Effects of zoledronic acid therapy in fibrous dysplasia of bone: a single-center experience

ABSTRACT Objective: Fibrous dysplasia (FD) is a rare bone disorder that can involve any part of the skeleton, leading to bone pain, deformities, and fractures. Treatment with intravenous bisphosphonates has been used with variable results. Therefore, we aimed to evaluate the effects of zoledronic acid (ZA) therapy in patients with monostotic or polyostotic FD. Subjects and methods: The medical records of thirteen patients with FD evaluated between 2015 and 2020 were retrospectively analyzed. In the subgroup of patients treated with ZA (n = 7), data on pain relief, changes in bone turnover markers (BTMs), and adverse events following ZA infusions were retrieved. Moreover, radiological changes in response to treatment were recorded in patients who underwent radiological follow-up. Results: Of the patients, 5 (38%) presented with monostotic whereas 8 (62%) had polyostotic FD. Bone pain was a common finding (69%), and most patients (62%) exhibited elevated baseline BTMs. Partial or complete pain relief was reported in 6 of 7 patients treated with ZA. BTMs, especially C-telopeptide of type I collagen (CTX), significantly decreased after therapy (change rate: −61.8% [IQR −71, −60%]), and median CTX levels were significantly lower than at baseline (0.296 ng/mL [0.216, 0.298] vs. 0.742 ng/mL [0.549, 0.907], respectively; P = 0.04). No radiological improvement was observed in cases with radiological follow-up (n = 3). No serious adverse effects of ZA were reported. Conclusion: ZA treatment was well tolerated and provided beneficial effects in relieving bone pain and reducing BTMs, especially CTX. Our data reinforce the role of ZA in the treatment of FD-related bone pain.

The Brazilian Society of Rheumatology recommendations on investigation and diagnosis of systemic autoimmune myopathies

Abstract Background: This research is recommended by the Myopathy Committee of the Brazilian Society of Rheumatology for the investigation and diagnosis of systemic autoimmune myopathies. Body: A systematic literature review was performed in the Embase, Medline (PubMed) and Cochrane databases, including studies published until October 2018. PRISMA was used for the review, and the articles were evaluated, based on the Oxford levels of evidence. Ten recommendations were developed addressing different aspects of systemic autoimmune myopathy investigation and diagnosis. Conclusions: The European League Against Rheumatism/ American College of Rheumatology (EULAR/ACR) classification stands out for the diagnosis of systemic autoimmune myopathies. Muscular biopsy is essential, aided by muscular magnetic resonance images and electroneuromyography in complementary research. Analysis of the factors related to prognosis with the evaluation of extramuscular manifestations, and comorbidities and intense investigation regarding differential diagnoses are mandatory.

Guidelines of the Brazilian Society of Rheumatology for the treatment of systemic autoimmune myopathies

Abstract Background: Recommendations of the Myopathy Committee of the Brazilian Society of Rheumatology for the management and therapy of systemic autoimmune myopathies (SAM). Main body: The review of the literature was done in the search for the Medline (PubMed), Embase and Cochrane databases including studies published until June 2018. The Prisma was used for the systematic review and the articles were evaluated according to the levels of Oxford evidence. Ten recommendations were developed addressing the management and therapy of systemic autoimmune myopathies. Conclusions: Robust data to guide the therapeutic process are scarce. Although not proven effective in controlled clinical trials, glucocorticoid represents first-line drugs in the treatment of SAM. Intravenous immunoglobulin is considered in induction for refractory cases of SAM or when immunosuppressive drugs are contra-indicated. Consideration should be given to the early introduction of immunosuppressive drugs. There is no specific period determined for the suspension of glucocorticoid and immunosuppressive drugs when individually evaluating patients with SAM. A key component for treatment in an early rehabilitation program is the inclusion of strengthbuilding and aerobic exercises, in addition to a rigorous evaluation of these activities for remission of disease and the education of the patient and his/her caregivers.

Coorte Sarar: atividade de doença, capacidade funcional e dano radiológico em pacientes com artrite reumatoide submetidos à artroplastia total de quadril e joelho / Sarar cohort: disease activity, functional capacity, and radiological damage in rheumatoid arthritis patients undergoing total hip and knee arthroplasty

RESUMOObjetivos:A coorte Sarar é composta por pacientes portadores de artrite reumatoide (AR) e artrite idiopática juvenil (AIJ) submetidos a artroplastias de quadril e joelho no hospital Sarah-Brasília. O objetivo deste estudo foi avaliar fatores clínicos e laboratoriais associados à atividade de doença, capacidade funcional e dano radiológico em pacientes com AR, participantes dessa coorte.Métodos:Estudo transversal, com coleta de dados em revisão de prontuário.Resultados:Foram incluídos 32 pacientes, com tempo médio de início da doença de 240 meses. Dezenove pacientes foram submetidos a ATJ e 17, a ATQ. Foi encontrada correlação positiva entre dose máxima de metotrexato (MTX) durante a evolução e Clinical Disease Activity Index (CDAI) (R = -0,46, p = 0,011) e negativa com Simplified Erosion and Narrowing Score (SENS) (R = -0,58, p = 0,004). Valores de SENS foram maiores nos pacientes com fator reumatoide (FR) (p = 0,005) e anticorpo antipeptídeo cíclico citrulinado 3 (anti-CCP3) positivo (p = 0,044), nos com maiores títulos de FR (p = 0,037) e anti-CCP3 (p = 0,025) e menores nos pacientes com história familiar de AR (p = 0,009). Valores de HAQ foram maiores em pacientes mais idosos (p = 0,031). Na regressão linear múltipla, somente “dose máxima de MTX” e “história familiar” permaneceram com associação significativa com SENS (r2= 0,73, p < 0,001 para ambas as variáveis). No modelo que avaliou CDAI, apenas “dose máxima de MTX” permaneceu com associação significativa (r2 = 0,35, p = 0,016).Conclusão:Na coorte Sarar, fatores clínicos e laboratoriais estiveram relacionados à atividade de doença, capacidade funcional e dano radiológico, semelhantemente a estudos que avaliaram pacientes com menor tempo de doença.

ABSTRACTObjectives:The Sarar cohort consists of patients with rheumatoid arthritis and juvenile idiopathic arthritis who underwent hip or knee arthroplasties at hospital SARAH-Brasília. The objective of this study was to evaluate clinical and laboratory factors associated with disease activity, functional capacity and radiological damage in rheumatoid arthritis patients, participants in this cohort.Methods:Cross-secal study, with data collection achieved from medical records review.Results:Thirty-two patients were included, with a mean time of disease onset of 240 months. Nineteen patients underwent total knee and 17 total hip arthroplasty. There was a positive correlation between maximum dose of methotrexate and Clinical Disease Activity Index (R = −0.46, p = 0.011), and a negative one with Simplified Erosion and Narrowing Score (R = −0.58, p= 0.004). Simplified Erosion and Narrowing Score values were higher in patients with rheumatoid factor (p = 0.005) and anti-cyclic citrullinated peptide antibody 3 positivity (p = 0.044), in those with higher rheumatoid factor (p = 0.037) and anti-cyclic citrullinated peptide antibody 3 (p = 0.025) titers, and lower in patients with family history of rheumatoid factor (p = 0.009). Health Assessment Questionnaire values were higher in older patients (p = 0.031). In multiple linear regression, only “maximum dose of methotrexate” and “family history” remained with significant association with Simplified Erosion and Narrowing Score (r2 = 0.73, p < 0.001 for both variables). In the model evaluating “Clinical Disease Activity Index” only “maximum dose of methotrexate” remained significantly associated (r2 = 0.35, p = 0.016).Conclusion:In the Sarar cohort, clinical and laboratory factors were related to disease activity, functional capacity and radiological damage, similar to studies evaluating patients with lower disease duration.

Incidência de infecções em artroplastias de quadril e joelho em pacientes com artrite reumatoide e osteoartrite / Incidence of infectious complications in hip and knee arthroplasties in rheumatoid arthritis and osteoarthritis patients

INTRODUÇÃO: A artrite reumatoide (AR) é uma das principais indicações de artroplastia total do quadril (ATQ) ou joelho (ATJ). Estudos internacionais indicam a AR como fator de risco para infecções de prótese. OBJETIVOS: Comparação entre pacientes com AR e osteoartrite (OA) de outras etiologias em relação à incidência de infecções de prótese, infecções incisionais e outras infecções sistêmicas pós-operatórias em ATQ e ATJ. MÉTODOS: Coorte retrospectiva, comparativa, de pacientes acompanhados após a realização de ATQ ou ATJ no Hospital SARAH-Brasília, no período entre 1996 e 2007. RESULTADOS: Setenta e cinco artroplastias em pacientes com AR foram identificadas, das quais 28 ATJ e 47 ATQ. Como controles, foram selecionadas 131 cirurgias em pacientes com OA, das quais 56 ATJ e 75 ATQ, de maneira aleatória e estratificada pela cirurgia e pelo gênero. Não houve diferenças significativas entre os grupos de AR e OA no que diz respeito às taxas de infecções de prótese (respectivamente, ATJ 7,1% vs. 0% e ATQ 2,1% vs. 0%, ambos com P > 0,1), infecção incisional (ATJ 14,3 vs. 3,3% e ATQ 4,3 vs. 1,3%, ambos com P > 0,1) e infecção sistêmica (ATJ 7,1 vs. 3,6%, P = 0,92 e ATQ 4,3 vs. 10,7%, P > 0,1). Após regressão logística múltipla, não houve alteração dos resultados. CONCLUSÕES: A presença de AR não foi identificada como fator de risco para infecções perioperatórias em ATQ e ATJ em casuística do Hospital SARAH-Brasília, em comparação com o grupo de pacientes com OA primária ou secundária a doenças não inflamatórias. A baixa incidência de infecções em ambos os grupos pode explicar os nossos achados.

INTRODUCTION: Rheumatoid arthritis (RA) is one of the major indications of total hip (THA) or knee (TKA) arthroplasty. International studies have suggested that RA is a risk factor for prosthesis infections. OBJECTIVES: To compare patients with RA and patients with osteoarthritis (OA) of other etiologies with regard to the incidence of prosthesis, incisional, and other systemic postoperative infections in THA and TKA. METHODS: Retrospective, comparative cohort of patients followed up after undergoing THA or TKA at the Hospital SARAH-Brasília, from 1996 to 2007. RESULTS: Seventy-five arthroplasties (28 TKA and 47 THA) were identified in RA patients. As controls, 131 surgeries (56 TKA and 75 THA) in OA patients were randomly selected and stratified by surgery and gender. No significant difference was observed between the RA and OA groups regarding the rates of prosthesis infections (TKA 7.1% vs. 0% and THA 2.1% vs. 0%, respectively, both with P > 0.1), incisional infections (TKA 14.3% vs. 3.3% and THA 4.3 vs. 1.3%, respectively, both with P > 0.1), and systemic infections (TKA 7.1% vs. 3.6%, P = 0.92 and THA 4.3% vs. 10.7%, P > 0.1, respectively). After multiple logistic regression, the results did not change. CONCLUSIONS: RA was not identified as a risk factor for perioperative infections in THA and TKA in this case series of the Hospital SARAH-Brasília, as compared with the group of patients with primary OA or OA secondary to non-inflammatory diseases. The low incidence of infections in both groups may explain our findings.

Freqüência de síndrome metabólica em pacientes com nefrite lúpica / Frequency of metabolic syndrome in patients with lupus nephritis

INTRODUÇÃO: Os pacientes com lúpus eritematoso sistêmico (LES) apresentam morbimortalidade importante por doenças cardiovasculares (DCV). A síndrome metabólica (SM) é um transtorno complexo representado por um conjunto de fatores de risco para DCV. OBJETIVOS: Avaliar a prevalência de SM em uma coorte de pacientes com nefrite lúpica em seguimento em um hospital universitário brasileiro, e o seu impacto na doença, além da freqüência de cada fator de risco cardiovascular analisado individualmente. MÉTODOS: Quarenta e seis pacientes com nefrite lúpica foram estudados e classificados de acordo o critério para SM da National Cholesterol Education Program - Adult Treatment Panel III (NCEP-ATP III). RESULTADOS: A SM esteve presente em 30,4 por cento dos pacientes. Foi observada correlação linear entre a presença de SM e níveis baixos de depuração de creatinina e valores altos de creatinina sérica (p < 0,04 e p < 0,008, respectivamente). Não houve correlação com a classificação de nefrite lúpica, duração da doença, dose cumulativa de prednisona, proteinúria de 24 horas e com o escore Systemic Lupus International Collaborating Clinics (SLICC). CONCLUSÃO: Os pacientes brasileiros com nefrite lúpica estudados apresentam alta prevalência de SM, comparável a de estatísticas internacionais. Os únicos fatores relacionados à SM nessa população foram os que definem a presença de dano renal crônico.

INTRODUCTION: patients with systemic lupus erythematosus (SLE) have n increased morbidity due to cardiovascular diseases (CVD). Metabolic syndrome (MS) is a complex syndrome composed by a cluster of cardiovascular risk factors. OBJECTIVES: study the prevalence of MS in a cohort of Brazilian patients with lupus nephritis in a university hospital; evaluate its impact in disease outcome, and the frequency of each specific risk factor. METHODS: 46 patients with lupus nephritis were studied and defined as having or not MS in accordance with the National Cholesterol Education Program - Adult Treatment Panel III (NCEP-ATP III) criteria. RESULTS: MS was present in 30.4 percent of patients. There was significant association between MS presence and low levels of creatinine clearance and high levels of blood creatinine (p < 0.04 and p < 0.008, respectively). The class of lupus nephritis, the disease duration, cumulative dose of prednisone, 24-hour urine protein and Systemic Lupus International Collaborating Clinics (SLICC) score were not significantly associated with MS. CONCLUSION: In our cohort of patients with lupus nephritis there was a high prevalence of MS, comparable to international data. The only factors associated with MS in that population were the ones that define chronic renal damage.