The impact of a Fracture Liaison Service after 3 years on secondary fracture prevention and mortality in a Portuguese tertiary center.

Despite the establishment of Fracture Liaison Services (FLS) worldwide, no study has evaluated their impact on the Portuguese population. Our work has shown that the implementation of an FLS is associated with a significant increase in OP treatment and a lower risk of secondary fracture. PURPOSE: Fracture Liaison Services (FLS) have been established worldwide, with positive effects on treatment, secondary fracture, mortality, and economic burden. However, no study has evaluated their impact on the Portuguese population. Therefore, we purposed to evaluate the effect of an FLS model in a Portuguese center on osteoporosis (OP) treatment, secondary fracture, and mortality rates, 3 years after a fragility fracture. METHODS: Patients over 50 years old, admitted with a fragility fracture, between January 2017 and December 2020, were included in this retrospective study. Patients evaluated after FLS implementation (2019-2020) were compared with those evaluated before (2017-2018) and followed for 36 months. Predictors of secondary fracture and mortality were assessed using a multivariate Cox regression model, adjusted to potential confounders. RESULTS: A total of 551 patients were included (346 before and 205 after FLS). The FLS significantly increased the rate of OP treatment, when compared with standard clinical practice (8.1% vs 77.6%). During follow-up, the secondary fracture rate was 14.7% and 7.3%, before and after FLS, respectively. FLS was associated with a lower risk of secondary fracture (HR 0.39, C.I. 0.16-0.92). Although we observed a lower mortality rate (25.1% vs 13.7%), FLS was not a significant predictor of survival. CONCLUSION: Implementing the FLS model in a Portuguese center has increased OP treatment and reduced the risk of secondary fracture. We believe that our work supports adopting FLS models in national programs.

High patient global assessment scores in patients with rheumatoid arthritis otherwise in remission do not reflect subclinical inflammation.

OBJECTIVES: To assess whether high patient global assessment (PGA) scores by patients with rheumatoid arthritis (RA) otherwise in remission reflect subclinical inflammation. METHODS: Cross-sectional, single-center study, including consecutive RA patients. Remission states were defined based on the ACR/EULAR Boolean definition: 4V-remission (tender and swollen 28-joint counts (TJC28/SJC28), C-reactive protein (CRP), and PGA all≤1), PGA-near-remission (the same, except PGA>1), and non-remission (any of TJC28, SJC28, CRP>1). A blinded expert musculoskeletal ultrasonographer scanned 44 joints, 38 tendon sheaths, 4 bursae on the same day of the clinical evaluation. Each structure was assessed for the presence of Grey Scale synovial hypertrophy (GS) and Power Doppler (PD), both scored using a semi-quantitative scale (0-3 points). The Global OMERACT-EULAR Synovitis Score (GLOESS, 0-132, primary outcome), and a global tenosynovitis/bursitis score (GTBS) were compared between remission states, using non-parametric tests. Different sensitivity analyses comparing GS and PD subscores were performed. RESULTS: In total, 130 patients (mean age 63 years, 86% female, average disease duration 14 years) were included 40 being in 4V-remission, 40 in PGA-near-remission, 50 in non-remission. 4v-remission and PGA-near-remission presented similar median (IQR) GLOESS, [6 (5-11) and 4 (1-7), P>0.05, respectively] and GTBS [0 (0-1) and 0 (0-2), P>0.05, respectively]. The same was observed in GS, PD scores, and in global synovitis score considering only the 16 joints not included in 28-joint counts. These observations were confirmed in patients with≤5 years disease duration. CONCLUSIONS: Subclinical inflammation is not present among persons with elevated PGA who are otherwise in remission. PGA-near-remission patients would be exposed to the risk of overtreatment if current treatment recommendations were strictly followed. This study supports the need to reconsider the role of PGA in definitions used to target immunosuppressive therapy and to provide a separate and enhanced focus to the patient's experience of the disease.

Unusual association between erythema nodosum and autoimmune atrophic gastritis.

We report a case of a 46-year-old woman with a history of autoimmune atrophic gastritis and recurrent erythema nodosum (EN). Laboratory results showed iron-deficiency anaemia, positive antiparietal cell antibodies and marginal deficiency of vitamin B12. Although EN was refractory to general measures (rest and non-steroidal anti-inflammatory drugs) and corticosteroid therapy, it was successfully treated with vitamin B12 supplementation.

Influence of the different "patient global assessment" formulations on disease activity score by different indices in rheumatoid arthritis.

Patient global assessment (PGA) is included in almost all rheumatoid arthritis (RA) composite disease activity indices and definitions of remission. However, different PGA formulations exist and are used interchangeably in research and clinical practice. We investigated how five different PGA formulations used in four disease indices affect the remission rates. This was an ancillary analysis of data from a cross-sectional study in patients with RA. The data comprised the following: 28-joint counts, C-reactive protein, and five PGA formulations. Remission rate variation was assessed using five PGA formulations in each index (ACR/EULAR Boolean, CDAI, SDAI, and DAS28-CRP). PGA agreement was assessed by the following: Pearson's correlation; Bland-Altman plots; paired samples t test; and establishing the proportion of patients who scored (i) all formulations within an interval of 20 mm and (ii) each formulation ≤ 10 mm. This analysis included 191 patients. PGA formulations presented good correlations (≥ 0.65), but Bland-Altman plots showed clinically significant differences, which were statistically confirmed by comparison of means. Just over a half (51.8%) of patients scored all PGA formulations within a 20-mm interval. The proportion of those scoring ≤ 10 mm varied from 11.5 to 16.2%. When different formulations of PGA were used in each index, remission differences of up to 4.7, 4.7, 6.3, and 5.2% were observed. When formulations were used in their respective indices, as validated, the remission rates were similar (13.1, 13.6, 14.1, and 18.3%). Using PGA formulations interchangeably may have implications in the assessment of disease activity and in the attainment of remission, and this can impact upon management decisions.

Magnetic resonance imaging in individuals at risk of rheumatoid arthritis.

Individuals with rheumatoid arthritis (RA) benefit from early diagnosis and initiation of therapy. There can be delays in both due to diagnostic uncertainties. Imaging modalities, including magnetic resonance imaging (MRI), can detect inflammation earlier than clinical examination alone in early RA patients. Furthermore, the predictive role of MRI for the future development of RA has recently been explored in 'at-risk' individuals. This review details the use of MRI in early and undifferentiated arthritis and summarises the studies to date in individuals at risk of RA.

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.

More Than Just a Case of Polymyalgia Rheumatica.

Malignant neoplasms are associated with a wide range of paraneoplastic rheumatological syndromes. These can be defined as remote effects of cancer, which are not caused by the tumor and its metastasis. The authors describe the case of an 82-year-old man, who presented with a one-month history of pain, stiffness, and functional limitation of the scapular and pelvic girdles. Blood tests showed raised levels of inflammatory markers. He was diagnosed with Polymyalgia Rheumatica, but the lack of response to corticosteroids led to the suspicion of an alternative diagnosis, like, for example, an occult neoplasm. Although patient evaluation was initially normal, five months later he developed macroscopic haematuria and was diagnosed with bladder and prostate carcinomas. After surgical treatment, the patient fully recovered from his rheumatological syndrome. LEARNING POINTS: In cases when polymyalgia rheumatica exhibits atypical clinical features and/or therapeutic resistance, additional investigation is recommended.Paraneoplastic syndromes can lead to the diagnosis of an underlying neoplasm.

Management of infections in rheumatic patients receiving biological therapies. The Portuguese Society of Rheumatology recommendations.

INTRODUCTION: Infections are a major cause of morbidity and mortality in systemic inflammatory rheumatic diseases and the management of infectious complications in patients under biological therapies deserves particular attention. OBJECTIVE: Develop evidence-based recommendations for the management of infections in rheumatic patients receiving biological therapies. METHODS: A search in PubMed (until 10 November 2014) and EMBASE (until 20 December 2014) databases was performed. Patients with systemic inflammatory rheumatic diseases treated with approved biologics in whom infections occurred were included. Search results were submitted to title and abstract selection, followed by detailed review of suitable studies. Information regarding presentation of the infectious complication, its diagnosis, treatment, and outcome, as well as maintenance or discontinuation of the biological agent was extracted and subsequently pooled according to the type of infection considered. Results of literature review were presented and critically reviewed in a dedicated meeting by a multidisciplinary panel. Recommendations were then formulated using the Delphi method. Finally, the level of agreement among rheumatologists was voted using an online survey. RESULTS: Fifteen recommendations were issued. Nine general recommendations concerned the assessment of infectious risk before and while on biologics, the procedures in case of suspected infection and the management of biologics during infectious complications. Six specific recommendations were developed for respiratory, urinary, gastrointestinal, skin, osteoarticular and disseminated infections. CONCLUSION: These fifteen recommendations are intended to help rheumatologists in the management of infections in patients on biological therapy. They integrate an extensive literature review, expert opinion and inputs from Portuguese rheumatologists.

Sclerosing Mesenteritis and Disturbance of Glucose Metabolism: A New Relationship? A Case Series.

BACKGROUND: Sclerosing mesenteritis is an idiopathic inflammatory and fibrotic disease that affects the mesentery. It is a rare disease, with the total number of reported cases in the literature ranging from 122 to 300. It mainly affects men in the sixth decade of life, and its etiology remains unknown. Clinical presentation is variable, but it is frequently asymptomatic. Diagnosis is often made by computed tomography (CT) scan, although biopsy may be needed for confirmation. An association between other diseases (e.g., neoplasms) and sclerosing mesenteritis has been described, but the relationship between the latter and glucose changes is not disclosed in the currently available literature. CASE REPORT: Five cases of sclerosing mesenteritis and glucose metabolism disorders (impaired fasting glucose and type 2 diabetes mellitus) were retrospectively collected and analyzed. The mean age was 65 ± 9.3 years, 80% were male, and all patients were white. Three patients were asymptomatic and the other 2 (40%) had non-specific chronic abdominal pain. Blood tests revealed normal inflammatory parameters (mean HbA1c was 6.4% and fasting blood glucose was 140 mg/dL). The diagnosis was made by abdominal CT scan. The 2 symptomatic patients underwent therapy with colchicine 1 mg/day, with clinical improvement. During the mean 43-month follow-up period, there was no symptomatic progression, thereby maintaining the usual benign course of this condition. CONCLUSIONS: Sclerosing mesenteritis has only been described in small series and isolated cases, but its diagnosis is becoming more common due to greater access to diagnostic methods and higher awareness of the disease in the medical community. Furthermore, despite the small sample size, we describe a possible association between glucose metabolism impairment and sclerosing mesenteritis.

[Activated C protein in the treatment of a newborn with sepsis, shock and multiple organ dysfunction systems: case report and literature review]. / Proteína C ativada no tratamento de recém-nascido com sepse, choque e disfunção de múltiplos órgãos e sistemas: relato de caso e revisão de literatura.

BACKGROUND AND OBJECTIVES: Severe sepsis represents the systemic inflammatory response resulting from an infection, associated with one of the following: cardiovascular organ dysfunction, acute respiratory distress syndrome or two or more organ dysfunctions. Although the mortality rate from sepsis in children has steadily decreased in the last decades, the mortality rate in newborns remains high (20% to 40%) despite the development in intensive care. The authors describe a newborn who suffered from sepsis, shock and multiple organ dysfunction syndromes (MODS) that recovered after the administration of activated C protein. CASE REPORT: A premature newborn underwent cesarean section because of a premature rupture of membranes and acute fetal distress. The newborn developed acute respiratory distress due to intrauterine pneumonia and was taken to the Neonatal Intensive Care Unit. The patient was given mechanical ventilation, exogenous pulmonary surfactant and antibiotics early in the treatment. Nevertheless, he developed persistent pulmonary hypertension and shock. The control of the infection was difficult, despite the adjustment of the antibiotics, resulting in the development of MODS. On the 28th day, activated C protein was given to the patient. The administration of the drug was successful and the patient recovered from the organ dysfunction without bleeding. CONCLUSIONS: The activated C protein can't be recommended as a routine in the treatment of newborns with severe sepsis. However, in this case, it contributed to the recovery of the organ dysfunctions presented by the patient.