Reumatismo 2019-05-19T15:30:05+02:00 Paola Granata Open Journal Systems <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> Novel autoantibodies in rheumatoid arthritis 2019-05-19T15:30:05+02:00 A.F. Bonifacio A. Alunno G.M.C. La Paglia E. Valentini M.C. Leone E. Bartoloni Roberto Gerli <p>Rheumatoid factor and antibodies against cyclic citrullinated peptides represent a diagnostic hallmark in rheumatoid arthritis (RA). However, over the last decades many other autoantibodies have been identified. Several proteins can trigger an aberrant autoimmune response in their native form while others acquire this feature after post-translational modifications such as citrullination, carbamylation or acetylation. It is of interest that also the enzymes catalyzing such post-translational modifications (<em>e.g</em>. the protein arginine deiminases) can transform themselves into autoantibodies in RA. The purpose of this review article is to provide an overview of relevant literature published over the last years regarding novel autoantibodies and their possible diagnostic and prognostic significance in RA.</p> 2019-04-01T00:00:00+02:00 ##submission.copyrightStatement## The Italian version of rheumatoid arthritis pain scale (IT-RAPS): psychometric properties on community and clinical samples 2019-05-19T15:30:05+02:00 M. Tofani M. Nobilia G. Culicchia G. Esposito A. Savona I. Tashi A. Ventura G. Galeoto <p>This paper describes the validation process of the Italian version of the Rheumatoid Arthritis Pain Scale (ITRAPS), describing its translation and adaptation to Italian culture. The cultural adaptation and validation were based on data from a sample of people affected by rheumatoid arthritis (RA). The process required a forward and backward translation of the original language, reviewed by an expert panel. The adapted version of the RAPS was then tested on a community and clinical sample, in order to test its psychometric properties. The IT-RAPS was submitted to 122 people affected by RA. The data was analyzed using Cronbach’s coefficient alpha and the Pearson product-moment correlation coefficients. The IT-RAPS showed an internal consistency reliability coefficient of 0.96. This is the first study reporting the validation and cross-cultural adaptation of the RAPS in Italian. The study’s findings provided support for the IT-RAPS as a reliable and valid measurement of multidimensional pain in RA patients.</p> 2019-04-01T11:39:54+02:00 ##submission.copyrightStatement## Serum klotho concentrations inversely correlate with the severity of nailfold capillaroscopic patterns in patients with systemic sclerosis 2019-05-19T15:30:04+02:00 R. Talotta F. Rigamonti T. Letizia S. Bongiovanni M.C. Ditto M. Antivalle S. Santandrea F. Atzeni T. Vago P. Sarzi-Puttini <p>Klotho is a transmembrane and soluble glycoprotein that governs vascular integrity. Previous studies have demonstrated reduced serum klotho concentrations in patients with systemic sclerosis (SSc), and it is known that klotho deficiency can impair the healing of digital ulcers related to microvessel damage. The aim of this study was to evaluate the association between serum klotho levels and nailfold capillaroscopic abnormalities in SSc patients. We retrospectively enrolled 54 consecutive patients with SSc diagnosed on the basis of the 2013 EULAR/ACR criteria [11 with diffuse SSc; 47 females; median age 68.0 years (IQ 18); median disease duration 11.0 years (IQ 7)]. Serum klotho concentrations were determined by means of an enzyme-linked immunosorbent assay. On the basis of the 2000 classification of Cutolo et al., 14 patients had normal nailfold capillaroscopic findings, 8 had an early scleroderma pattern, 21 an active scleroderma pattern, and 11 a late scleroderma pattern. The median serum klotho concentration was 0.29 ng/mL (IQ 1). Regression analysis of variation showed an inverse correlation between serum klotho concentrations and the severity of the capillaroscopic pattern (p=0.02; t -2.2284), which was not influenced by concomitant treatment. Logistic regression did not reveal any significant association between the risk of developing digital ulcers and nailfold capillaroscopic patterns, serum klotho levels, or concomitant medications. The presence of avascular areas significantly correlated with calcinosis (p=0.006). In line with previous studies, our findings confirm that klotho plays a role in preventing microvascular damage detected with nailfold capillaroscopy.</p> 2019-04-01T11:41:17+02:00 ##submission.copyrightStatement## Occult HBV infection may negatively impact on drug survival in patients with rheumatoid arthritis on treatment with a first biologic drug. An appraisal from the Biologic Apulian Registry (BIOPURE) 2019-05-19T15:30:03+02:00 G. Carlino M. Fornaro L. Santo R. Bucci A. Semeraro L. Quarta F. D'Onofrio A. Marsico C. Zuccaro P.C. Falappone D. Mazzotta F.P. Cantatore M. Muratore F. Iannone <p>We performed a retrospective analysis to evaluate the survival on first line biologic drug of rheumatoid arthritis (RA) patients with potential occult HBV infection (pOBI). We analysed longitudinal data of 486 consecutive RA patients starting a first biological drug in a time frame from 1<sup>st</sup> January 2008 to 31<sup>st</sup> December 2014. Demographic and disease related characteristics were collected at baseline and at the last observation visit. Baseline serological markers of HBV infection and causes of treatment discontinuation were also recorded. Primary endpoint was the influence of pOBI on drug survival, estimated by Kaplan-Meier life table analysis. Estimates hazard ratios (HRs) of drug discontinuation, adjusted for disease characteristics, biological drug class and HBcAb status were computed by Cox-regression models. The retention rate was significantly lower in pOBI positive patients (58.2%) when compared to pOBI negative ones (67.8%) and this data was confirmed also when only discontinuation due to ineffectiveness was considered (pOBI positive 66.4% <em>vs</em> pOBI negative 75.3%, long rank 7.93, p=0.005). Cox regression models showed a significant association between HBcAb-neg (HR 0.58, 0.41-0.84), higher ESR-DAS28 at baseline (HR 1.07, 1.03-1.11) or RF/ACPA-neg (HR 1.46, 1.04-2.06) and drug discontinuation. Occult HBV infection seems to influence negatively the effectiveness of biological therapies in RA patients.</p> 2019-04-01T11:42:30+02:00 ##submission.copyrightStatement## A rare disease with a rare presentation: hemi-atrophy caused by fibromuscular dysplasia in a 27 month old girl 2019-05-19T15:30:02+02:00 B. Gharib A. Ghareh Zadeh Shirazi E. Moradi B. Yaghmaei V. Ziaee <p>We discuss the case of a 27-month-old girl afflicted with fibromuscular dysplasia. She presented with hemiatrophy of left upper and lower limbs, nail dystrophy, ulcers on the tips of her toes, cold and painful limbs, foot drop, and hypertension. The initial appearance started at 2 months of age and other diagnoses such as <em>complex regional pain syndrome, reflex sympathetic syndrome, vasculitis </em>and<em> coagulation disorders</em> had been considered. Angiography revealed that all the arterial branches of the left lower and upper limbs, from brachial to ulnar and radial, and from iliac and femoral to tibialis arteries were affected. Sural nerve biopsy confirmed the diagnosis. In the follow-up visits until 2 years after the patient’s discharge she did not develop any new problem and her blood pressure was controlled by enalapril and amlodipine.</p> 2019-04-01T11:43:50+02:00 ##submission.copyrightStatement## Acquired Haemophilia A. Which is the best therapeutic choice in older adults? Single center study of 4 cases 2019-05-19T15:30:01+02:00 E. Mauro E. Garlatti Costa A. Zanier M. Maset A. Ermacora M. Ghersetti P. Casarin <p>Acquired haemophilia A (AHA) is a rare bleeding disorder due to autoantibodies directed against coagulation factor VIII. The treatment is based on recombinant activated factor VII and activated prothrombin complex concentrate. However, mainly in older patients, severe thrombotic complications have been reported. Here we report the different therapeutic approaches in 4 cases of elderly patients with AHA and co-morbidities.</p> 2019-04-01T11:44:39+02:00 ##submission.copyrightStatement## Incidental papillary thyroid cancer diagnosis in patient with adult-onset Still’s disease-like manifestations 2019-05-19T15:30:01+02:00 R. Tirri D. Capocotta <p>Adult onset Still’s disease (AOSD) is a systemic inflammatory disease characterized primarily by a triad consisting of daily fever, arthritis and maculopapular exanthema. The pathogenesis and etiology of AOSD are unknown and the diagnosis, which can be very challenging, is often made by exclusion. Here, we report a case of a 61-year-old woman with a history of mild psoriatic arthritis, fever, arthritis and maculopapular exanthema. Her initial laboratory tests showed neutrophilic leukocytosis, hypertransaminasemia, and markedly elevated levels of the erythrocyte sedimentation rate and C-reactive protein. With a presumptive diagnosis of AOSD, based on Yamaguchi criteria, the patient started an extensive diagnostic work-up to exclude other potential differential diagnoses. With fluorodeoxyglucose (FDG) positron-emission tomography, a thyroid nodule with moderate FDG uptakes was detected. The fine needle aspiration biopsy led to diagnosis of papillary thyroid cancer. The history of psoriatic arthritis, the patient’s age, and atypical features of the skin rash described as not concomitant with fever flares, suggested a diagnosis of paraneoplastic AOSD-like manifestations.</p> 2019-04-01T11:45:17+02:00 ##submission.copyrightStatement## Poncet’s disease diagnostic criteria decodes conundrum: Poncet’s vs pseudo Poncet’s 2019-05-19T15:30:00+02:00 V. Shobha A.M. Desai <p>Poncet’s disease is very important and yet a challenging diagnosis of importance in countries with high TB endemicity (<em>e.g</em>. India). In this case series, we present 5 patients with diagnosed as Poncet’s disease and in our tertiary health care center over 12 months and examine the performance of the diagnostic criteria suggested by Sharma and Pinto. The majority (4/5) of the patients were subsequently diagnosed and responded to anti-tuberculous therapy. In the other patient, a diagnosis of atypical seropositive rheumatoid arthritis or Pseudo Poncet’s disease was established on follow up.</p> 2019-04-01T11:45:59+02:00 ##submission.copyrightStatement## The hominid thumb and its influence on the employment during evolution 2019-05-19T15:29:59+02:00 J.H. Villafañe R. Cantero-Tellez P. Berjano <p>Not available</p> 2019-04-01T11:47:09+02:00 ##submission.copyrightStatement## Long-term treatment with anakinra and canakinumab resolves patellar subchondral erosion in neonatal-onset multisystem inflammatory disease 2019-05-19T15:29:57+02:00 A. Leone R. Manna E. Verrecchia M. Cipullo D. Rigante <p>Not available</p> 2019-04-01T11:47:50+02:00 ##submission.copyrightStatement##