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>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> Gender equality in Rheumatology <p>Not available</p> S.L. Bosello M.S. Chimenti P. Conigliaro C. Iannuccelli E. Gremese F.R. Spinelli M. Vadacca ##submission.copyrightStatement## 2020-01-28 2020-01-28 71 4 173 176 10.4081/reumatismo.2019.1259 Adult-onset systemic autoinflammatory disorders: a clinical approach <p>Autoinflammatory disorders (AIDs) are a subgroup of immune-mediated syndromes that result from a primary dysfunction of the innate immune system. AIDs can be either monogenic or polygenic diseases. Unlike organspecific AIDs, systemic AIDs are characterized by fever and/or elevation of acute-phase reactants. This review aims to describe the most common adult-onset systemic AIDs, focusing mostly on polygenic and mixed-pattern diseases which are expected to be more prevalent in adult patients than monogenic AIDs overall. The literature was searched in Medline database. Organ-specific or childhood-onset systemic AIDs were excluded. AIDs were divided in three distinct groups: mixed-pattern, polygenic and adult-onset monogenic AIDs. Most adult-onset AIDs are polygenic but late-onset disease is not rare among monogenic AIDs such as familial Mediterranean fever (FMF). The diagnosis of systemic AIDs in adults is often delayed due to several factors and sometimes it is only established when amyloidosis or other complications are present. Therefore, it probably makes sense to primarily exclude common AIDs in adult patients with fever of unknown origin (and probably different presentations such as polyserositis) since a high prevalence of adult-onset Still’s disease or FMF is usually expected. Colchicine, nonsteroidal anti-inflammatory drugs, steroids, immunosuppressive agents, interleukin-1 inhibitors and tumor necrosis factor antagonists constitute common therapeutic options for systemic AIDs.</p> T. Borges A. Barbosa S. Silva ##submission.copyrightStatement## 2020-01-28 2020-01-28 71 4 177 188 10.4081/reumatismo.2019.1192 Macrophage activation syndrome as a complication of rheumatologic disorders, a report from Iran <p>The objective was to evaluate the clinical and laboratory manifestations and outcomes of the MAS cases in the context of systemic juvenile idiopathic arthritis (SJIA), systemic lupus erythematosus (SLE), Kawasaki disease, poly-articular juvenile idiopathic arthritis (PJIA). Twenty consecutive patients diagnosed with MAS between 2005 and 2016 entered the study. The cases were divided into two groups: in the first, MAS emerged in the context of a previously diagnosed rheumatologic disease, while in the second, MAS was the first presentation of a rheumatologic disease. In the other classification, the cases were divided into recurrent and non-recurrent cases. Laboratory data were recorded at three times: before MAS attack, during MAS attack, and 1 month after discharge from hospital. Nineteen cases with the median age of 5.9 (3.6-10) years entered the study. Four cases (21.1%) showed recurrent attacks of MAS. MAS was the first presentation of disease in 10 cases. The median age of the patients in the underlying disease group (10 years) was significantly higher than in the first presentation group [4.5(1.7-6.1) years, p=0.003]. The median fibrinogen value during MAS attack in the underlying disease group (601 mg/ dL) was also significantly higher than in the first presentation group (174 mg/dL, p=0.038). The platelet count during MAS attack in the recurrent group (30,500/microliter) was significantly lower than in the non-recurrent group (135,000/microliter, p=0.042). Our series of MAS cases demonstrated an overview of the symptoms, signs, laboratory manifestations, treatment, and prognosis of these cases. The higher median fibrinogen in MAS in the underlying disease group revealed that a decreasing level of fibrinogen in chronic disease is more significant than a single cut off value. Indeed, the lower platelet count in the recurrent MAS group may indicate greater platelet consumption due to organomegaly. Early diagnosis and treatment may save the patients’ lives.</p> R. Assari P. Sadeghi A. Mirmohammadsadeghi F. Ebadi V. Ziaee ##submission.copyrightStatement## 2020-01-28 2020-01-28 71 4 189 198 10.4081/reumatismo.2019.1204 Extreme hyperferritinemia: etiological spectrum and impact on prognosis <p>Hyperferritinemia can be the result of inflammation, infection, iron overload, or other uncommon pathologies including hemophagocytic lymphohistiocytosis (HLH). The significance of its elevation and its association with poor prognosis and critical clinical situations is unclear. To study the spectrum of diagnosis associated with elevated serum ferritin, we made a retrospective review of patients admitted to our center from 2015 and 2017 with serum ferritin levels above 2000 μg L<sup>-1</sup>. The H score was retrospectively assessed in all cases to evaluate the possible presence of HLH. The degree of ferritinemia found was compared with the evaluation of the undelying diagnosis and the results of laboratory examinations. A total of 77 patients were identified with a serum ferritin level &gt;2000 μg L<sup>-1</sup>. Hematological malignancy was the most prevalent diagnosis with n=20; severe infection was next with n=14. Eleven patients were diagnosed with HLH. The hemophagocytosis pictures on bone marrow smear and mortality rate were significantly correlated with ferritin level above 6000 μg L<sup>-1</sup> (p&lt;0.01). The comparison of the HLH subgroup with the rest of the cohort showed that fever, cytopenia (anemia, leucopenia, neutropenia and thrombocytopenia), hemophagocytosis pictures, and major liver disturbances were significantly increased in the HLH subgroup. The H score was significantly elevated in the subgroup of patients with ferritin above 6000 μg L<sup>-1</sup>, with a significatively higher probability of HLH (p&lt;0.01). The mortality rate at 3 months was significantly increased in the HLH subgroup. Extreme hyperferritin cannot be considered as a specific marker for the diagnosis. The cut off of 6000 μg L<sup>-1</sup> is significantly associated with HLH diagnosis. The H score is an interesting screening tool that physicians should use to rule out the probability of HLH when facing critical clinical situations.</p> N. Belfeki A. Strazzulla M. Picque S. Diamantis ##submission.copyrightStatement## 2020-01-28 2020-01-28 71 4 199 202 10.4081/reumatismo.2019.1221 Is CDAI comparable to DAS 28 and SDAI regarding inter-observer agreement and correlation to MHAQ in Egyptian RA patients? <p>Choosing between the different disease activity indices used for rheumatoid arthritis RA evaluation in clinical practice and research is often difficult. The aim of the current study was to compare clinical disease activity index (CDAI) to simplified disease activity index (SDAI), and disease activity score 28 (DAS28) regarding inter-observer reliability and correlation to the modified health assessment questionnaire (MHAQ) in a cohort of Egyptian RA patients. This study included one hundred RA patients. Every patient had an independent clinical evaluation made by two rheumatologists (professor and candidate) to evaluate disease activity using DAS28 with its 4 types, CDAI and SDAI. We used Cohen’s weighted kappa coefficient to measure the inter-observer agreement between the professor and candidate in different disease activity measures. Correlation between MHAQ and disease activity measures was made with Spearman’s rho test. Inter-observer agreement in CDAI and DAS28 values was almost perfect. A strong positive correlation was found between professor and candidate regarding the tested activity indices (p&lt;0.001), and a positive correlation was found between MHAQ and all Disease Activity Scores made by both professor and candidate (p&lt;0.001). CDAI proved to be comparable to other disease activity scores regarding inter-observer agreement and relation to MHAQ.</p> K. El-Hadidi S.M. Gamal S. Saad N.Y. Elsaid ##submission.copyrightStatement## 2020-01-28 2020-01-28 71 4 203 208 10.4081/reumatismo.2019.1222 Spectrum of musculoskeletal disorders in Nigerians with types 2 diabetes mellitus: prevalence and predictors <p>Musculoskeletal (MSK) conditions are more frequently found among patients with diabetes mellitus (DM) than in the non-diabetics. Despite several reports outside Africa, they have been under-studied among Africans. This study aimed to assess the overall prevalence and predictors of MSK conditions in Nigerian with types 2 diabetes mellitus (T2DM). A total of 268 adult with T2DM and 268 non-diabetic controls were recruited. All study subjects had their socio-demographics and clinical parameters obtained using interviewer-administered questionnaire. Musculoskeletal conditions among study subjects were classified using validated criteria and case definitions. Musculoskeletal disorders (MSKD) were significantly more frequent amongst subjects with DM (56% vs 22%, OR=4.5 p=0.001). Osteoarthritis (9.3% vs 4.1%, p=0.016), lumbosacral spondylosis (5.6% vs 2.2%, p=0.045), limited joint mobility (13.8% vs 5.6%, p-0.001), adhesive capsulitis (4.5% vs 1.5%, p-0.042) and rotator cuff tendinitis (3.7% vs 0.4%, p=0.006) were more frequent in DM subjects than in controls. Logistic regression showed that age (OR=2.1, CI=1.5-2.6) and waist circumference (OR=3.0, CI=2.6- 3.4) are independent predictors of MSKD among patients with diabetes. This study found higher prevalence of MSKD among diabetic subjects. It also identified certain factors associated with MSKD among patients with diabetes mellitus.</p> H. Olaosebikan A. Azenabor R. Akintayo O. Adelowo A. Ogbera A. Brodie-Mends ##submission.copyrightStatement## 2020-01-28 2020-01-28 71 4 209 217 10.4081/reumatismo.2019.1232 Severe Behçet’s disease equally affects both genders in Egyptian patients: A multicentre retrospective follow-up study <p>Behçet’s disease is a relapsing multisystemic disease. Its highest prevalence is seen along the Silk Road. While several studies reported gender disparities, others didn’t. Scarce data are available about the Arabs and the gender differences detected in some ethnicities could not be applied to others. Our study aimed to detect gender differences among a cohort of adult Egyptian patients with Behçet’s disease. Medical files of 255 adult patients diagnosed with Behçet’s disease at the Rheumatology and Rehabilitation Department of Kasr Al-Aini Hospital, Cairo University and the Internal Medicine Department of Tanta University, between 2002 and 2018, were retrospectively reviewed. The demographic features, the cumulative clinical features, the use, if any, of an intravenous pulse of methylprednisolone and immunosuppressive/biological drugs, and the frequency of the cumulative damage to the different organ systems were described. The disease severity score was calculated as well. The study revealed a prominent male predominance; the male to female ratio was 6.7:1. Acne/pseudofolliculitis was more common in males (28.6% vs 13.2%, p=0.046); the same was observed regarding any vascular involvement and peripheral venous disease (36.3% vs 18.4%, p=0.03 and 30.4% vs 13.2%, p=0.03, respectively). On the other hand, encephalitis and cranial nerve lesions were more prevalent in females (15.8% vs 6%, p=0.03 and 10.5% vs 3.2%, p=0.04, respectively). Severe Behçet’s disease equally affects Egyptian males and females. BD should not be considered a benign disease in Egyptian females. The same quality of medical care should be equally offered to both genders.</p> D.H.S. Attia R.A. Abdel Noor ##submission.copyrightStatement## 2020-01-28 2020-01-28 71 4 218 225 10.4081/reumatismo.2019.1223 Erysipelas-like presentation of Wells’ syndrome (eosinophilic cellulitis) <p>Wells’ syndrome, also called eosinophilic cellulitis, is a rare eosinophilic dermatosis characterized by an unspecific inflammatory erythematous eruption often associated with systemic symptoms. Here we report the case of a 57-year-old female with bilateral painful pitting and pruritic feet progressive for two weeks despite one week of oral antibiotics. Skin biopsy was performed showing dermal eosinophilic infiltration. The patient showed a spontaneous progressive improvement of the condition. The presented case demonstrates both clinical and histologic presence of lesions of Wells’ syndrome in the course of the disease. A careful diagnostic approach is needed because of the lack of specific signs. The global outcome is favorable and spontaneous resolution is possible.</p> N. Belfeki E. Gharbi C. Flateau S. Diamantis ##submission.copyrightStatement## 2020-01-28 2020-01-28 71 4 226 229 10.4081/reumatismo.2019.1252 Subcalcaneal bursitis as the initial manifestation of rheumatoid arthritis: ultrasonographic observation of two cases <p>In early rheumatoid arthritis (RA), proliferative synovitis sometimes occurs earlier in the tenosynovium or bursal synovium than in the articular synovium. Here we report two patients who presented with subcalcaneal bursitis while progressing from undifferentiated arthritis with high-titer anti-CCP antibodies (ACPA) to a diagnosis of RA. They had initially presented with palindromic transient pain in the hands and the feet. They were strongly positive for ACPA and negative for rheumatoid factor (RF) at the onset of symptoms. A few years later, they developed persistent plantar heel pain and underwent musculoskeletal ultrasonography (MSUS). MSUS revealed subcalcaneal bursitis with synovial proliferation. At that time, they became positive for RF and they were clinically diagnosed and began receiving treatment for RA. They developed overt synovitis in their wrists and fingers several months later. To the best of our knowledge, this is the first report on MSUS-detection of subcalcaneal bursitis with synovial proliferation in patients in the very early phase of RA, although there have been many reports of forefoot bursitis. These cases suggest that MSUS scanning of the plantar surface of the heel may be useful for patients with plantar heel pain who are suspected of having a very early phase of RA, because proliferative synovitis can be detected as subcalcaneal bursitis.</p> T. Suzuki H. Shirai ##submission.copyrightStatement## 2020-01-28 2020-01-28 71 4 230 234 10.4081/reumatismo.2019.1249