Acta reumatologica portuguesa最新文献

Aims: Ankylosing spondylitis (AS) reduces spinal mobility, which results in structural and functional impairments. Pulmonary problems eventually occur in most AS patients due to interstitial lung disease or as a result of chest wall abnormalities. The aim of this study was to evaluate the effects on pulmonary functions and disease related scales of aquatic and land-based multidimensional functional mobility exercises on pulmonary functions in patients with AS.

Methods: In this randomized controlled study, 57 patients with definite AS according to the modified New York criteria were randomly allocated to an aquatic (AG), land-based (LG), or home (HG) exercise group and performed multidimensional mobility exercise sessions twice a week for 8 weeks. The Bath indices were used to measure disease activity, functional limitation, and spinal mobility, and a 10-cm visual analog scale assessed pain during activity and at rest. Pulmonary function tests, maximal inspiratory mouth pressure (MIP), and maximal expiratory mouth pressure (MEP) were measured before and after the intervention. The study is registered at ClinicalTrials.gov, number NCT03667625 (27/08/2018).

Results: Forty-six patients (30.4% female) with a mean age of 42.0 years completed the study. Multidimensional exercises improved disease-related symptoms such as pain, spinal mobility, and functionality, but there were no significant changes in HG. Patients in AG showed significant improvements in peak expiratory flow (p=0.004), vital capacity (p=0.025), maximum voluntary ventilation (p=0.006), and MIP (p=0.001), while those in LG showed significant increases in forced expiratory volume during the first second to forced vital capacity (FEV1/FVC) ratio (p=0.049), peak expiratory flow (p=0.007), and maximum voluntary ventilation (p=0.004). There were no significant changes in HG.

Conclusions: Multidimensional functional mobility exercises performed either in water or on land are important in the management of pulmonary manifestations of AS.

Bone marrow edema syndrome is a rare disease with an unknown etiology, self-limited and usually associated with an indolent course, which can also generate severe pain with tremendous functional impairment. The authors present a case of a 19-year-old female patient with a progressive, non-traumatic and unrelentless pain involving both knees, requiring persistently walking aids and analgesic drugs. The imaging studies showed a bilateral distal femur and proximal tibia bone marrow edema in the magnetic resonance imaging. Finally, and after an extensive investigation without any abnormal findings, a bone marrow edema syndrome diagnosis was established, with a spontaneous regression of the clinical and imaging presentation. One year after the initial complaints the patient is fully recovered, without pain or medication, presenting an MRI showing complete regression of the initial findings. Despite the rarity of this entity, being aware of its existance and clinical manifestations is crutial to allow a proper diagnosis. The case herein presented is, to our understanding, pragmatic regarding bone marrow edema syndrome presentation and clinical course.

Introduction: Fragility fractures cause significant mortality and morbidity. Even though there are multiple guidelines for the management of fragility fractures, european countries still report treatment rates of less than 30%. Implementation of fracture liaison services can increase this percentage by 21%. Our goal is to describe the management of osteoporosis, in patients with hip fragility fracture treated in a portuguese hospital with no internal protocols in place.

Methods: A retrospective study was conducted. Patients treated surgically for hip fragility fracture in our hospital, during 2017, were included. Data until May 2020 was collected on osteoporosis recognition and pharmacological treatment prescription.

Results: A total of 102 patients were included, 87% female, with a mean age of 79.9±9.9 years at the time of the fracture. Pharmacological anti-osteoporotic treatment after the hip fragility fracture was prescribed in 35%. From those, 53% did not include bisphosphonates. General practice doctors were responsible for 44% of anti-osteoporotic prescriptions and "Osteoporosis" ICD10 codification in primary care was present in 10.7%.

Discussion/conclusion: We found a gap in osteoporosis treatment after a hip fragility fracture, similar to literature reports when no fracture liaison service is in place. We believe that the lack of such protocols, the low rate of "osteoporosis" or "fragility fracture" mentioning at hospital discharge, together with the under recognition at primary care level, contribute to this reality. The implementation of new measures is crucial to improve prevention and management of fragility fractures.

Psoriatic disease (Psoriasis and Psoriatic Arthritis, PsD) is a condition that affects the skin, the musculoskeletal system, and beyond, impairing patients' quality of life. A multidisciplinary approach of combined dermatology-rheumatology clinics is recommended and valuable to respond to PsD diagnosis, management, and treatment challenges. In Portugal, five Hospitals have implemented a multidisciplinary clinic for PsD assessment. This report aims to describe how these multidisciplinary clinics were developed, their characteristics, and the main obstacles to their implementation. Although the different hospitals adopted distinct functional models, a consensus respecting the minimal core set assessment for PsD in Multidisciplinary Dermatology/Rheumatology Clinics should comprise all disease manifestations and, if possible, quality of life. The main objective of these clinics is to achieve remission/minimal disease activity. Limitations to these multidisciplinary approaches are discussed, namely financial, time management, and human resources obstacles that can be a handicap in their implementation, despite the benefits of PsD integrated care.

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. Clinical data of every patient with a confirmed diagnosis of SSc is prospectively registered in Reuma.pt/SSc. In this manuscript, we aim to describe the general functioning of our SSc outpatient clinic, and to characterise the population of patients with SSc who are followed herein.

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. A follow-up CT scan at four months revealed total regression without any treatment. Three years later, the patient presented with diplopia and right proptosis. MRI of the orbits revealed a retro-ocular mass; biopsy excluded a malignant process. Infectious, autoimmune or paraneoplastic diseases were also excluded. Although the patient's IgG4 serum levels were normal, histopathological reobservation of the renal biopsy revealed IgG4-positive plasma cells, thus confirming the diagnosis. The patient was prescribed a daily dose of 40 mg of prednisolone and regression of the right orbital lesion was observed.

Discussion: IgG4-RD is a rare and recently described condition. Most anatomic pathology laboratories do not routinely test for it. Spontaneous pseudotumor remission is possible, even when associated with the renal phenotype, although this has not been described until now.

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. Women with diagnosis of primary HOA were included.

Primary outcome: hand functional status, assessed by Cochin questionnaire.

Secondary outcomes: hand pain, assessed by visual analogue scale and tender hand joint count (THJ). Explanatory variables: obesity, diabetes mellitus, arterial hypertension and hypercholesterolemia. Possible associations between obesity and the other components of metabolic syndrome with Cochin score, hand pain and THJ were tested in a multivariable linear regression model. Potential confounders considered: age, education level and countrywide distribution.

Results: 473 women with primary HOA were included. Forty percent were overweight and 29% obese. Ninety-three (19.8%) participants had diabetes, 261 (55.8%) reported hypertension and 261 (55.9%) hypercholesterolemia. Mean Cochin score was 15.5±14.8, mean pain VAS was 4.7±2.6 and mean THJ 1.4±3. In the multivariable analysis, obesity (β 4.6 CI 0.7;8.5) and diabetes (β 4.0 CI 0.4;7.6) were found to significantly associate with HOA functional disability. In addition, diabetes, but not obesity, associated with hand pain. There was no association between obesity or diabetes with THJ.

Conclusion: In a Portuguese female population with primary HOA, obesity and diabetes mellitus independently associated with a worse hand functional status. These data add to evidence suggesting a role of metabolic factors in the severity of HOA.

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.

Methods: A single centre retrospective cohort study was conducted comprising 150 patients with axSpA that attended the Rheumatology Outpatient Clinic from January to December 2019. Logistic Multivariate Regression was performed to understand which factors independently contributed to the use of bDMARDs.

Results: Fifty-two patients (34,7%) were under biological treatment. In comparison to the group that was not under treatment with bDMARDs, these were significantly more likely to be hard-workers (57,8% vs 29,7%; p = ,003), to have had elevated C-Reactive Protein at the time of diagnosis (81,6% vs 48,9%; p < ,001), to have had a grade of sacroiliitis at diagnosis greater than 2 (67,4% vs 29,5%; p < ,001) and to have history of enthesitis, (32,7% vs 13,3%; p = ,006). In multivariate regression analysis, only the hard-worker type (OR = 3.09, CI: 1.14 - 8.37; p = .027) and the highest grade of sacroiliitis (OR = 4.41, CI: 1.69 - 11.50; p = .002) were found to be independently associated with the use of bDMARDs.

Conclusion: In this study, the performance of work associated with greater biomechanical stress and the presence of greater structural damage at diagnosis were shown to be associated with the use of bDMARDs. The authors highlight the importance of recognizing these factors that seem to relate to more aggressive disease, with higher use of bDMARDs, thus suggesting a need for a tighter control management strategy in these patients.