A cross-sectional survey was conducted in Italy between March and April 2022. A questionnaire was distributed through social media platforms such as Instagram, Facebook, and Telegram, and 2180 Italian adults who follow a vegan diet completed it. The survey found that most of the vegan population surveyed were female, showed a greater sensitivity to ethical issues, were aware of the need for vitamin B12 supplementation, and followed healthy-eating guidelines. It is evident that despite the increasing popularity of plant-based diets, many medical and health professionals remain cautious and hesitant to recommend them.

Strategic use of levofolinic acid for methotrexate-induced side effects in juvenile idiopathic arthritis: a prospective observational study.

OBJECTIVE: To evaluate the efficacy of levofolinic acid (LVF) administered 48 h before methotrexate (MTX) in reducing gastrointestinal side effects without interference with drug efficacy. METHODS: A prospective observational study was performed including patients with Juvenile Idiopathic Arthritis (JIA) reporting significant gastrointestinal discomfort after MTX despite taking a dose of LVF 48 h after MTX. Patients with anticipatory symptoms were excluded. A LVF supplemental dose was added 48 h before MTX and patients were followed every 3-4 months. At each visit data on gastrointestinal symptoms, disease activity (JADAS, ESR, CRP values) and treatment changes were collected. Friedman test for repeated measures analyzed differences between these variables over time. RESULTS: Twenty-one patients were recruited and followed for at least 12 months. All patients received MTX subcutaneously (mean 9.54 mg/m2) and LVF 48 h before and after MTX (mean 6.5 mg/dose), 7 received a biological agent too. Complete remission of gastrointestinal side effects was reported in 61.9% of study patients at first visit (T1) and increased over time (85.7%, 95.2%, 85.7% and 100% at T2, T3, T4, T5, respectively). MTX efficacy was maintained as showed by significant reduction of JADAS and CRP (p = 0.006 and 0.008) from T1 to T4 and it was withdrawn for remission in 7/21. CONCLUSIONS: LVF given 48 h before MTX significantly reduced gastrointestinal side effects and did not reduce drug's efficacy. Our results suggest that this strategy may improve compliance and quality of life in patients with JIA and other rheumatic diseases treated with MTX.

Prognostic role of euthyroid sick syndrome in MIS-C: results from a single-center observational study.

Background: Euthyroid sick syndrome (ESS) is characterized by low serum levels of free triiodothyronine (fT3) with normal or low levels of thyroid stimulating hormone (TSH) and free thyroxine (fT4) and is reported in different acute clinical situations, such as sepsis, diabetic ketoacidosis and after cardiac surgery. Our aim was to evaluate the predicting role of ESS for disease severity in patients with Multisystem Inflammatory Syndrome in children (MIS-C). Methods: A single-centre observational study on consecutive patients with MIS-C. Before treatment clinical, and laboratory data were collected and, in a subset of patients, thyroid function tests were repeated 4 weeks later. Variables distribution was analyzed by Mann-Whitney U-test and correlations between different parameters were calculated by Spearman's Rho coefficient. Results: Forty-two patients were included and 36 (85.7%) presented ESS. fT3 values were significantly lower in patients requiring intensive care, a strong direct correlation was shown between fT3 and Hb, platelet count and ejection fraction values. A significant inverse correlation was retrieved between fT3 levels and C-reactive protein, brain natriuretic peptide, IL-2 soluble receptor and S-100 protein. Subjects with severe myocardial depression (EF < 45%) had lower fT3 values than subjects with higher EF. The thyroid function tests spontaneously normalized in all subjects who repeated measurement 4 weeks after admission. Conclusion: ESS is a frequent and transient condition in acute phase of MIS-C. A severe reduction of fT3 must be considered as important prognostic factor for severe disease course, with subsequent relevant clinical impact in the management of these patients.

Monoarticular juvenile idiopathic arthritis as a distinct clinical entity A proof-of-concept study.

BACKGROUND: Currently, monoarticular Juvenile Idiopathic Arthritis (monoJIA) is included in the ILAR classification as oligoarticular subtype although various aspects, from clinical practice, suggest it as a separate entity. OBJECTIVES: To describe the clinical characteristics of persistent monoJIA. METHODS: Patients with oligoJIA and with at least two years follow-up entered the study. Those with monoarticular onset and persistent monoarticular course were compared with those with oligoJIA. Variables considered were: sex, age at onset, presence of benign joint hypermobility (BJH), ANA, uveitis, therapy and outcome. Patients who had not undergone clinical follow-up for more than 12 months were contacted by structured telephone interview. RESULTS: Of 347 patients with oligoJIA, 196 with monoarticular onset entered the study and 118 (60.2%), identified as persistent monoJIA, were compared with 229 oligoJIA. The mean follow-up was 11.4 years. The switch from monoarticular onset to oligoarticular course of 78 patients (38.8%) occurred by the first three years from onset. In comparison with oligoJIA, the most significant features of monoJIA were later age at onset (6.1 vs. 4.7 years), lower female prevalence (70.3 vs. 83.4%), higher frequency of BJH (61.9 vs. 46.3%), lower frequency of uveitis (14.4 vs. 34.1%) and ANA+ (68.6 vs. 89.5%) and better long-term outcome. CONCLUSIONS: MonoJIA, defined as persistent arthritis of unknown origin of a single joint for at least three years, seems to be a separate clinical entity from oligoJIA. This evidence may be taken into consideration for its possible inclusion into the new classification criteria for JIA and open new therapeutic perspectives.