Reumatismo <p>Official <em>Journal Of The Italian Society Of Rheumatology</em>. Founded In 1949.</p> <p><strong>Reumatismo</strong> is the Official Journal of the Italian Society of Rheumatology (SIR). It publishes Abstracts and Proceedings of Italian Congresses and original papers concerning rheumatology. Reumatismo is published quarterly and is sent free of charge to the Members of the SIR who regularly pay the annual fee. Those who are not Members of the SIR as well as Corporations and Institutions may also subscribe to the Journal.</p> <p>This journal does not apply the article processing charge&nbsp;to Authors as it is supported by institutional funds.</p> PAGEPress Scientific Publications, Pavia, Italy en-US Reumatismo 0048-7449 <p>Authors who publish with this journal agree to the following terms:</p> <ol type="a"> <li class="show">Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a <a href="" target="_new">Creative Commons Attribution License</a> that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.</li> <li class="show">Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.</li> <li class="show">Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work.</li> </ol> The Italian Society of Rheumatology clinical practice guidelines for the management of polymyalgia rheumatica <p>Objective: to provide evidence-based up-to-date recommendations for the management of patients with a definite diagnosis of polymyalgia rheumatica (PMR). Methods: A systematic literature review was performed to find the existing clinical practice guidelines (CPGs) on PMR and the framework of the Guidelines International Network Adaptation Working Group was used to appraise (AGREE II), synthesize, and customize the recommendations according to the needs of the Italian healthcare context. Rheumatologists on behalf of the Italian Society of Rheumatology (SIR) and from the SIR Epidemiology Unit joined the working group and identified the key health questions on PMR to guide the systematic literature review. Physicians, including general practitioners and specialists, and health professionals who manage PMR in the clinical practice were the target audience. The final recommendations were rated externally by a multi-disciplinary and multi-professional group of stakeholders. Results: From the systematic search in databases (Medline, Embase) and grey literature, 3 CPGs were identified and appraised by two independent raters. Combining the statements and the evidence from these CPGs, 9 recommendations were developed by endorsement or adaptation in response to the initial key health questions. The quality of evidence was graded and the working group discussed the final recommendations in view of their implementation in the Italian healthcare context. Conclusions: In absence of national guidelines so far, these recommendations are the first to provide guidance for the management of patients with a diagnosis of PMR in Italy and they are expected to ensure the best evidence-based clinical practice for this disease.</p> N. Ughi G.D. Sebastiani R. Gerli C. Salvarani S. Parisi A. Ariani I. Prevete M. Manara F. Rumi C.A. Sciré A. Bortoluzzi Copyright (c) 2020 the Author(s) 2020-04-10 2020-04-10 72 1 1 15 10.4081/reumatismo.2020.1268 Association between red cell distribution width and response to methotrexate in rheumatoid arthritis <p>Red cell distribution width (RDW) is an unconventional biomarker of inflammation. We aimed to explore its role as a predictor of treatment response in rheumatoid arthritis (RA). Eighty-two RA patients (55 females), median age [interquartile range] 63 years [52-69], were selected by scanning the medical records of a rheumatology clinic, to analyze the associations between baseline RDW, disease activity scores and inflammatory markers, as well as the relationship between RDW changes following methotrexate (MTX) and treatment response. The lower the median baseline RDW, the greater were the chances of a positive EULAR response at three months, 13.5% [13.0-14.4] being among those with good response, <em>vs</em> 14.0% [13.2-14.7] and 14.2% [13.5- 16.0] (p=0.009) among those with moderate and poor response, respectively. MTX treatment was followed by a significant RDW increase (p&lt;0.0001). The increase of RDW was greater among patients with good EULAR response, becoming progressively smaller in cases with moderate and poor response (1.0% [0.4-1.4] <em>vs</em>. 0.7 [0.1-2.0] <em>vs</em>. 0.3 [-0.1-0.8]; p=0.03). RDW is a strong predictor of early response to MTX in RA. RDW significantly increases after MTX initiation in parallel to treatment response, suggesting a role as a marker of MTX effectiveness.</p> M. Bellan D. Soddu E. Zecca A. Croce R. Bonometti R. Pedrazzoli D. Sola C. Rigamonti L.M. Castello G.C. Avanzi M. Pirisi P.P. Sainaghi Copyright (c) 2020 the Author(s) 2020-04-10 2020-04-10 72 1 16 20 10.4081/reumatismo.2020.1243 Translation and cross-cultural adaptation into Italian of the self-administered FLARE-RA questionnaire for rheumatoid arthritis <p>The aim was to provide a translation into Italian with cross-cultural adaptation of the French FLARE-Rheumatoid Arthritis (RA) questionnaire, and to test its acceptability, feasibility, reliability and construct validity in a single-centre cohort study. The French version of the FLARE-RA questionnaire was cross-culturally adapted and translated into Italian following an established forward–backward translation procedure, with independent translations and backtranslations. To validate the Italian version we tested the internal validity with Cronbach’s alpha, test-retest reliability with the intraclass correlation coefficient, agreement between assessments with Bland-Altman plots and construct validity with Spearman’s correlation coefficients. The questionnaire was tested on 283 consecutive RA outpatients (mean age 56.1±13.9 years, 226/283 females, median disease duration 12.6 years ranging from 0.2 to 70.6). For the global score (11 items) the Cronbach’s alpha coefficient was 0.94. The intraclass correlation coefficient was 0.87 (95% CI, 0.76-0.96). The correlation of FLARE-RA global score was 0.59 (95% CI, 0.50-0.66) with the Disease Activity Score on 28 joints, 0.63 (95% CI, 0.55-0.71) with the Simplified Disease Activity Index, 0.77 (95% CI, 0.71-0.83) with the RA Impact of Disease and 0.67 (95% CI, 0.59-0.73) with the Health Assessment Questionnaire. The Italian version of the FLARE-RA is feasible, brief and easy to administer. The translated and cross-cultural adapted showed accordingly to be valid and reliable. This questionnaire has some practical advantages, such as clarity, comprehensiveness, simplicity, and a minimum filling time. The development of cross-cultural adapted questionnaires in different languages is of pivotal importance to obtain standardized and comparable data across countries.</p> N. Ughi T. Schioppo I. Scotti V. Merlino A. Murgo O. De Lucia B. Fautrel F. Guillemin R. Christensen F. Ingegnoli Copyright (c) 2020 the Author(s) 2020-04-10 2020-04-10 72 1 21 30 10.4081/reumatismo.2020.1230 Understanding and perceptions of gout: an interdisciplinary assessment among patients, physicians and pharmacists in Italy <p>The objective was to assess knowledge and therapeutic approaches to the management of gout among healthcare professionals and people with/without gout, in Italy. This was a cross-sectional internet-based survey targeting general practitioners (GPs), specialists, pharmacists, and people with/without gout. Between December 2017 and March 2018, participants completed questionnaires on epidemiology, cause/risk factors, therapy objectives and management/treatment strategies to improve outcomes. Overall, 3184 people completed the survey: 699 GPs, 426 specialists, 655 pharmacists and 1404 subjects from the general population: 126 (9.0%) with and 1278 (91.0%) without gout. Notably, less than half of GPs, specialists and people without gout confirmed the published 1% prevalence of gout in Italy. Lifestyle was acknowledged as the main risk factor for gout by nearly 50% of specialists and GPs, while only 13.8% and 12.4%, respectively, considered the role of genetic factors. Uric acid overproduction was deemed as the cause of gout by 60% of GPs and specialists, whereas insufficient excretion by only 30%. Fewer than half of patients were aware that gout permanently damages joints, and even fewer of the renal and cardiovascular implications (19.4% and 12%, respectively); moreover, most people without gout replied that their doctor had never talked with them about uric acid and its correlation with gout development. Finally, GPs were divided on uric acid target levels (48.3% said &lt;6 mg/dL and 18.9% &lt;7 mg/dL). Despite major advances in the knowledge of physiopathological mechanisms of gout, the results of our survey highlight the many treatment and knowledge gaps in its management. Cooperation between multidisciplinary teams is required to break down barriers and ensure optimal treatment with effective and innovative agents of this ever-increasing debilitating condition.</p> L. Punzi G. Medea Copyright (c) 2020 the Author(s) 2020-04-10 2020-04-10 72 1 31 43 10.4081/reumatismo.2020.1227 The clinical value of the delta finger to palm distance in systemic sclerosis <p>Systemic sclerosis (SSc) is a collagen-vascular disorder characterized by fibrosis and vasculopathy. Delta finger to palm distance (delta FTP) is an index measuring the distance between the tip of the third finger to the distal palmar crease in the flexed and extended position. The present study aimed to evaluate the clinical value of delta FTP and to assess the correlation of delta FTP with modified Rodnan skin score (mRSS) and forced vital capacity (FVC) over the 12-month follow-up. This prospective longitudinal study began with 50 participants who were followed for twelve months. Lowess smoothing and linear regression were applied to detect and assess the relationship between delta FTP and mRSS. p-values were adjusted by the Benjamini-Hochberg method (BHM) as a control for false discovery rate. Delta FTP was lower among patients with higher disease duration (p-value<sup>adj</sup>: 0.008), diffuse cutaneous SSc (p-value<sup>adj</sup>: 0.006), digital ulcers (p-value<sup>adj</sup>: 0.003), telangiectasia (p-value<sup>adj</sup>: 0.006) and dysphagia (p-value<sup>adj</sup>: 0.036). The mRSS has a significant negative linear effect on the delta FTP at the baseline and the end of the follow-up (<em>r</em>: -0.31 and -0.40, respectively). Moreover, changes of mRSS and delta FTP showed a negative linear association over time (<em>r</em>: -0.22). These linear effects remained significant after regrouping the patients based on their SSc subtype. Delta FTP and FVC were not correlated either at the baseline or at the end. It seems that the delta FTP can be a valuable clinical index, supported by its correlated changes with mRSS and other SSc clinical manifestations over the one-year follow-up.</p> A. Javinani S. Mostafaei F. Gharibdoost A.R. Jamshidi R. Atef Yekta T. Kiani Fard Z. Tamartash F. Gorgani M. Mirbeyk M. Mahmoudi K. Sadeghi H. Kavosi Copyright (c) 2020 the Author(s) 2020-04-10 2020-04-10 72 1 44 51 10.4081/reumatismo.2020.1233 Remission, low disease activity and improvement of pain and function in psoriatic arthritis patients treated with IL-12/23 and IL-17 inhibitors. A multicenter prospective study <p>The development of new biologic and targeted synthetic DMARDs can lead to good disease control. The aim of the present study was to assess the rate of remission and low disease activity, and the improvement of pain and function, in psoriatic arthritis (PsA) patients treated with new anti-IL-12/23 and anti-IL-17 biologic agents. A prospective 6-month study was performed. Patients fulfilling the CASPAR criteria for PsA that started ustekinumab, secukinumab and ixekizumab were enrolled and prospectively followed in a setting of clinical practice. Patients were considered in minimal disease activity (MDA), when they met at least 5/7 of the criteria previously defined. DAPSA score ≤4 was also evaluated as a remission criterion. Pain on VAS, PtGA and HAQ were also assessed in all patients. Patients achieving MDA were compared to non-MDA to identify outcome predictive factors. Of the 70 patients treated with ustekinumab, secukinumab and ixekizumab, at baseline, no patients were in MDA or had a DAPSA score ≤4. Ten patients (14.2%) were lost during the follow-up. After 6 months, MDA was achieved in 22 (31.4%) patients. DAPSA≤4 was achieved in 17 (24.2%) patients. Significant improvement in pain, PtGA and HAQ was also found. Patients naïve to anti-TNF treatment achieved more frequently MDA compared to anti-TNF-experienced patients. Male sex, high levels of CRP and absence of comorbidities were found to be predictors of MDA. In our prospective observational study, MDA was achieved in 31.4% and DAPSA remission in 24.2% of patients treated with inhibitors of IL-12/23 and IL-17, thus making this target achievable in PsA patients treated with these drugs.</p> F.M. Perrotta A. Delle Sedie S. Scriffignano P. Volpe E. Cordisco N. Milano M. Gabini E. Lubrano Copyright (c) 2020 the Author(s) 2020-04-10 2020-04-10 72 1 52 59 10.4081/reumatismo.2020.1266 The role of architectural design for rheumatic patients’ wellbeing: the point of view of Environmental Psychology <p>Rheumatic diseases (RD) are among the most frequent disorders in the population and the major causes of chronic pain and disability. The resulting consequences are catastrophic, leading to a significant socio-economic burden, which includes significant reductions in quality of life (QoL) and limitations in regular work and daily activities of patients. In spite of this, rheumatic diseases are often misunderstood or diagnosed late, probably due to their characteristics of silent diseases, sometimes unrecognizable to unaffected or unskilled people. Actually, it is surprising that, despite their consequences on QoL and on individual impact, rheumatic diseases are underestimated by the public opinion, which is probably more attracted by other major diseases causing death. This <em>silent</em> perception can even be seen in some among the most recent psycho-social approaches to population needs in the fields of Health Psychology and Environmental Psychology. The latter, also known as Architectural Psychology, is a branch of Psychology that analyses the effects of the built environment on humans, including those affected by diseases. Paradoxically, in many cases, some components of the environments created to protect individuals and/or the population may represent barriers and subsequently causes of disability and suffering in patients with rheumatic diseases. In order to increase awareness about this particular aspect of social life, HEMOVE Onlus, a non-profit association, has promoted the creation of a multidisciplinary Task Group, which included mainly rheumatologists, psychologists and architects, with the aim of applying also for the benefit of rheumatic patients the most modern technical skills available in the context of Environmental Psychology, including in particular design and information technology.</p> L. Punzi M. Chia S. Cipolletta C. Dolcetti P. Galozzi O. Giovinazzi S. Tonolo R. Zava F. Pazzaglia Copyright (c) 2020 the Author(s) 2020-04-10 2020-04-10 72 1 60 66 10.4081/reumatismo.2020.1251 Gitelman syndrome associated with chondrocalcinosis and severe neuropathy: a novel heterozygous mutation in SLC12A3 gene <p>Gitelman syndrome (GS) is an inherited salt-wasting tubulopathy characterized by hypocalciuria, hypokalemia, hypomagnesemia and metabolic alkalosis, due to inactivating mutations in the <em>SLC12A3</em> gene. Symptoms may be systemic, neurological, cardiovascular, ophthalmological or musculoskeletal. We describe a 70 year-old patient affected by recurrent arthralgias, hypoesthesia and hyposthenia in all 4 limbs and severe hypokalemia, complicated by atrial flutter. Moreover, our patient reported eating large amounts of licorice, and was treated with medium-high dosages of furosemide, thus making diagnosis very challenging. Genetic analysis demonstrated a novel heterozygous mutation in the <em>SLC12A3</em> gene; therefore, we diagnosed GS and started potassium and magnesium replacement. GS combined with chondrocalcinosis and neurological involvement is quite common, but this is the first case of an EMG-proven severe neuropathy associated with GS. Herein, we underline the close correlation between hypomagnesemia, chondrocalcinosis and neurological involvement. Moreover, we report a new heterozygous mutation in exon 23 (2738G&gt;A), supporting evidence of a large genetic heterogeneity in this late-onset congenital tubulopathy.</p> E. Conticini A. Negro L. Magnani R. Ugolini B. Atienza-Mateo B. Frediani C. Salvarani Copyright (c) 2020 the Author(s) 2020-04-10 2020-04-10 72 1 67 70 10.4081/reumatismo.2020.1255