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Copyright of Acta Reumatológica Portuguesa is the property of Sociedade Portuguesa de Reumatologia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Acta Reumatológica Portuguesa is the property of Sociedade Portuguesa de Reumatologia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Acta Reumatológica Portuguesa is the property of Sociedade Portuguesa de Reumatologia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Acta Reumatológica Portuguesa is the property of Sociedade Portuguesa de Reumatologia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Acta Reumatológica Portuguesa is the property of Sociedade Portuguesa de Reumatologia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Acta Reumatológica Portuguesa is the property of Sociedade Portuguesa de Reumatologia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: Paget's disease is a chronic progressive metabolic bone disorder that can affect one or multiple sites, but with a predilection for the axial skeleton. The majority of patients with Paget disease of bone are asymptomatic and the diagnosis is usually made incidentally. The prevalence of the disease in Portugal is unknown. Objectives: To report and describe the presentation and clinical characteristics of a rarely diagnosed rheumatic disease. Methods: Retrospective study of patients with a diagnosis of Paget's disease with follow up in the rheumatology department in a tertiary center. The demographic data, clinical features, investigation results and treatment of the cases were analyzed from medical records. Results: We identified 7 patients with Paget's disease of the bone (3 women and 4 men; mean diagnosis age 59.1±7.0 years). Four patients had bone pain at the affected site and three had unspecific arthralgias and presented as an incidental finding during the diagnostic investigation. All but one had raised serum alkaline phosphatase level with normal serum calcium levels. The majority of the patients (71.4%, n=5) had a monostotic disease and the most commonly involved site was the pelvis. All of them had hypercaption of the radiopharmaceutical agent at bone scintigraphy and alterations that were suggestive of pagetic bones at the radiographs. All of them were treated with bisphosphonates (intravenous pamidronate (n=2), oral alendronate (n= 3), oral risedronate (n=1), intravenous zoledronate (n=1)). The two patients treated with one administration of intravenous pamidronate were then treated with zoledronate or alendronate. After one year of treatment, the serum alkaline phosphatase levels have decreased in all patients. None of the patients had related complications (musculoskeletal, neurologic, and cardiovascular problems). Conclusion: The patients included are mainly men with bone pain at the site involved. The majority was a monostotic disease. All of them were treated with bisphosphonates with good clinical results. [ABSTRACT FROM AUTHOR]

Background: Several studies have consistently showed that the extent of skin involvement has a major impact on disease prognosis in the diffuse cutaneous subtype of systemic sclerosis. However, there is scarce data regarding skin course in the limited cutaneous subset (LcSSc). Objectives: Our aims were to (1) to investigate skin involvement course, and (2) identify predictors for this course in patients with LcSSc, using our cohort registry. Methods: We performed a retrospective analysis including patients with LcSSc fulfilling the 2103 ACR/EULAR Classification Criteria for Systemic Sclerosis. Skin improvement, progression or stable course was defined by a cut-off of plus or minus 3.5 points change in mRSS from baseline to last appointment. The European Scleroderma Trial and Research (EUSTAR) group Disease Activity Index (EUSTARDAI), clinical and laboratorial features were assessed. Baseline characteristics were compared between groups using chisquare and independent samples t-test, as appropriate. Logistic regression analysis was performed to identify possible predictive factors associated with skin course involvement. Results: We included 71 LcSSc patients [87.2% female, mean age at diagnosis 55.7 (SD 11.6) years]. At baseline median mRSS (IQR) was 4.0 (0-10) (Table I). The mean follow-up time was 15.5±9.2 years from baseline to last appointment. Maintenance of mRSS was observed in 65.2% (n=45), improvement in 27.6% (n=21) and progression in 7.2% (n=5) of the patients. Patients with stable mRSS were significantly older at baseline than improvers (p=0.05). In addition, these patients had more frequently digital ulcers, arthritis and positive antinuclear and anti-centromere antibodies than patients that improve, although not statistically significant. LcSSc patients with an inactive disease (EUSTAR-DAI<2.5) at baseline tend to have a stable mRSS course (p=0.001). In multivariate analysis, no predictors were identified for skin disease progression. Conclusions: Recognizing distinct patterns of mRSS course could help tailoring clinical management and cohort enrichment. Studies with a larger sample size will be needed to shed some light on skin involvement and its prognostic value in LcSSc. [ABSTRACT FROM AUTHOR]

Background: biosimilars of biotechnological agents represent an important opportunity to increase accessibility to these medications. Clinicians still maintain reservations regarding the similarity of their efficacy and safety in practice. Aim: to evaluate the clinical consequences of a nonmedical switch of etanercept (ETN) original to biosimi - lar in a clinical practice setting. Methods: the study included all patients aged 18+ treated in a Tertiary Rheumatology Department with original ETN who were switched to his biosimilar following a decison by the hospital administration, accepted by rheumatologists. Patients were informed of the switch prior to its occurrence, in simple terms underlining the similarity in terms of regulatory issues and scientific evidence. Disease activity, adverse events and adaptation to the drug delivery system were eva - luated at each visit. Disease activity at baseline (time of switch), 3 and 6 months after was compared using Paired samples T-test or Wilcoxon test as adequate. A p£0.05 was considered statistically significant. Continuous variables are presented as means and categori - cal variables as proportions. Results: from 98 patients treated with original ETN in our department, 89 were switched to his biosimilar. The remaining ones maintained the treatment with the reference biological product for several reasons. Eight patients were excluded from this analysis due to poor adherence to treatment (n=4) and early interruption of treatment [n=4: due to surgery (n=1), respiratory infection (n=1), suspected allergic reaction to biosimilar (n=1) and own initiative (n=1)]. Of the remaining 81 patients (58% female, mean age 56.2±12.1 years), 38.3% had RA, 40.7% SpA, 18.5% PsA and 2.5% JIA. Disease activity was stable over the follow up in patients with RA, PsA and SpA as no statistically significant differences were observed in acute phase reactants, patient or physician global assessment between the three time points. Minor adverse events were reported by 2 patients (pain and local cutaneous reaction), 2 reported sense of disease exacerbation in the first three months that was not confirmed by clinical and analytical evaluation and 2 patients reported minor infections. Good adaptation to the drug delivery instrument was reported by 93.8% of patients (n=76). Conclusion: the switch from ETN to his biosimilar in this group of patients followed in routine care did not affect the overall efficacy and safety of treatment. We did not observe a significant impact of nocebo effect. [ABSTRACT FROM AUTHOR]

Objectives: To summarize the published evidence in the literature on the use of intravenous immunoglobulin in gastrointestinal tract involvement in systemic sclerosis patients and report the experience of our department., Methods: A systematic literature review was performed; and a literature search was conducted in MEDLINE and Embase until 1/5/2024, using the participants, intervention, comparator and outcomes framework. Only full-text articles involving systemic sclerosis adults, submitted to intravenous immunoglobulin (at least one administration) to treat primary gastrointestinal tract manifestations. The outcome was the University of California at Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0 score to evaluate response to treatment. Two reviewers performed the assessment of data extraction and synthesis, independently., Results: Four papers (two case reports and two retrospective studies) out of 35 references were included. In addition, we added two systemic sclerosis patients from our department in this review. In 25 systemic sclerosis patients, with various gastrointestinal tract manifestations, the intravenous immunoglobulin therapy was found to improve digestive tract symptoms in SSc patients, as shown by the decrease in the scores of University of California at Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0. No adverse events were reported, except for one case of low-grade fever post-administration., Conclusion: The results from this systematic literature review based on case series suggest that intravenous immunoglobulin may improve gastrointestinal tract symptoms assessed by the University of California at Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0 scale, with only minor reported adverse events, suggestive of an acceptable safety profile. We believe that this systematic literature review will contribute to shed light on the efficacy and safety aspects of intravenous immunoglobulin treatment in the management of gastrointestinal tract symptoms; and multicenter randomized placebo-controlled trials are urgently needed to foster progress in this field., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship and/or publication of this article., (© The Author(s) 2024.)

Dermatomyositis (DM) is an immune-mediated myopathy characterized by proximal skeletal muscle weakness, muscle inflammation, and distinct skin manifestations. Calcinosis cutis, involving the deposition of insoluble calcium salts in the soft tissues, affects approximately 8% of DM patients1 and poses significant treatment challenges. It can complicate with inflammation, ulceration, pain, and local and systemic infections, resulting in considerable morbidity. We present the case of a 69-year-old-woman with dermatomyositis who developed an exuberant form of calcinosis cutis.

Objective: To develop evidence-based recommendations for the non-pharmacological and pharmacological management of Raynaud's phenomenon (RP) and digital ulcers (DUs) in patients with systemic sclerosis and other immune-mediated connective tissue diseases (CTDs)., Methods: A task force comprising 21 rheumatologists, two surgeons (vascular and plastic), two nurses, and one patient representative was established. Following a systematic literature review performed to inform the recommendations, statements were formulated and discussed during two meetings (one online and one in-person). Levels of evidence, grades of recommendation (GoR), and level of agreement (LoA) were determined., Results: Five overarching principles and 13 recommendations were developed. GoR ranged from A to D. The mean ± standard difference (SD) LoA with the overarching principles and recommendations ranged from 7.8±2.1 to 9.8±0.4. Briefly, the management of RP and DUs in patients with CTDs should be coordinated by a multidisciplinary team and based on shared decisions with patients. Nifedipine should be used as first-line therapy for RP and/or DUs. Sildenafil, tadalafil, and/or iloprost IV are second-line options for severe and/or refractory patients with RP and/or DUs. Sildenafil, tadalafil and/or Iloprost IV, should be prescribed for healing and prevention (also including bosentan) of DUs. In patients with RP and/or DUs, non-pharmacological interventions might be considered as add-ons, but there is limited quality and quantity of scientific evidence supporting their use., Conclusions: These recommendations will inform rheumatologists, specialist nurses, other healthcare professionals, and patients about a comprehensive and personalized management of RP and DUs. A research agenda was developed to address unmet needs, particularly for non-pharmacologic interventions.

Objectives: To examine the influence of contextual factors upon the evaluation of skin thickness and stiffness by ultrasound and to assess the reliability of these parameters., Methods: Ultrasound dermal thickness (by B-mode, 18MHz) and skin stiffness (by shear-wave elastography, 9MHz) were assessed in persons with systemic sclerosis (SSc) and in healthy controls. The influence of contextual factors upon repeated measures was evaluated: (i) room temperature (16-17ºC vs. 22-24ºC); (ii) time of day (morning vs. afternoon), and (iii) menstrual cycle phase (menstrual vs. ovulatory). Differences were analysed using the related-samples Wilcoxon signed-rank test. Inter- and intra-rater reliability of ultrasound skin thickness and stiffness were evaluated in the 17 skin Rodnan sites of 20 persons with SSc and 20 healthy controls, under stable contextual conditions., Results: A significant increase in ultrasound dermal thickness was observed at the leg in the afternoon vs morning, in both patients and controls. Similar observations were made for skin stiffness at the leg (in SSc) and at the foot (in SSc and controls) in the afternoon. No significant changes were observed in association with room temperature and menstrual cycle. Intra- and inter-rater-reliability was good to excellent for ultrasound dermal thickness and stiffness, both in SSc and healthy controls., Conclusions: The timing of the ultrasound procedure within each day seems to influence the ultrasound measures at the legs and feet. Our study corroborates that ultrasound dermal thickness and skin stiffness are reliable domains to quantify skin involvement in SSc.

Introduction: Despite years of experience with biological disease modifying anti-rheumatic drugs (bDMARD) in rheumatoid arthritis (RA), little is known about differences in infectious risk among bDMARDs. The aim of this study was to assess the incidence and type of infections in RA patients on bDMARDs and to determine possible predictors., Methods: A retrospective multicenter cohort study that included patients registered in the Rheumatic Diseases Portuguese Registry (Reuma.pt) with RA, and exposed to at least one bDMARD until April 2021. RA patients under bDMARD and with at least one episode of severe infection (SI), defined as infection that requires hospitalization, use of parenteral antibiotics or that resulted in death, were compared to patients with no report of SI. Demographic and clinical data at baseline and at the time of each SI were collected to establish comparisons between different groups of bDMARDs. Comparisons between different bDMARDs were assessed and logistic regression was performed to identify predictors of SI., Results: We included 3394 patients, 2833 (83.5%) female, with a mean age at RA diagnosis of 45.5±13.7 years. SI was diagnosed in 142 of the 3394 patients evaluated (4.2%), totaling 151 episodes of SI. At baseline, patients with SI had a significantly higher proportion of prior orthopedic surgery, asthma, interstitial lung disease, chronic kidney disease and corticosteroid use, higher mean age and longer median disease duration at first bDMARD. Nine patients died (6.0%). Ninety-two SI (60.9%) occurred with the first bDMARD, the majority leading to discontinuation of the bDMARD within 6 months (n=75, 49.7%), while 65 (43.0%) restarted the same bDMARD and 11 (7.3%) switched to another bDMARD (6 of them to a different mechanism of action). In the multivariate analysis, we found that chronic kidney disease, asthma, infliximab, corticosteroid use, interstitial lung disease, previous orthopedic surgery, higher Health Assessment Questionnaire and DAS284V-ESR are independent predictors of SI., Conclusion: This study described the incidence and types of SI among Portuguese RA patients on biologics, identifying several predictors of SI, both globally and with different bDMARDs. Physicians should be aware of the real-word infectious risk in RA patients on bDMARDs when making treatment decisions.

Background: Systemic sclerosis (SSc) is a rare connective tissue disorder with heterogeneous manifestations and outcomes. Besides differences in disease characteristics among distinct ethnic groups and geographical regions, several questions regarding the impact of the disease and the effectiveness of treatments remain unanswered. To address these questions, the Rheumatic Diseases Portuguese Register (Reuma.pt) launched a specific protocol for the prospective follow-up of SSc patients., Objectives: To describe the baseline characteristics, disease subsets, treatments used and survival of SSc patients registered in Reuma.pt/SSc., Methods: Data from adult patients with SSc included in Reuma.pt up to November 2020 were analysed. Demographic features, SSc subsets, fulfilment of classification criteria, main clinical and immunological features, comorbidities, treatments used and survival data were described and compared between diffuse cutaneous (dc) and limited cutaneous (lc) disease subsets. Survival was calculated for patients included in Reuma.pt within the first two years of diagnosis., Results: In total, 1054 patients were included, 87.5% female, with a mean age at diagnosis of 52.7 +/- 14.8 years. The most common subset was lcSSc (56.3%), followed by dcSSc (17.5%), preclinical SSc (13%), overlap syndrome (9.8%) and SSc sine scleroderma (3.3%). Raynaud's phenomenon (93.4%) and skin thickening (76.9%) were the most frequently observed clinical manifestations. Gastrointestinal (62.8% versus 47.8%), pulmonary (59.5% versus 23%) and cardiac (12.8% versus 6.9%) involvements were significantly more prevalent in dcSSc than lcSSc. Ninety per-cent of patients were Antinuclear antibody positive, 52.5% were Anti-centromere antibody positive and 21% anti-topoisomerase positive, with significant differences between lcSSc and dcSSc. One-third of patients were treated with immunomodulators, 53.6% with vasodilators, 23% with glucocorticoids and 2.3% with biologics. During follow-up, 83 deaths (7.9%) were reported. The overall 1-, 2- and 5-year survivals were 98.0%, 96.8% and 92.6%, respectively, without significant differences between lcSSc and dcSSc., Conclusion: Reuma.pt/SSc data highlights the importance of registries in improving knowledge about rare and complex diseases, such as SSc. Clinical features of Portuguese SSc patients are similar to those of other populations. In recently diagnosed patients, 5-year survival is over 92%. To the best of our knowledge, this is the first study showing that clinical features of Portuguese SSc are similar to those of other cohorts.

Pain is a common feature of most rheumatic diseases and it is often the main reason for the patient to seek for a clinical appointment. Chronic pain has a major impact on patient's quality of life, being frequently associated with functional incapacity, sleep and mood disorders. This leads to absenteeism and heavy consumption of health resources, both representing huge burdens on national economy. Managing musculoskeletal pain is pivotal but can be challenging. The use of the available pharmaceutical armamentarium should be parsimonious. Opioids are strong analgesic drugs that mostly act through their agonist action on µ-receptors in the central nervous system. Opioid-related side effects are not negligible and are mediated through both central and peripheral opioid receptors. The use of opioids is well established in the treatment of oncologic pain but their role in the management of musculoskeletal pain is still controversial. Inflammatory rheumatic diseases, osteoarthritis, osteoporotic fractures, chronic low back pain and fibromyalgia represent diverse major rheumatic conditions that frequently lead to chronic pain. In order to standardize and optimize management of musculoskeletal chronic pain in these prevalent diseases, the Portuguese Rheumatology Society elaborated this position paper. The objectives were: a) to define the importance of pain assessment and classification; b) to guide patient selection, appropriate choice of opioids, their management, and raise awareness of their adverse effects; c) to review the existent data on possible indications of opioid therapy on rheumatic diseases.