Assessment of calcinosis in Portuguese patients with systemic sclerosis - a multicenter study.

INTRODUCTION/OBJECTIVES: The study aims to define the clinical and subclinical calcinosis prevalence, the sensitivity of radiographed site and clinical method for its diagnosis, and the phenotype of Portuguese systemic sclerosis (SSc) patients with calcinosis. METHOD: A cross-sectional multicenter study was conducted with SSc patients fulfilling Leroy/Medsger 2001 or ACR/EULAR 2013 classification criteria, registered in the Reuma.pt. Calcinosis was assessed through clinical examination and radiographs of hands, elbows, knees, and feet. Independent parametric or non-parametric tests, multivariate logistic regression, and sensitivity calculation of radiographed site and clinical method for calcinosis detection were performed. RESULTS: We included 226 patients. Clinical calcinosis was described in 63 (28.1%) and radiological calcinosis in 91 (40.3%) patients, of which 37 (40.7%) were subclinical. The most sensitive location to detect calcinosis was the hand (74.7%). Sensitivity of the clinical method was 58.2%. Calcinosis patients were more often female (p = 0.008) and older (p < 0.001) and had more frequently longer disease duration (p < 0.001), limited SSc (p = 0.017), telangiectasia (p = 0.039), digital ulcers (p = 0.001), esophageal (p < 0.001) and intestinal (p = 0.003) involvements, osteoporosis (p = 0.028), and late capillaroscopic pattern (p < 0.001). In multivariate analysis, digital ulcers (OR 2.63, 95% CI 1.02-6.78, p = 0.045) predicted overall calcinosis, esophageal involvement (OR 3.52, 95% CI 1.28-9.67, p = 0.015) and osteoporosis (OR 4.1, 95% CI 1.2-14.2, p = 0.027) predicted hand calcinosis, and late capillaroscopic pattern (OR 7.6, 95% CI 1.7-34.9, p = 0.009) predicted knee calcinosis. Anti-nuclear antibody positivity was associated with less knee calcinosis (OR 0.021, 95% CI 0.001-0477, p = 0.015). CONCLUSIONS: Subclinical calcinosis high prevalence suggests that calcinosis is underdiagnosed and radiographic screening might be relevant. Multifactorial pathogenesis may explain calcinosis predictors' variability. Key Points • Prevalence of subclinical calcinosis in SSc patients is substantial. • Hand radiographs are more sensitive to detect calcinosis than other locations or clinical method. • Digital ulcers were associated with overall calcinosis, esophageal involvement and osteoporosis were associated with hand calcinosis, and late sclerodermic pattern in nailfold capillaroscopy was associated with knee calcinosis. • Anti-nuclear antibody positivity may be a protective factor for knee calcinosis.

Effectiveness of switching between TNF inhibitors in patients with axial spondyloarthritis: is the reason to switch relevant?

BACKGROUND: To investigate whether the reason to discontinue the first TNF inhibitor (TNFi) affects the response to the second TNFi in axial spondyloarthritis (axSpA). METHODS: Patients with axSpA from the Rheumatic Diseases Portuguese Register (ReumaPt), who discontinued their first TNFi and started the second TNFi between June 2008 and May 2018, were included. Response was assessed by the Ankylosing Spondylitis Disease Activity Score (ASDAS) clinically important improvement (ASDAS-CII), major important improvement (ASDAS-MI), low disease activity (ASDAS-LDA), and inactive disease (ASDAS-ID). The reason for discontinuation of the first TNFi was defined, according to ASDAS-CII as primary failure (no response ≤ 6 months), secondary failure (response ≤ 6 months but lost thereafter), adverse events, and others. The association between the reason for discontinuation of the first TNFi and response to the second TNFi over time was assessed in multivariable generalized equation (GEE) models. RESULTS: In total, 193 patients were included. The reason for discontinuation of the first TNFi did not influence the response to the second TNFi, according to the ASDAS-CII. However, a difference was found with more stringent outcomes, e.g., there was a higher likelihood to achieve ASDAS-ID with the second TNFi for patients discontinuing the first TNFi due to secondary failure (OR 7.3 [95%CI 1.9; 27.7]), adverse events (OR 9.1 [2.5; 33.3]), or other reasons (OR 7.7 [1.6; 37.9]) compared to primary failure. CONCLUSION: Patients with axSpA with secondary failure to their first TNFi, compared to those with primary failure, have a better response to the second TNFi according to stringent outcomes.

Adherence to the recommended prevention strategies before and after a hip fragility fracture: what makes us go blind?

OBJECTIVES: Our main objective was to evaluate the percentage of patients under anti-osteoporotic treatment (OT) at the time of hip fracture (HF) and one and four years after the HF event. We compared these results with the percentage of patients who should be under treatment at all three stages, according to the recently published Portuguese cost-effectiveness recommendations (PCER) for OT. Data regarding occurrence of new fragility fractures and mortality were also determined, one and four years after the HF event. Our secondary objective was to evaluate characteristics of patients associated with OT at the time of hip fracture.. MATERIAL AND METHODS: Patients hospitalized due to HF between May 1st and October 31st of 2013 in a single tertiary hospital, were selected for this study. Data regarding demographic, clinical features (including the clinical risk factors for fracture considered by FRAX®), level of independence in daily activities (Katz index), comorbidity (Charlson index) and OT were recorded at the time of the HF. The subsequent risk of fracture was estimated for each patient with FRAX® (without mineral bone density). Mortality and the percentage of patients receiving an OT prescription and suffering a new osteoporotic fracture, at one and four years after the HF event, were established. RESULTS: One hundred and thirty patients were included, with a mean age of 81.6±8.6 years. At the time of the HF only 28(21.5%) of the patients were receiving some form of OT. According to PCER, 115(88.5%) of these patients should be undergoing treatment according to FRAX® estimated risk, 30(23.1%) based on previous fractures and 119(91.5%) based on either criteria. The score of comorbidities was negatively associated with the prescription of OT at baseline (OR=0.17 [0.05-0.53], p=0.011) while the level of independence in daily activities was associated with higher probability of being treated (OR=3.20 [1.30-7.89], p=0.003). At one year after the HF, 39/130(30%) of patients had died. Although, according to PCER, all the remaining patients should be under OT based on the history of HF, only 11/91(12.1%) had received an OT prescription and 5/91(5.5%) suffered a new osteoporotic fracture during this period. At four years after the HF, 65/130 (50%) of patients had died. Only 6 of the remaining 65 (9.2%) were receiving an OT prescription and 9/65(13.8%) had suffered an additional fractured. CONCLUSIONS: Similar to other countries, the percentage of patients receiving OT at the time and especially after a HF is extremely low. Risk estimations with FRAX® and application of current PCER should allow clinicians to introduce appropriate primary and secondary preventive measures. Comorbidities and dependence seem to be important reasons for this undertreatment.

Implementing aeioTU: quality improvement alongside an efficacy study-learning while growing.

Effectiveness trials of increasing childhood development interventions across low- and middle-income countries have shown significant variability. The strength and consistency of benefits for children are dependent on program quality, and this requires paying attention to program implementation. In this paper, we summarize findings on program quality and teacher practices and perceptions for the aeioTU program, a center-based Reggio-inspired program in Colombia, now serving more than 13,000 children. The research found engaged, committed staff who valued the emergent approach and understood the children as requiring opportunities to express themselves, being the source for the curriculum, and having relationships with the materials around them. Although the average classroom quality was low in 2011, it increased significantly by 2014, particularly in the language and reasoning and interactions items. Indicator-level analyses showed that higher-order interactions and language processes were observed in a large proportion of classrooms by 2014. Teachers' self-reports on the environment and their teaching and learning showed high levels of quality by 2013. These findings illustrate the significance of process data for program improvement, especially when a program is young. Program quality can be raised after teachers improve their skills, have experience enacting a curriculum, and after training has been strengthened in response to information, while simultaneously scaling up the program.

Chylous ascites in a patient with an overlap syndrome: a surprising response to rituximab.

The authors present a case of a 51-year-old woman with clinical diagnosis of mixed connective tissue disease and overlap systemic lupus erythematosus features, with a 6-month history of progressive painless abdominal distension. On examination, evident signs of ascites were present. Both the abdominal-pelvic ultrasound and CT scan confirmed a large amount of ascites. A diagnostic paracentesis was performed, which revealed typical features of chylous ascites (CA). An extensive diagnostic work-up led by a multidisciplinary team was performed, excluding malignancy, cirrhosis, infectious, as well as cardiac and primary lymphatic causes. The patient was kept under surveillance, with dietary therapy and periodic ascitic drainages. The hypothesis of an autoimmune cause for CA was considered by exclusion. Rituximab therapy was initiated and an excellent response was achieved, with reduction of the rate of accumulation of CA and an increase in quality of life of the patient.

Portuguese Recommendations for the use of biological therapies in patients with rheumatoid arthritis- 2016 update.

OBJECTIVE: To update the recommendations for the treatment of Rheumatoid Arthritis (RA) with biological therapies, endorsed by the Portuguese Society of Rheumatology (SPR). METHODS: These treatment recommendations were formulated by Portuguese rheumatologists based on literature evidence and consensus opinion. At a national meeting the 10 recommendations were discussed and updated. The document resulting from this meeting circulated to all Portuguese rheumatologists, who anonymously voted online on the level of agreement with the recommendations. RESULTS: These recommendations cover general aspects as shared decision, prospective registry in Reuma.pt, assessment of activity and RA impact and treatment objective. Consensus was also achieved regarding specific aspects as initiation of biologic therapy, assessment of response, switching and definition of persistent remission. CONCLUSION: These recommendations may be used for guidance of treatment with biological therapies in patients with RA. As more evidence becomes available and more therapies are licensed, these recommendations will be updated.

Portuguese recommendations for the use of methotrexate in rheumatic diseases - 2016 update.

BACKGROUND: Methotrexate (MTX) is the first-line drug in the treatment of rheumatoid arthritis (RA) and the most commonly prescribed disease modifying anti-rheumatic drug. Moreover, it is also used as an adjuvant drug in patients under biologic therapies, enhancing the efficacy of biologic agents. OBJECTIVES: To review the literature and update the Portuguese recommendations for the use of MTX in rheumatic diseases first published in 2009. METHODS: The first Portuguese guidelines for the use of MTX in rheumatic diseases were published in 2009 and were integrated in the multinational 3E Initiative (Evidence Expertise Exchange) project. The Portuguese rheumatologists based on literature evidence and consensus opinion formulated 13 recommendations. At a national meeting, the recommendations included in this document were further discussed and updated. The document resulting from this meeting circulated to all Portuguese rheumatologists, who anonymously voted online on the level of agreement with the updated recommendations. RESULTS: Results presented in this article are mainly in accordance with previous guidelines, with some new information regarding hepatitis B infection during MTX treatment, pulmonary toxicity monitoring, hepatotoxicity management, association with hematologic neoplasms, combination therapy and tuberculosis screening during treatment. CONCLUSION: The present recommendations combine scientific evidence with expert opinion and attained desirable agreement among Portuguese rheumatologists. The regular update of these recommendations is essential in order to keep them a valid and useful tool in daily practice.