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> 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>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>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>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> Association between anti-citrullinated alpha enolase antibodies and clinical features in a cohort of patients with rheumatoid arthritis: a pilot study In recent years several antibodies against citrullinated peptides (ACPAs) have been identified in patients with rheumatoid arthritis (RA) and their pathogenic, diagnostic and prognostic significance is under intense investigation. Among ACPAs, those targeting citrullinated alpha enolase (anti-CEP1) have been identified in RA but data about their ability to predict the development of erosive disease are conflicting. Furthermore, no data are currently available concerning their possible association with extra-articular manifestations (EAMs) in RA. The aim of this study was to investigate the prevalence and significance of anti-CEP1 from a prognostic point of view. In this pilot study we confirmed that anti-CEP1 Abs are associated with higher prevalence of bone erosions, but we also provided the first evidence of an association between anti-CEP1 Abs and RA interstitial lung disease (ILD). These results provide the basis to investigate the association between anti-CEP1 Abs and EAMs in larger cohorts of RA patients to possibly confirm its role as biomarker for RA-ILD. A. Alunno O. Bistoni F. Pratesi F. Topini I. Puxeddu V. Valentini G. Cafaro E. Bartoloni P. Migliorini R. Gerli ##submission.copyrightStatement## 2018-07-06 2018-07-06 67 71 10.4081/reumatismo.2018.1028 Low body mass index in long standing rheumatoid arthritis: relation to RA disease activity and functional indices The aim of the work was to study the relationship between the body mass index (BMI) in longstanding rheumatoid arthritis (RA) and RA disease activity and functional indices. This study included 105 RA patients. For all patients, we recorded the presence of erosions on radiographs, the presence of subcutaneous nodules (SCN), the 28-tender joint count (TJC), 28-swollen joint count (SJC) scores, the visual analogue scale (VAS), physicians’ global assessments (PhGA), the erythrocyte sedimentation rate (ESR), and the rheumatoid factor (RF). The disease activity index (DAS28) and BMI were calculated and current treatment was recorded. Patients were divided into two groups: group I: BMI 25. Group I included 32 (30.5%) patients, whereas group II included 73 (69.5%) patients. There were statistically significant differences between the two groups regarding each of the following: SJC (p=0.006), erosions (p=0.006), DAS28 (p=0.016) and PhGA (p=0.007). All were higher in group I (underweight and normal) than in group II (overweight and obese). No statistically significant differences emerged regarding age (p=0.11), smoking (p=0.69), disease duration (p=0.46), TJC (p=0.14), SCN (p=1.00), HAQ (p=0.26), VAS (p=0.16), ESR (p=0.25), RF (p=0.54) and steroid cumulative dose (p=0.08). Low BMI in longstanding RA patients may indicate more active and erosive disease and it may be considered as a poor prognostic factor. S.M. Gamal A.K. Alkemary M.A. Abdo A.H.M. El Dakrony ##submission.copyrightStatement## 2018-07-06 2018-07-06 72 77 10.4081/reumatismo.2018.999 The use of rituximab in idiopathic inflammatory myopathies: description of a monocentric cohort and review of the literature Rituximab (RTX), a chimeric monoclonal antibody targeted against CD20, has been used to treat refractory inflammatory myopathies (IIM). The primary objective of this study was to retrospectively assess the efficacy of RTX in reducing disease activity in patients with IIM refractory to conventional therapy. Secondary aim was the evaluation of adverse events (AE) during the treatment period. We examined 26 patients with a diagnosis of IIM, referred to our Rheumatology Unit and treated with RTX for active refractory disease. Patients were treated with RTX 1000 mg i.v., twice, with a 2-week interval. RTX treatment was associated with a significant reduction of creatine kinase (p=0.001) after six months compared to the baseline, an improved muscular strength measured with MMT8 (p&lt;0.001) and a reduction of the extramuscular activity of the disease measured with MYOACT (p&lt;0.001). In particular, RTX improved DM skin rash, arthritis and pulmonary manifestations. Autoantibody positivity (in particular antisynthetase, anti- SRP and antiRo/SSA), and a disease duration &lt;36 months at the moment of the treatment are associated with a better response rate. Treatment with RTX was also associated with a reduction of the mean daily dose of steroids needed to control disease activity (p=0.002). Our results have confirmed that RTX is efficacious in the treatment of refractory IIM. Ad hoc controlled trials are needed to better clarify the specific subset of patients who may better respond to the treatment and the optimal therapeutic schedule. S. Barsotti E. Cioffi A. Tripoli A. Tavoni A. d’Ascanio M. Mosca R. Neri ##submission.copyrightStatement## 2018-07-06 2018-07-06 78 84 10.4081/reumatismo.2018.1011 Antibodies to extractable nuclear antigens (ENAS) in systemic lupus erythematosus patients: correlations with clinical manifestations and disease activity The aim was to explore possible correlations of antibodies to extractable nuclear antigens (ENA) with clinical manifestations and disease activity indices in systemic lupus erythematosus (SLE) patients. A total of 70 consecutive SLE patients (64 females) were included. Disease activity was assessed by SLE activity index (SLEDAI), and British Isles Lupus Assessment Group (BILAG). Anti-Ro/SSA correlated positively with, headache (r=0.24, p=0.04), blurring of vision (r=0.25, p=0.03) and SLEDAI (r=0.25, p=0.04) and negatively with C3 (r=–0.35, p=0.003). Anti-Ro/SSA correlated with anti La/SSB antibodies (r=0.69, p&lt;0.001), but not with anti-DNA, anti-RNP and anti-Sm antibodies. Anti-La/SSB antibodies correlated with headache (r=0.26, p=0.03), SLEDAI (r=0.25, p=0.03) and negatively with C3 (r=–0.34, p=0.004). Anti-La/SSB did not correlate with anti-RNP or anti-Sm antibodies. Anti-Sm antibodies correlated with disease duration (r=0.34, p=0.003), 24 hours urinary proteins (r=0.31, p=0.008), SLEDAI (r=0.31, p=0.009), BILAG renal score (r=0.29, p=0.02) and negatively with age at onset (r=–0.27, p=0.02), WBCs (r=–0.29, p=0.014) and C4 (r=–0.25, p=0.049). In multivariate analyses, anti-Ro/SSA antibodies remained associated with headache, blurring of vision and C3 and anti-La/SSB antibodies remained associated with C3 and with headache. Anti-Sm antibodies were independently associated with disease duration and total SLEDAI scores, while anti-RNP antibodies remained significantly associated with BILAG mucocutaneous scores only. Antibodies to ENAs are associated with clinical aspects of SLE and may play a role in the assessment of disease activity. Insight into these ENAs may lead to new approaches to diagnostic testing, accurate evaluation of disease activity and lead to target approach for SLE. Y. Emad T. Gheita H. Darweesh P. Klooster R. Gamal H. Fathi N. El-Shaarawy M. Gamil M. Hawass R.M. El-Refai H. Al-Hanafi S. Abd-Ellatif A. Ismail J. Rasker ##submission.copyrightStatement## 2018-07-06 2018-07-06 85 91 10.4081/reumatismo.2018.1027 Determinants of occupational multisite musculoskeletal disorders: a cross sectional study among 254 patients The aim was to describe the profile of workers with occupational multi-site musculoskeletal disorders (MSMSD) and study the relationship between these lesions and socio-professional factors. This is a cross-sectional study involving 254 subjects with occupational musculoskeletal disorders (MSD), identified in the Department of Occupational Medicine at the University Hospital of Mahdia, in Tunisia, over a period of 10 years from 2005 to 2014. The study population was subdivided into two groups; mono-site MSD and multi-site (≥2 sites) groups. Data collection was based on a questionnaire prepared beforehand and covered the description of sociodemographic and professional characteristics. To study psychosocial constraints at work, we have used the Karasek questionnaire. MS-MSD was correlated to the number of dependent children (p=0.02), job/place of work (p=0.00), qualification (p=0.02), taking a rest period (p=0.03), decision latitude (p=0.00), mental demands (p=0.002), social support (p=0.00) and job stress (p=0.04). After binary logistic regression, MS-MSD depended significantly on the number of dependent children (p=0.013; OR=0,33; IC=0,17-0,83), working spouse (p=0.05; OR=0.35; IC=0.12-0.99), job/place of work (p=0.00; OR=4.16; IC=1.95-8.88), qualification (p=0.008; OR=0.28; IC=0.11-0.72), taking a break during work (p=0.04; OR=3.10; IC=1.04-9.22) and social support (p=0.00; OR=7,1; IC=1,9-25,3). When individual risk factors are fixed, the prevention of MS-MSD must target modifiable levers, related to the professional environment of the employees. A. Mahfoudh K. Fennani M. Akrout K. Taoufik ##submission.copyrightStatement## 2018-07-06 2018-07-06 92 99 10.4081/reumatismo.2018.1047 Macrophage activation syndrome in adult systemic lupus erythematosus: report of seven adult cases from a single Italian rheumatology center The aim was to describe the macrophage activation syndrome (MAS), a life-threatening syndrome characterized by excessive immune activation that can be triggered by conditions affecting immune homeostasis, in a cohort of adult Italian patients with systemic lupus erythematosus (SLE). This was a monocentric retrospective evaluation. The utility of the H-score, developed to estimate the individual risk of having reactive MAS in adult patients, was assessed. Among 511 patients with SLE, 7 cases (1.4%) of MAS (all females) were identified and their medical records reviewed. In all cases, MAS was simultaneous to the onset of SLE. All patients had fever, lymphadenopathy, hematological involvement, and high titer of anti-dsDNA antibodies. Workup for infections and malignancies was negative. In all cases, the H-score was higher than the cut-off suggested for the classification of reactive MAS. All cases required hospital admission, and 2 patients were admitted to the intensive care unit. Most patients were treated successfully with high doses of corticosteroids and with immunosuppressive drugs, whereas the full therapeutic regimen developed for primary hemophagocytic lymphohistiocytosis HLH was used only in one case. No death from MAS was observed. MAS is a rare and severe disorder that complicated the onset of SLE in our cohort. The H-score may be useful in the classification of these patients. F. Dall'Ara I. Cavazzana M. Frassi M. Taraborelli M. Fredi F. Franceschini L. Andreoli M. Rossi C. Cattaneo A. Tincani P. Airò ##submission.copyrightStatement## 2018-07-06 2018-07-06 100 105 10.4081/reumatismo.2018.1023 Coexistence of sarcoidosis and Hashimoto thyroiditis Sarcoidosis is a chronic, inflammatory disease with unknown cause characterized by non-caseating granuloma formations. It can present with bilateral hilar lymphadenopathy, skin lesions, eye involvement and locomotor system findings. Hashimoto thyroiditis is an organ-specific autoimmune disease characterized by increased autoantibody synthesis. Sarcoidosis can involve different endocrine glands. Thyroid gland involvement may lead to increased thyroid function disorders and autoantibodies. Herein, we report an 80-year-old female patient with sarcoidosis and Hashimoto coexistence. H. Semiz M. Yalcin S. Kobak ##submission.copyrightStatement## 2018-07-06 2018-07-06 106 110 10.4081/reumatismo.2018.1017 Pulmonary cavitary lesions may be one of the presenting features in Ig A nephropathy Immunoglobulin A (Ig A) nephropathy is the most frequent primary glomerulonephritis. Renal limited disease is the most widespread clinical form of the disease. Pulmonary involvement may also be seen concomitantly and capillaritis with pulmonary hemorrhage is the most frequent pulmonary involvement. In this paper, for the first time in literature, we describe an Ig A nephropathy patient with multiple pulmonary cavities as one of the presenting features of the disease. Also, no other etiology for the cavities was found other than Ig A nephropathy. Herein, possible pathogenesis might be capillaritis or deposition of immune complexes. As a result, it should be kept in mind that pulmonary cavity may be the presenting feature of Ig A nephropathy especially with other frequent signs of the disease. B. Isci T.S. Gamsiz S. Kayipmaz O. Keskin M.E. Tezcan ##submission.copyrightStatement## 2018-07-06 2018-07-06 111 114 10.4081/reumatismo.2018.1037 Acute myocarditis as a revealing clue of complete Kawasaki disease Not available G. De Rosa L. Andreozzi M. Piastra B. Castelli D. Rigante ##submission.copyrightStatement## 2018-07-06 2018-07-06 115 116 10.4081/reumatismo.2018.1101 The real evidence for polymyalgia rheumatic as a paraneoplastic syndrome Not available M. Bellan P.P. Sainaghi M. Pirisi ##submission.copyrightStatement## 2018-07-06 2018-07-06 117 117 10.4081/reumatismo.2018.1148 The real evidence for polymyalgia rheumatic as a paraneoplastic syndrome Not available S. Muller S. Hider T. Helliwell R. Partington C. Mallen ##submission.copyrightStatement## 2018-07-06 2018-07-06 118 119 10.4081/reumatismo.2018.1166 RETRACTION: Pain in systemic sclerosis <p>To our readers: <br />With deep regrets, we inform that the article <em>Pain in systemic sclerosis</em> (DOI: <a href="" target="_blank">https://</a>), which has been published in Reumatismo (2014; 66(1): 44-47), contains verbatim text plagiarized from another paper. The manuscript must be considered as retracted.<br />On behalf of the Editorial Board of <em>Reumatismo</em>, I apologize to the Author of the manuscript whose text was plagiarized by Stisi <em>et al.</em> that this was not picked up in the peer review process. I also apologize to the affected journal for the violation of copyright due to plagiarism. <br /><em>Reumatismo</em> is uncompromising in its commitment to scientific integrity. When credible evidence of misconduct is brought to our attention, our commitment to the scientific record and to our readership requires immediate notification. <br /><em>Reumatismo</em> is increasingly employing sophisticated software to detect plagiarism. Other journals use similar tools. Authors should be aware that most journals routinely employ plagiarism detection software, and that any plagiarism is likely to be detected.</p><p>Marco A. Cimmino<br /><em>Editor-in-Chief</em><br /><em>Reumatismo</em></p> M.A. Cimmino ##submission.copyrightStatement## 2018-07-06 2018-07-06 120 120 10.4081/reumatismo.2018.1171