Acta Reumatológica Portuguesa

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No abstract text is available yet for this article.

BACKGROUND: Axial Spondyloarthritis (axSpA) is a chronic, inflammatory rheumatic disease that affects the axial skeleton, causing pain, stiffness, and fatigue. Genetics and environmental factors such as microbiota and microtrauma are known causes of disease susceptibility and progression. Murine models of axSpA found a decisive role for biomechanical stress as an inducer of enthesitis and new bone formation. Here, we hypothesize that muscle properties in axSpA patients are compromised and influenced by genetic background...

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Granulocyte colony-stimulating factor (G-CSF) is increasingly being used to prevent febrile neutropenia associated with chemotherapy. Large-vessel vasculitis (LVV) has been recognized as a rare side effect of G-CSF treatment. We report a case of G-CSF associated LVV in a patient with breast cancer. While clear pathogenic mechanisms remain unknown, G-CSF may cause vasculitis due to inflammatory cytokines production. This adverse reaction should be recognized in patients with suggestive symptoms following the administration of pegfilgrastim...

INTRODUCTION: Long-term survivors of cystic fibrosis (CF) have a dramatic increase in the risk of osteoporosis and incident fracture. The objective of this work is to characterize a CF related bone disease in a Portuguese cohort of CF patients. METHODS: We performed a cross-sectional, observational study on a cohort of CF adult patients. Clinical status, laboratory parametres, nutrition, lung function tests, genetics and bone mineral density (BMD) data were collected from a CF reference centre...

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Tumor necrosis factor alpha inhibitors (TNFi) are basilar treatments in a number of inflammatory rheumatic conditions and autoimmune phenomena such as de novo neuroinflammatory events were already described in these populations under TNFi. We conducted a single-center retrospective study in a cohort of rheumatic patients treated with TNFi to characterize neurological demyelinating/inflammatory disease in these patients. We report 3 cases (n= 744): all of them had spondyloarthritis, the onset of neurological manifestations occurred between 37 and 58 years old and all of them initially presented with an optic neuritis...

Systemic sclerosis (SSc) is an uncommon condition, with a wide range of manifestations, characterized by specific antibody production, vasculopathy and fibrosis of the skin and other internal organs. It is a complex disease, which is estimated to be rare in Portugal, although specific incidence data are missing. The aetiology of SSc remains unknown, but is likely to be multifactorial, involving genetic and environmental aspects. Its management is challenging and often requires a multidisciplinary approach. In 2011, we established a dedicated outpatient clinic for patients with SSc...

PURPOSE: COVID-19 changed the dynamics of all healthcare system, leading to the restructuring of inpatient teams as well as the emergency department. Scheduled surgeries were suspended, operating rooms were closed, and anesthesiologists redistributed among the various intensive care units. At the Centro Hospitalar do Baixo Vouga the number of patients admitted to the emergency department decreased to approximately 8.000 during the period of lockdown which ranged from 18th March to 1st June 2020...

BACKGROUND: Axial Spondyloarthritis (axSpA) refers to a group of rheumatic diseases that mainly affect the axial skeleton. Treatment with Biological Disease Modifying Anti-Rheumatic Drug (bDMARDs) is indicated when low disease activity is not achieved with Non-Steroid Anti-inflammatory Drugs. Certain clinical and socio-demographic features may be predictive of future need for treatment with bDMARDs in a patient with axSpA. OBJECTIVES: To study a population of patients with axSpA and determine whether the presence of certain factors at diagnosis is associated with a later need for biological treatment...

AIMS: Few reports have focused on the distal interphalangeal (DIP) joint in patients with rheumatoid arthritis (RA). The purposes of this study were to evaluate the frequency of bone erosion of the DIP joint, and to determine the factors associated with its deformity. METHODS: This study reviewed 204 patients with RA in whom radiographs of hands were obtained. According to the presence/absence of bone erosion of the DIP joint, patients were divided into two groups (DIP-positive and DIP-negative groups)...

INTRODUCTION: IgG4-related disease (IgG4-RD) is characterized by the growth of pseudotumors, which can affect almost every organ. Elevated serum IgG4 levels are present in only two-thirds of the patients and are not specific. Diagnosis is difficult and is usually based on a biopsy. CLINICAL CASE: A 39-year-old man presented complaints of low back pain and fever. A renal computed tomography (CT) scan revealed a voluminous mass next to the right kidney and a biopsy showed an inflammatory process and excluded a neoplasm...

BACKGROUND: Hand osteoarthritis (HOA) is a highly prevalent rheumatic disease that predominates in females and causes pain and loss of functional capacity. Obesity and metabolic syndrome have been previously suggested to associate with the severity of HOA, but clarity on these associations is yet to be achieved. OBJECTIVE: Test the association between obesity and other components of the metabolic syndrome and disability in women with hand osteoarthritis (HOA). DESIGN: Individuals from EpiReumaPt epidemiological community-based study (2011-2013) are representative of the Portuguese population...

OBJECTIVE: Biobanks for research (BBR) have enormous value for research, including those specifically oriented to chronic diseases. Knowing public attitudes and perceptions is key to design and implement patient-centered BBR. We assessed patient awareness, perception and choices among rheumatology outpatients regarding aging biobanking activities. METHODS: We conducted a cross-sectional survey of patients, aged 50 or older, attending an outpatient rheumatology tertiary department...

We report a case of Trichorhinophalangeal syndrome type I (TRPS1) in a 16-year-old boy who was referred due to painless finger deformities over the last year. Legg-Calvé-Perthes disease (LGP) had been diagnosed at age 7 and required surgical treatment at age 12. Parents were healthy and non consanguineous; there was family history of pectus carinatum of maternal lineage. On examination the patient presented a bulbous nose, thin and sparse scalp hair; pectus carinatum; clinodactyly of the first and fifth fingers and hard painless swelling of all of the proximal interphalangeal joints; brachydactyly of the toes...

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We present the case of a 32-year-old man with a diagnosis of type-III osteogenesis imperfecta who developed a telangiectatic osteosarcoma in the proximal right tibia. An above-knee amputation was performed and after one-year follow-up, pulmonary metastatic lesions were detected on the thoracic CT scan. Palliative chemotherapy was proposed and to date the patient is still living and is under medical treatment. The association between osteogenesis imperfecta and osteosarcoma is rare. There are only ten confirmed reports of this unusual situation, but to our knowledge this is the first case reported with a telangiectatic osteosarcoma arising in this particular setting...

The Portuguese Rheumatology Society (SPR) embraced quality as a major goal and launched, in early 2015, a program to aim for excellence in global clinical care: Rheuma SPACE - Standard Practice Aiming Clinical Excellence. Evaluating daily reality is the first step in a quality development timeline, ultimately contributing for health gains. Herein we describe the results of the evaluation of the quality indicators defined for this project and the improvement strategies identified. The Rheuma SPACE project included three phases: 1) establishing a set of quality indicators and an excellence quality model; 2) assessment of the current care at Rheumatology departments concerning the defined quality indicators in the scope of the excellence model; and 3) elaboration of global and customized reports for each participating Rheumatology department, resulting in the identification of improvement opportunities...

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