Reumatismo
https://www.reumatismo.org/reuma
<p><em>Official</em> <em>Journal of The Italian Society of Rheumatology</em>. Founded In 1949.</p> <p><strong>Reumatismo - The Italian Journal of Rheumatology</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 to Authors as it is supported by institutional funds.</p> <p><em>----------------------------------------------------------------------</em></p>PAGEPress Scientific Publications, Pavia, Italyen-USReumatismo0048-7449<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="http://creativecommons.org/licenses/by/3.0/" 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>Anti-tumor necrosis factor α: originators <i>versus</i> biosimilars, comparison in clinical response assessment in a multicenter cohort of patients with inflammatory arthropathies
https://www.reumatismo.org/reuma/article/view/1602
<p><em>Objective</em>. To compare etanercept and adalimumab biosimilars (SB4 and ABP501) and respective bioriginators in terms of safety and efficacy in a real-life contest.</p> <p><em>Methods</em>. We consequently enrolled patients affected by rheumatoid arthritis, psoriatic arthritis, and ankylosing spondylitis, treated with SB4, and ABP501, or with corresponding originators, belonging to the main biological prescribing centers in the Lazio region (Italy), from 2017 to 2020. Data were collected at recruitment and after 4, 8, 12, and 24 months of therapy.</p> <p><em>Results</em>. The multicenter cohort was composed by 455 patients treated with biosimilars [SB4/ABP501 276/179; female/male 307/146; biologic disease-modifying anti-rheumatic drug (b-DMARD) naïve 56%, median age/ interquartile range 55/46-65 years] and 436 treated with originators (etanercept/adalimumab 186/259, female/ male 279/157, b-DMARD naïve 67,2%, median age/interquartile range 53/43-62 years). No differences were found about safety, but the biosimilar group presented more discontinuations due to inefficacy (p<0.001). Female gender, being a smoker, and being b-DMARD naïve were predictive factors of reduced drug survival (p=0.05, p=0.046, p=0.001 respectively). The retention rate at 24 months was 81.1% for bioriginators and 76.5% for biosimilars (median retention time of 20.7 and 18.9 months, respectively) (p=0.002). Patients with remission/low disease activity achievement at 4 months showed a cumulative survival of 90% to biosimilar therapy until 24 months (p=0.001); early adverse reactions instead represented a cause of subsequent drug discontinuation (p=0.001).</p> <p><em>Conclusions</em>. Real-life data demonstrated a similar safety profile between biosimilars and originators, but a reduced biosimilar retention rate at 24 months. Biosimilars could be considered a valid, safe, and less expensive alternative to originators, allowing access to treatments for a wider patient population.</p>C. GioiaA. Picchianti DiamantiR. PerriconeM.S. ChimentiA. AfeltraL. NavariniA. MiglioreU. MassafraV. BruzzeseP. ScolieriC. MeschiniM. ParoliR. CaccavaleP. ScapatoR. ScrivoF. ContiB. LaganàM. Di Franco
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2023-12-192023-12-1975410.4081/reumatismo.2023.1602Lumbosacral pain in a patient with psoriatic arthritis: when the rheumatic disease is innocent
https://www.reumatismo.org/reuma/article/view/1565
<p>Lumbar pain is a very common symptom that derives from benign musculoskeletal conditions, rheumatic inflammatory diseases, neoplasms, and referred and/or nociplastic pain. A 70-year-old man with psoriatic arthritis presented with early-onset lumbosacral pain without evident red flags. Symptomatic treatment was unhelpful. Radiographic imaging showed subtle signs of a disease that could easily be missed. Magnetic resonance imaging revealed a massive prostatic malignancy with bone (sacral and iliopubic) metastasis. Awareness must be given not to disregard every lumbar pain as part of the preexisting rheumatic inflammatory disease (spondyloarthropathy in this case) or a common muscle/ligament/articular disarrangement. Persistence of pain, albeit not inflammatory nor sharp in nature, despite adequate treatment might be just as important as an acute red flag and requires proper follow-up.</p>H. ParenteM. Pontes FerreiraC. SoaresF. GuimarãesS. AzevedoD. Santos-FariaJ. Tavares-CostaD. PeixotoC. AfonsoD. RorizF. Teixeira
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2023-12-192023-12-1975410.4081/reumatismo.2023.1565Can restless legs be a sign of something else? A case report of spondyloarthritis presenting with restless legs syndrome and a review of the literature
https://www.reumatismo.org/reuma/article/view/1557
<p>Restless legs syndrome (RLS) is a chronic movement disorder characterized by an urge or need to move the limbs, usually associated with uncomfortable sensations in the legs and sleep disorders. In general, two clinical forms of RLS are described: primary and secondary. Although primary RLS has a familial component, the underlying mechanism is still not fully understood but seems to be related to abnormalities in the dopaminergic and glutamatergic pathways of the central nervous system. The secondary forms of the syndrome are associated with iron deficiency, renal failure, pregnancy, diabetes mellitus, peripheral neuropathy, and several rheumatologic disorders such as rheumatoid arthritis and Sjögren’s syndrome. In a few clinical trials, an increased frequency of RLS has been reported in patients with spondyloarthritis. In this report, a case of coexistence of spondyloarthritis and RLS is presented, showing satisfactory improvement with conservative treatment and additionally adding naproxen. Anemia of chronic disease occurring in rheumatic diseases, and associated iron deficiency may contribute to the development of RLS.</p>E. Yılmaz
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2023-12-192023-12-1975410.4081/reumatismo.2023.1557Acquired hemophilia A treated with rituximab in a 62-year-old female with rheumatoid arthritis: a case-based review
https://www.reumatismo.org/reuma/article/view/1585
<p>Acquired hemophilia A (AHA) is a rare autoimmune disorder with unpredictable hemostasis that is caused by autoantibody formation against coagulation factor VIII. AHA can occur in the context of autoimmune inflammatory rheumatic disorders. Here we report the case of a 62-year-old female with an 11-year history of rheumatoid arthritis (RA) who presented with cutaneous and mucosal bleeding. Activated partial thromboplastin time was prolonged and not corrected by the mixing test. Factor VIII activity was decreased, and the anti-factor VIII antibody was positive. AHA associated with RA was diagnosed. The patient was treated with rituximab 500 mg weekly for 4 doses and prednisolone 10 mg/daily. The patient did not experience bleeding events after treatment, and factor VIII activity and inhibitor normalized. At the end of the article, we discuss similar cases of RA-associated AHA.</p>D. MohamadzadehS. AssarF. Farsad
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2023-12-192023-12-1975410.4081/reumatismo.2023.1585Polymyositis following varicella and mumps infection in adults: report of two cases
https://www.reumatismo.org/reuma/article/view/1562
<p>Idiopathic immune myopathies (IIMs) are autoimmune diseases caused by immune-mediated muscle damage. The etiology remains unclear. Epidemiological and experimental studies, both in animals and humans, hint at viruses as major environmental factors able to trigger aberrant immune responses through many different mechanisms. However, only a few cases of either dermatomyositis or polymyositis following a specific viral infection have been reported in the literature. The objective of this study is to describe the clinical features and the treatment strategy of 2 cases of polymyositis developing shortly after chickenpox and mumps, respectively, and to review the existing literature on the topic. The clinical records of the 2 patients suspected to have developed inflammatory myositis following a viral infection were reviewed. Their clinical history, main laboratory findings, and treatment outcome are presented here. Moreover, a literature search was performed in the PubMed and MEDLINE databases to identify reports describing the association between viral infections and IIMs in patients aged ≥18. The 2 patients reported here developed polymyositis shortly after chickenpox and mumps, respectively, suggesting a causal role for viruses in triggering autoimmunity. Only a few reports published between 1990 and 2020 were found in the literature, possibly linking infections to myositis development. Intravenous immunoglobulin and rituximab were effective for the treatment of viral-triggered polymyositis.</p>F. MasiniK. GjeloshiE. PinottiR. FerraraC. RomanoG. Cuomo
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2023-12-192023-12-1975410.4081/reumatismo.2023.1562Eosinophilic granulomatosis with polyangiitis: sequential use of mepolizumab following rituximab for inadequate asthma control despite vasculitis remission
https://www.reumatismo.org/reuma/article/view/1588
<p>We report the case of a 54-year-old woman with antineutrophilic cytoplasmic antibody-negative eosinophilic granulomatosis with polyangiitis presenting with mononeuritis multiplex, intestinal hemorrhage, cardiomyopathy, fever, and worsening asthma symptoms. She was initially treated with steroids and cyclophosphamide but eventually required rituximab to control a vasculitis flare. However, her asthmatic symptoms did not improve, despite attaining vasculitis remission. Symptoms abated only after the treatment transition to mepolizumab. After a 1-year interval, there were no further episodes of asthma exacerbation and no requirement for systemic steroid therapy. This report reinforces the use of rituximab for induction and maintenance of remission in patients with eosinophilic granulomatosis with polyangiitis and predominant vasculitic manifestations, whereas mepolizumab demonstrated better control of the persistent eosinophilic manifestations, ensuing sustained remission and improved quality of life.</p>B. Cremonezi LammogliaL. de Aguiar TreviseT. Paslar LealM. Pereira Lopes Vieira PintoG. HasselmannN. Salles Rosa Neto
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2023-12-192023-12-1975410.4081/reumatismo.2023.1588Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis recurrence temporally associated with allergen-specific immunotherapy in a female adolescent: a case report
https://www.reumatismo.org/reuma/article/view/1594
<p>Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in pediatric patients. It is clinically characterized by fever flares lasting 3-7 days, reappearing every 2-8 weeks with a distinctive clockwork regularity. PFAPA generally begins before 5 years of age and usually ceases 3-5 years after onset. Recurrences may be observed in adolescence and adulthood in up to 20% of cases. The authors aim to describe a case of PFAPA recurrence in adolescence temporally associated with allergen-specific immunotherapy (ASIT). A 16-year-old female patient was referred to the rheumatology unit due to recurrent episodes of fever one month after initiating ASIT for allergic rhinitis. These episodes occurred every 4 weeks and lasted 3 days. During these episodes, she also presented with a sore throat, tonsillar exudates, and cervical lymphadenopathy. Abortive treatment with oral prednisolone was attempted in these episodes, with complete resolution of fever after a single dose. After reviewing her medical background, she had previously experienced febrile episodes accompanied by aphthous ulcers and tonsillar exudates occurring every 7-8 weeks from age 2-7. The etiopathogenesis of PFAPA remains uncertain. Environmental triggers, particularly those with immunomodulator effects, may interfere with the immune responses responsible for PFAPA occurrence, but the mechanisms are still unclear. The authors describe the first report of the reappearance of PFAPA flares, possibly due to ASIT. Further studies are needed to fully clarify if ASIT constitutes a true environmental trigger of PFAPA.</p>C. Granjo MoraisA. MartinsS. GanhãoF. AguiarM. RodriguesI. Brito
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2023-12-192023-12-1975410.4081/reumatismo.2023.1594Emergence of acute promyelocytic leukemia in a patient with granulomatosis with polyangiitis during treatment with cyclophosphamide: a rare case report
https://www.reumatismo.org/reuma/article/view/1593
<p>Granulomatosis with polyangiitis (GPA) is a rare autoimmune disease that affects multiple organs and causes inflammation, necrosis, and vasculitis in small blood vessels. Treatment for GPA involves achieving and maintaining remission. In recent studies, cyclophosphamide-based regimens have been linked to comorbidity hazards, including an increased risk of malignancies, especially hematological ones. Acute myeloid leukemia is the main hematologic malignancy that can complicate GPA. In this context, we report the case of a middle-aged woman with GPA who developed acute promyelocytic leukemia (APL) during maintenance with cyclophosphamide. She was treated with all-trans retinoic acid at 50 mg/day and arsenic trioxide at 10 mg/day, along with steroids. This case highlights the unique emergence of APL in a GPA patient during cyclophosphamide therapy. A single case has previously been reported on the development of APL in a patient with GPA while using azathioprine monotherapy for 2 years.</p>F.I. GorialN.I. AwadhN.W. ShaheenA. DheyaaZ.H. AliN. Wajdi
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2023-12-192023-12-1975410.4081/reumatismo.2023.1593Infliximab as successful treatment option in a case of adenosine deaminase 2 deficiency
https://www.reumatismo.org/reuma/article/view/1543
<p>Deficiency of adenosine deaminase 2 (DADA2) is a recessively inherited autoinflammatory disease characterized by systemic inflammation and immunodeficiency. Infliximab proved to be favorable in the treatment of this condition. This case report is concerned with a DADA2 deficient patient treated with infliximab. This is a rare case of DADA2 in a 32-year-old female patient. The patient was admitted with a clinical presentation of erythema, ulcers, and pruritus on both legs and ankles, accompanied by red ulcerative oral lesions, fatigue, malaise, and dizziness. The patient’s genetic analysis was positive for DADA2. Treatment based on TNF-α inhibition was highly effective for this patient. We used laboratory testing and punch biopsy as differential diagnostic tools, where antinuclear antibody positivity, high prolactin levels, and high serum C-reactive protein were observed. The punch biopsy revealed both orthohyperkeratosis and parahyperkeratosis of the dermis, diffuse core fragments, plasma in the stratum corneum, and hypergranulous acanthosis. DADA2 treatment is centered on tumor necrosis factor α suppression. Although high-dose systemic glucocorticoids can reduce inflammation in the initial stages of the disease, most patients have a resistant or relapsing response to tapering attempts. The prevalence of undiagnosed cases of autoinflammatory diseases is anticipated to diminish with the growing awareness of them.</p>M.B. AtesS. KarupS. Ugurlu
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2023-12-192023-12-1975410.4081/reumatismo.2023.1543Anti-N-methyl-d-aspartate receptor encephalitis: mimicker of lupus and multiple sclerosis
https://www.reumatismo.org/reuma/article/view/1598
<p>Anti-N-methyl-d-aspartate receptor encephalitis (NMDARE) is a B-cell-mediated autoimmune encephalitis with wide non-specific symptoms like acute-onset psychiatric or neurological ones mimicking various other conditions. A careful history and appropriate workup, including cerebrospinal fluid analysis for anti-NMDAR antibodies, imaging, and electroencephalogram, should be conducted, considering all differential diagnoses that can mimic its presentation. Combination therapy with high-dose steroids, plasma exchange, or immunoglobulin therapy has been shown to be more efficacious. In patients who fail first-line therapy, rituximab or cyclophosphamide should be considered. It is essential to rule out ovarian teratoma or other occult malignancies that can cause NMDARE, as removal of the tumor itself resolves this condition. Timely diagnosis and early intervention are necessary to avoid an untoward outcome.</p>N. SanghaviB. Ayesha
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2023-12-192023-12-1975410.4081/reumatismo.2023.1598Janus kinase inhibitors: between prescription authorization and reimbursability
https://www.reumatismo.org/reuma/article/view/1627
<p>Following the restrictions on the reimbursability of Janus kinase inhibitors introduced by the Italian Medicines Agency, the Italian Society of Rheumatology has drafted this document to shed light on the clinical conditions and reimbursability criteria set out in the prescription forms.</p>F.R. SpinelliF. ContiR. CaporaliF. IannoneF. Cacciapagliaon behalf of the Steering Committee of the Italian Society of Rheumatology
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2023-12-192023-12-1975410.4081/reumatismo.2023.1627Ultrasound imaging in crystal arthropathies: a pictorial review
https://www.reumatismo.org/reuma/article/view/1583
<p><em>Objective</em>. The prevalence of crystal arthropathies in the general population is rising. The purpose of this pictorial study is to describe the sonographic elements of the most prevalent crystal arthropathies by emphasizing particular sonographic findings using illustrative images and cases while considering technical details and common pitfalls.</p> <p><em>Methods</em>. Using established recommendations, specialists in the fields of sonography and crystal arthropathies agreed by consensus on the unique ultrasound signs associated with each of the conditions.</p> <p><em>Results</em>. Gout, calcium pyrophosphate deposition arthropathy, and hydroxyapatite arthropathy are the three most prevalent crystal arthropathies. Today’s high-resolution sonography enables reliable evaluation of the underlying crystal deposits, post-inflammatory changes, and a precise description of joint inflammation.</p> <p><em>Conclusions</em>. High-prevalence crystal arthropathies are reliably detectable by ultrasound with current ultrasound equipment. It is necessary to have extensive ultrasound training, know specific sonographic findings, and understand all possible differential diagnoses for disorders affecting the musculoskeletal system.</p>G. TamborriniT. HügleV. RicciG. Filippou
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2023-12-192023-12-1975410.4081/reumatismo.2023.1583