Patrícia Nero

Centro Hospitalar de Lisboa Ocidental, Lisboa, Lisbon, Portugal

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Publications (28)118.53 Total impact

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    ABSTRACT: Introduction: Low-dose weekly methotrexate (MTX) is the mainstay in the therapy of rheumatoid arthritis (RA). It can be given via oral, intramuscular or subcutaneous (SC) route. This study sought to determine the real-world pattern of treatment with SC MTX in Portuguese adult patients with active RA. Methods: Utilization of Metoject(®) in Rheumatoid Arthritis (UMAR) was a non-interventional, cohort multicenter study with retrospective data collection. Eligible patients had active RA, at least 18 years of age, and started SC MTX treatment in 2009 or 2010 after failure or intolerance to oral MTX. Data were collected from patient's clinical records. Both non-parametric and parametric survival methods were used to obtain a detailed understanding of SC MTX treatment duration. Result: Fifty patients were included, of which only 9 discontinued SC MTX during the study follow-up period. The probability of discontinuation after 1, 2, and 3 years of treatment of SC MTX treatment is expected to be 6.10%, 8.50%, and 23.20%, respectively. The extrapolated median duration of SC MTX using an exponential model was 106.4 months/8.87 years. Mean dose of SC MTX was 18.36 mg. The reasons for treatment discontinuation were occurrence of adverse events in six patients and lack of efficacy in three. Conclusion: The long treatment duration of SC MTX highlights its excellent tolerability compared to oral MTX, especially concerning the frequent adverse gastrointestinal events of MTX. Furthermore, long MTX treatment duration provides the opportunity to postpone or even avoid expensive therapies with biologics. The results obtained from the UMAR study provide important information for the utilization and public financing of SC MTX in Portugal.
    Full-text · Article · Jan 2016 · Advances in Therapy
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    ABSTRACT: Background Systemic lupus erythematosus (SLE) is a multi-organ immune-mediated disease that affects predominantly women at reproductive age but may present itself at any age. Age at disease onset has a strong modulating effect on clinical presentation and course of disease. Although young patients may have a more aggressive disease, controversies persist regarding the impact of age at disease onset on SLE outcome. Objectives Characterize childhood-onset, adult-onset and late-onset SLE and assess whether disease outcome differs in these three patient groups. Methods Patients with childhood-onset (diagnosis ≤18 years) SLE fulfilling ACR 1997 criteria were identified in the Portuguese registry Reuma.pt/SLE and compared with adult-onset (≥19y and ≤49 years) and late-onset (≥50 years) SLE patients paired for disease duration. Results Two hundred and sixty seven SLE patients with mean disease duration of 11.9±9.3 years were analyzed (Table 1). The number of fulfilled ACR criteria was significantly higher in childhood-onset SLE. A greater proportion of women, higher prevalence of arthritis and anti-SSA antibodies were noted in the adult-onset group. Hypertension, diabetes and thyroid disease were significantly more prevalent in late-onset SLE. Disease activity at last visit evaluated using the SLEDAI-2K was significantly higher in childhood-onset group than in the late-onset counterparts. SLICC/ACR damage index was numerically higher in late-onset SLE and significantly more patients in this group had irreversible damage. Cyclophosphamide and mycophenolate mophetil were used more frequently in childhood-onset SLE patients. Conclusions The skin, kidney and neurological involvement are most common in childhood-onset, as well as the use of immunosuppressants, supporting the concept of a more severe disease. In contrast, patients with late-onset SLE have more comorbidities and irreversible damage. The age of SLE onset has a significant impact not only on the clinical characteristics and disease activity, but is also important for disease outcome. Disclosure of Interest None declared
    Full-text · Article · Jun 2015 · Annals of the Rheumatic Diseases
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    ABSTRACT: Objectives: To compare the effectiveness of TNF inhibitors (TNFi) and tocilizumab in rheumatoid arthritis (RA) treatment, according to different response criteria. Methods: We included RA patients registered in the Rheumatic Diseases Portuguese Register treated with TNFi or tocilizumab for at least 6 months, between January 2008 and July 2013. We assessed remission/low disease activity (LDA) at 6 months according to DAS28, CDAI, and SDAI, as well as Boolean ACR/EULAR remission and EULAR response rate, adjusting for measured confounders. Results: Tocilizumab-treated patients (n = 95) presented higher baseline disease activity and were less frequently naïve to biologics compared to TNFi users (n = 429). Multivariate logistic regression analysis including the propensity score for receiving tocilizumab showed that patients treated with tocilizumab were more likely to achieve remission or LDA according to DAS28 (OR = 11.0/6.2, 95% CI 5.6-21.6/3.2-12.0), CDAI (OR = 2.8/2.6, 95% CI 1.2-6.5/1.3-5.5), or SDAI (OR = 3.6/2.5, 95% CI 1.5-8.7/1.1-5.5), as well as a good EULAR response (OR = 6.4, 95% CI 3.4-12.0). However, both groups did not differ in Boolean remission (OR = 1.9, 95% CI 0.8-4.8) or good/moderate EULAR response (OR = 1.8, 95% CI 0.8-4.5). Conclusions: Compared with TNFi, tocilizumab was associated with greater likelihood of achieving DAS28, CDAI, and SDAI remission/LDA and EULAR good response. Boolean remission and EULAR good/moderate response did not differ significantly between groups.
    Full-text · Article · Apr 2015 · BioMed Research International
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    ABSTRACT: Objective: The new Systemic Lupus International Collaborating Clinics (SLICC) 2012 classification criteria aimed to improve the performance of systemic lupus erythematosus (SLE) classification over the American College of Rheumatology (ACR) 1997 criteria. However, the SLICC 2012 criteria need further external validation. Our objective was to compare the sensitivity for SLE classification between the ACR 1997 and the SLICC 2012 criteria sets in a real-life, multicenter, international SLE population. Methods: We conducted a cross-sectional observational study of patients with a clinical diagnosis of SLE followed at the participating rheumatology centers and registered in the Portuguese and Spanish national registries. The sensitivity of the 2 classification sets was compared using McNemar's test. The sensitivity of ACR 1997 and SLICC 2012 was further examined in 5 subgroups, defined according to disease duration. Results: We included 2,055 SLE patients (female 91.4%, white 93.5%, mean ± SD age at disease onset 33.1 ± 14.4 years, mean ± SD age at SLE diagnosis 35.3 ± 14.7 years, and mean ± SD age at the time of the study 47.4 ± 14.6 years) from 17 centers. The sensitivity for SLE classification was higher with the SLICC 2012 than with the ACR 1997 (93.2% versus 85.6%; P < 0.0001). Of 296 patients not fulfilling the ACR 1997, 62.8% could be classified with the SLICC 2012. The subgroup of patients with ≤5 years since disease onset presented the largest difference in sensitivity between the SLICC 2012 and the ACR 1997 (89.3% versus 76.0%; P < 0.0001); this difference diminished with longer disease duration, and it was no longer significant for patients with >20 years of disease duration. Conclusion: The SLICC 2012 criteria were more sensitive than the ACR 1997 criteria in real-life clinical practice in SLE. The SLICC 2012 criteria may allow patients to be classified as having SLE earlier in the disease course.
    No preview · Article · Dec 2014
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    ABSTRACT: Background Tocilizumab (TCZ), an interleukin-6 receptor blocker, and anti-tumor necrosis factor (TNF) biologic agents are key therapies in the management of rheumatoid arthritis (RA). They are considered to be equally effective and very few head-to-head comparisons have been published. Objectives To compare remission rates in RA patients treated with anti-TNF agents and TCZ and assess the impact of previous biologic therapies in treatment response. Methods We included RA patients registered in the Rheumatic Diseases Portuguese Register, Reuma.pt, who started anti-TNF or TCZ after January 1, 2008, were treated for at least 6 months and had available DAS28 scores at baseline and at 6 months. Our primary outcome was the proportion of patients who achieved remission at 6 months by DAS28, CDAI, SDAI and Boolean remission criteria. Logistic regressions were performed to compare the groups and subgroup analyses of biologic-naïve patients were conducted. Results 524 RA patients were enrolled, (106 adalimumab, 202 etanercept, 43 golimumab, 78 infliximab, 95 TCZ). At baseline, the groups were similar except for proportion of biologic-naïve patients (lower in TCZ group, p<0.0001) and mean DAS28, CDAI and swollen joint count, all higher in the TCZ group (respectively: p=0.0005, p=0.037 and p<0.0001). At 6 months, more TCZ-treated patients were in DAS28 remission, with no differences for CDAI, SDAI or Boolean remission. Considering only naïve patients, DAS28, CDAI and SDAI remission were significantly higher in the TCZ group compared to anti-TNF, with similar rates of Boolean remission. This was confirmed in the multivariate logistic regression, adjusting for age, gender, number of previous biologics and baseline disease activity: DAS28 OR 10.8 (5.9-19.7), CDAI OR 2.9 (1.3-6.5), SDAI OR 4.1 (1.8-9.5), Boolean OR 1.9 (0.88-4.3). Conclusions TCZ treatment was associated with higher rate of DAS28 remission at 6 months and previous biologic therapy significantly affected CDAI/SDAI remission. Naïve patients treated with TCZ had better DAS28, CDAI and SDAI remission rates compared to those treated with TNF inhibitors, whereas the more stringent Boolean remission was similar among all groups. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.2668
    No preview · Article · Jun 2014 · Annals of the Rheumatic Diseases
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    ABSTRACT: Background Previous studies demonstrated functional and morphological microcirculatory abnormalities that may be relevant to the pathophysiology and clinical manifestations of fibromyalgia (FM). Objectives To ascertain and compare capillary morphology of FM patients with and without Raynaud's phenomenon (RP) using nailfold videocapillaroscopy (NVC). Methods Patients fulfilling the 2010 American College of Rheumatology Diagnostic Criteria for FM were allocated in 2 groups according to the presence or absence of RP (RP+ or RP-, respectively). All patients with secondary causes of RP other than FM were excluded. One blinded operator performed all the NVC using a 200x amplification digital microscope and analytical software. Capillary parameters evaluated in fingers II-V of both hands included: tortuosity, apex enlargement, branch enlargement, microhemorrhages, giant capillaries, capillary density, capillary branching and architectural derangements. Each parameter was rated 0-3 (0= no changes; 1= <33% abnormalities; 2=33-66% abnormalities; 3= >66% abnormalities) and the mean scores were calculated. The association of RP with the capillary parameters was assessed using multivariate linear regression adjusted for age, FM duration and occupation. Results Twenty FM patients were enrolled, 10 in each group, however 2 patients RP+ were excluded due to thyroid disease. Included patients were all female, had a mean age of 50.4 years and a mean duration of disease of 12.8 years. In both RP+ and RP- groups, the most frequently found abnormalities were minor dysmorphies, namely tortuosities, apex enlargement and branch enlargement. However, scoring of these parameters showed no significant difference between the 2 groups. More than half of RP+ and RP- patients presented slight focal decreases in capillary density, once again with no difference between groups. The only giant capillaries were found in a RP+ patient with an “early scleroderma pattern” as proposed by Cutolo et al. Hemorrhages were scarce and had a traumatic appearance, except in the previously mentioned patient. Interestingly, RP associated negatively with the capillary branching score. Table 1 summarizes the results. Conclusions No association was established between RP and microcirculatory abnormalities in FM patients. However, capillary tortuosity and enlargement were present in almost all subjects, supporting previous findings that FM may associate with minor NVC dysmorphies. Some authors suggest that tissue hypoxia arising from repeated autonomic-mediated vasoconstriction might explain these changes, as well as the apparent reduction in capillary density. Studies with a larger sample of FM patients are needed to confirm these results. Disclosure of Interest : None declared DOI 10.1136/annrheumdis-2014-eular.2157
    No preview · Article · Jun 2014 · Annals of the Rheumatic Diseases
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    ABSTRACT: Background Approximately one-third of patients with ankylosing spondylitis (AS) treated with tumor necrosis factor inhibitors (TNFi) need to switch their biological therapy. However, a significant clinical response only occurs in half of switcher patients. Objectives To determine predictive factors of treatment response at 12 weeks in AS patients switching treatment to a second TNFi. Methods Patients with AS (1984 modified New York classification criteria) switching treatment to a second TNFi were identified in the Rheumatic Diseases Portuguese Register, Reuma.pt. Data was extracted at baseline (at second TNFi start) and at 12 weeks of follow-up. Variables included in the analysis were age, race, gender, BMI, education level, work status, tobacco and alcohol consumption, disease duration, time to diagnosis, peripheral arthritis, extra-articular manifestations, ESR, CRP, HLA-B27 positivity, previous DMARD and steroid therapy, BASDAI, ASDAS, BASFI, BASMI, first TNFi used, reason and time to switch. Univariable logistic regression analysis of baseline predictors of BASDAI response (improvement ≥2 units or ≥50%) was performed and variables with a p-value<0.25 were re-tested in multivariable regression models. Results Of the 334 AS patients treated with biologicals, 85 (25.4%) switched to a second TNFi. Reasons for switch included treatment failure (16; 18.8%), adverse events (25; 29.4%) and other reasons (44; 51.8%) like refractory extra-articular manifestations, surgery or patient preferences. Patients were included in the analysis if they stayed in therapy at least for 3 months and have BASDAI at baseline and at 3 months available. Forty-nine patients were analyzed, 22 responders (44.9%) and 27 non-responders (55.1%). BASDAI clinical response at 12 weeks was predicted by BASMI (p=0.04; OR 3.8 [95% CI 1.0, 14.1]) adjusting for gender, erythrocyte sedimentation rate and age at biological treatment onset. No other statistical significant demographical, clinical or laboratorial predictors were found. Conclusions We found 44.9% switchers who responded to the second TNFi. An increased BASMI score was an independent predictor of BASDAI clinical response to a second TNFi in AS patients. The inclusion of a greater number of patients in future studies may allow the determination of further predictive factors. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.5590
    No preview · Article · Jun 2014 · Annals of the Rheumatic Diseases
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    ABSTRACT: Background Systemic lupus erythematosus (SLE) affects predominantly women of reproductive age. However, in about 15% of patients SLE begins before the age of 18 years (early-onset) and in 10-20% of patients SLE is first diagnosed after the age of 50 years (late-onset). The age at disease onset significantly impacts on clinical presentation, disease course, response to treatment and prognosis. Objectives To compare demographic, clinical features and disease outcome between patients with early-onset and those with late-onset SLE and to determine whether patients' age affects the time interval until diagnosis. Methods All SLE patients from the Portuguese registry Reuma.pt/LES with disease onset at age≤18 years-old or at age≥50 years-old were included. Patients' data were cross-sectionally analyzed upon records from the last visit. The differences between groups with early and late onset were determined by Student t-tests, chi-square or Fisher's exact tests. Results 313 SLE patients were included (157 early-onset; 156 late-onset). In the early-onset group, 88% were women, mean disease duration 16.8±10.5y and mean age of diagnosis 17±5.9y. Higher education level was noted in the younger group. 81.6% of patients with late-onset SLE were women, mean disease duration 9.37±5.2y and mean age of diagnosis 60.5±7.5y. Photosensitivity, arthritis and neurological disorder were statistically more prevalent in the late-onset group. Anti-Sm positivity was observed more frequently in early-onset SLE. Co-morbidities were also more common in this age group. Disease activity evaluated using the SLEDAI-2K was higher in the early-onset (3.0±3.3 vs 2.0±2.8; p=0.01) while accumulated damage was higher in the older age group (1.0±1.3 vs 0.69±1.4; p<0.001). Diagnosis delay was significantly greater in patients with early-onset than in the late-onset group (3.1±5 vs 1.7±3 y; p=0.001). Conclusions Patients with late-onset SLE have more co-morbid conditions and greater accumulated damage despite shorter disease duration and lower disease activity. Age of onset has a significant impact not only on the clinical characteristics and disease outcome, but also on time until diagnosis. Disclosure of Interest : None declared DOI 10.1136/annrheumdis-2014-eular.5306
    No preview · Article · Jun 2014 · Annals of the Rheumatic Diseases
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    ABSTRACT: Background Many patients with a clinical diagnosis of Systemic lupus erythematosus (SLE) do not fulfill the ACR 1997 classification criteria (ACR'97). Consequently, patients included in SLE studies might not be representative of the real spectrum of the disease. The new SLICC 2012 classification criteria (SLICC'12) were proposed to improve the sensitivity of SLE classification. However, these criteria require external validation. Objectives To compare the sensitivity for SLE diagnosis of the ACR'97 and SLICC'12 classification criteria sets in a real life, multicenter, international SLE population. Methods We included in this cross-sectional observational study all patients with a clinical diagnosis of SLE followed at the participating Rheumatology centers and registered in the Portuguese and Spanish national registries (Reuma.pt and RELESSER, respectively). For each patient, we evaluated the fulfillment of the ACR'97 and SLICC'12 criteria. The sensitivity for SLE diagnosis of each classification set was calculated. We compared the proportion of cases fulfilling ACR'97 and SLICC'12 using the McNemar's test. To compare dichotomous variables in two independent populations, we applied a Chi-square or Fisher's exact test, as appropriate. The statistical significance level was p<0.05. Results We included 2055 SLE patients (female = 91.4%; Caucasian =93.5%; age at disease onset = 33.1±14.4; age at SLE diagnosis = 35.3±14.7; age at SLE criteria scoring = 47.4±14.6) from 17 centers. The sensitivity for SLE diagnosis was higher with the SLICC'12 (93.1%) compared with the ACR'97 (85.6%) (p<0.0001). At least one of the classification criteria sets was fulfilled by 94.7% of cases. From 295 patients not fulfilling the ACR'97, 63% could be classified as SLE with SLICC'12. In the group of patients with positive SLE classification using the ACR'97 criteria, 1.8% did not fulfill the SLICC'12. The sensitivity for SLE diagnosis increased with longer disease duration, for both the ACR'97 and SLICC'12 (p<0.0001). In the group of patients with <5 years since disease onset, there was a larger improved sensitivity of SLICC'12 (89.3%) compared to ACR'97 (76.0%) (p<0.0001); this difference in sensitivity reduced with longer disease duration, and loses significance for the group with >20 years of disease. Conclusions In real-life clinical setting, the SLICC'12 classification criteria present a higher sensitivity for SLE compared to ACR'97. The SLICC'12 might allow a SLE classification earlier in the disease course. Disclosure of Interest : None declared DOI 10.1136/annrheumdis-2014-eular.2506
    No preview · Article · Jun 2014 · Annals of the Rheumatic Diseases
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    ABSTRACT: Background Tocilizumab (TCZ) and anti-tumor necrosis factor (TNF) biologic agents are key therapies in the management of rheumatoid arthritis (RA). They are considered to be equally effective and very few head-to-head comparisons have been published. Objectives To compare response to therapy in RA patients treated with anti-TNF agents and TCZ according to different response measures and determine the factors influencing it. Methods We included RA patients registered in the Rheumatic Diseases Portuguese Register, Reuma.pt, who started anti-TNF or TCZ after January 1, 2008, were treated for at least 6 months and had available DAS28 scores at baseline and at 6 months. Our primary outcome was the change in DAS28, CDAI and SDAI at 6 months. We performed linear regressions to compare the groups and determined the best model predicting change in disease activity for each index. Results 524 RA patients were enrolled, (106 adalimumab, 202 etanercept, 43 golimumab, 78 infliximab, 95 TCZ). At baseline, TCZ users were less frequently naïve to biologic therapies (54.7% vs. 85%, p<0.0001), had more swollen and tender joint counts (p<0.0001 and p=0.02, respectively) and higher disease activity according to all indexes: DAS28 6.1±1.1 vs. 5.4±1.3 (n=524, p<0.0001), CDAI 33.3±13.2 vs. 28.1±13.6 (n=376, p=0.005), SDAI.35.6±13.1 vs. 29.1±30.4 (n=361, p=0.004). At 6 months, change in DAS28, CDAI, SDAI and joint counts was significantly higher in the TCZ group (Table 1). Multivariate linear regression models best predicting change in disease activity included biologic class, number of previous biologics, baseline activity, gender and ACPA/RF status (Table 2). Compared to anti-TNF, TCZ was associated with a larger difference in ΔDAS28, ΔCDAI and ΔSDAI of, respectively, 1.45, 4.25 and 5.41. Conclusions TCZ treatment was associated with greater change in DAS28, CDAI, SDAI and joint counts at 6 months. Biologic class, number of previous biologics, baseline activity, gender and ACPA/RF status predicted change in disease activity. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.3414
    No preview · Article · Jun 2014 · Annals of the Rheumatic Diseases
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    ABSTRACT: Rheumatoid arthritis' synovitis affects mostly small hand and feet joints, although it may compromise any joint with a synovial lining. Cervical involvement occurs usually in longstanding disease in over half of these patients. We report the case of a 35-year old male patient who was referred to our outpatient clinic for a 2-year severe and disabling inflammatory neck pain, with incomplete response to intramuscular non-steroidal anti-inflammatory drugs and unremarkable cervical imaging studies. He also mentioned self-limited episodes of symmetric polyarthralgia involving hands, wrists, elbows, knees and feet, which started after his cervical complaints. On laboratorial workup, positive rheumatoid factor and anti-citrullinated peptide antibody and negative HLA-B27 were found. Cervical spine magnetic resonance imaging revealed atlantoaxial subluxation and odontoid process inflammatory pannus and erosions. Rheumatoid arthritis with cervical spine involvement as initial manifestation of disease was the definite diagnosis. The patient was started on methotrexate and prednisone and he was referred to neurosurgery outpatient clinic for cervical spine fixation.
    No preview · Article · Oct 2013 · Acta reumatologica portuguesa
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    ABSTRACT: B-cells play an important role not only in cellular but also in humoral immunity through differentiation into plasma cells and antibody production. B-cell depletion may, theoretically, change the course of systemic rheumatic diseases (SRD) in which self-reactive antibodies are part of the pathogenic pathway. In Rheumatology, anti-B-cell antibody Rituximab is currently licensed for the treatment of rheumatoid arthritis, however there is growing evidence of its potential use in other SRD. The authors present a case series of eight patients in which Rituximab was used off-label including overlap syndrome Rhupus, systemic lupus erythematosus and Wegener's granulomatosis. In the end, a brief literature review about this subject is performed.
    No preview · Article · Oct 2013 · Acta reumatologica portuguesa
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    ABSTRACT: Spinal infections are rare but potentially life-threate - ning disorders. A high level of clinical suspicion is neces sary for rapid diagnosis and treatment initiation. The treatment combines both antibiotics and surgical intervention in the vast majority of cases. The authors report the case of a 84-year old female patient with a three week history of persistent lumbar back pain radiating to both thighs following a lower respiratory tract infection. She had lumbar spine tenderness but no neurological compromise. Her inflammatory markers were elevated and lumbar spine magnetic resonance imaging revealed L4-L5 spondylodiscitis with spinal epidural abscess. Blood cultures isolated Klebsiella pneumoniae and, since she was neurologically stable, conservative treatment with two-week intravenous gentamicin and eight-week intravenous ceftriaxone was initiated with positive inpatient and outpatient evolution.
    No preview · Article · Jul 2012 · Acta reumatologica portuguesa
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    ABSTRACT: Objectives: To develop Portuguese evidence-based recommendations for pain management by pharmocotherapy in inflammatory arthritis. Methods: The Portuguese project was integrated in the multinational 3E Initiative (Evidence, Expertise, Exchange) 2010 where a total of 453 rheumatologists from 17 countries have participated. The clinical questions concerning pain were formulated and the Portuguese group added 2 more questions. A systematic literature search was performed in Medline, Embase, Cochrane Library and 2008-2009 EULAR and ACR abstracts. The selected articles were systematically reviewed and the evidence was defined according to the Oxford Levels of Evidence. In each country a group of experts joined to discuss their national recommendations. In Portugal, the national meeting was held in October 2010, where 33 rheumatologists discussed and voted by Delphi method the national recommendations. Finally, the agreement among the rheumatologists and the potential impact on their clinical practice was assessed. Results: Thirteen national recommendations were formulated: pain measure scores; analgesic combination therapy; pharmacotherapy in preconception, pregnancy and lactation periods; pharmacotherapy according to comorbilities; safety of NSAIDs and/or paracetamol with methotrexate combination therapy; efficacy and safety of continuous/on-demand NSAIDs; opioids, paracetamol, corticosteroids, antidepressants, neuromodulators and muscle relaxants role and effectiveness; risk factors for the development of chronic pain and the role of topic analgesics. Conclusion: The portuguese recommendations for the pain management by pharmacotherapy in inflammatory arthritis were formulated according to the best evidence and supported by a panel of 63 rheumatologists. The differences between the national and international recommendations are reported in this article.
    Full-text · Article · Apr 2012 · Acta reumatologica portuguesa
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    ABSTRACT: Patients with systemic lupus erythematosus (SLE) have a longer life expectancy. The occurrence of irreversible damage has become a major concern. The present study assessed damage progression in patients with SLE over a 2-year period and identified baseline features associated with damage accrual. Two hundred and twenty-one patients that fulfilled criteria for SLE and had a follow-up longer than 6 months were enrolled. Demographic, clinical, and immunological data were collected at baseline. Accumulated organ damage was scored using the Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index (SDI). Patients were prospectively followed and SDI assessment repeated at 2 years. At baseline 72 patients (33%) presented some irreversible damage, and after 2 years 53 had accrued new damage. The mean SDI for the whole cohort increased from 0.582 to 0.980. Damage progression was higher in ocular, cardiovascular, and musculoskeletal systems. Older age [OR = 1.045; 95% confidence interval (CI) 1.021-1.069; P = 0.03], presence of antiphospholipid antibodies (OR = 3.047; 95% CI 1.169-7.941; P = 0.02), steroid use (OR = 6.401; 95% CI 1.601-25.210; P = 0.008), azathioprine use (OR = 3.501; CI 1.224-10.012; P = 0.01), and hypertension (OR = 3.825; 95% CI 1.490-9.820; P = 0.005) were predictors of damage progression in multivariate analysis. Overall SDI increased over time, with some systems being affected more frequently. Demographic and clinical characteristics, co-morbidity, and treatment options may contribute to irreversible damage. It is necessary to determine whether the control of modifiable factors (e.g., hypertension and judicious use of medications) might prevent damage progression in SLE patients.
    Full-text · Article · Sep 2009 · Annals of the New York Academy of Sciences
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    ABSTRACT: The purpose of this study was to characterize the clinical and serological features of a large cohort of patients with antinuclear antibody (ANA) positive undifferentiated connective tissue disease (UCTD). Consecutive patients with UCTD, followed up at the Rheumatology Clinic of the participating centers, were included. Data from these patients were obtained by clinical evaluation and chart review. All patients were diagnosed as having UCTD on basis of the following criteria: positive ANA plus at least one clinical feature of connective tissue disease, but not fulfilling classification criteria for any differentiated connective tissue disease. One hundred eighty-four patients were studied (female patients-94.5%; mean age at time of evaluation-47 years). The most prevalent manifestations were arthralgia (66%), arthritis (32%), Raynaud's phenomenon (30%), sicca symptoms (30%), and leukopenia (19%). The prevalence of ANA was 100%, anti-SSA 20%, anti-dsDNA 14%, and anti-SSB 7%. Patients with anti-dsDNA/anti-Sm, anticentromere/anti-Scl70, or anti-SSA/anti-SSB antibodies more frequently presented a set of manifestations close to systemic lupus erythematosus (SLE), systemic sclerosis, or Sjögren syndrome, respectively. We analyze a large cohort of UCTD. Seventy-two percent of these UCTD patients present lupus-, scleroderma-, or Sjögren-like features but do not fulfill classification criteria and mostly present a mild disease.
    Full-text · Article · May 2009 · Clinical Rheumatology
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    ABSTRACT: Juvenile dermatomyositis (JDM) is a rare systemic disease of unknown etiology characterized by inflammation of the muscle, skin and digestive tract, with variable outcome. The diagnostic criteria include proximal symmetrical muscular weakness, characteristic skin rashes, elevation of skeletal muscle enzymes and specific electromyographic and muscle biopsy abnormalities. Pulmonary and gastro-intestinal involvements, calcinosis and generalized edema usually indicate severe disease. Recent data suggest an association between the genotype -308 AA of the Tumour Necrosis Factor (TNF) gene and disease chronicity. We present a case of a 14 year-old female with JDM and generalized oedema which is a rare manifestation of the disease and it is associated to a poor outcome.
    No preview · Article · Apr 2009 · Acta reumatologica portuguesa
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    ABSTRACT: Association between ankylosing spondylitis (AS) and two genes, ERAP1 and IL23R, has recently been reported in North American and British populations. The population attributable risk fraction for ERAP1 in this study was 25%, and for IL23R, 9%. Confirmation of these findings to ERAP1 in other ethnic groups has not yet been demonstrated. We sought to test the association between single nucleotide polymorphisms (SNPs) in these genes and susceptibility to AS among a Portuguese population. We also investigated the role of these genes in clinical manifestations of AS, including age of symptom onset, the Bath Ankylosing Spondylitis Disease Activity, Metrology and Functional Indices, and the modified Stoke Ankylosing Spondylitis Spinal Score. The study was conducted on 358 AS cases and 285 ethnically matched Portuguese healthy controls. AS was defined according to the modified New York Criteria. Genotyping of IL23R and ERAP1 allelic variants was carried out with TaqMan allelic discrimination assays. Association analysis was performed using the Cochrane-Armitage and linear regression tests of genotypes as implemented in PLINK for dichotomous and quantitative variables respectively. A meta-analysis for Portuguese and previously published Spanish IL23R data was performed using the StatsDirect Statistical tools, by fixed and random effects models. A total of 14 nsSNPs markers (8 for IL23R, 5 for ERAP1, 1 for LN-PEP) were analysed. Three markers (2 for IL23R and 1 for ERAP1) showed significant single-locus disease associations, confirming that the association of these genes with AS in the Portuguese population. The strongest associated SNP in IL23R was rs1004819 (OR=1.4, p=0.0049), and in ERAP1 was rs30187 (OR=1.26, p=0.035). The population attributable risk fractions in the Portuguese population for these SNPs are 11% and 9.7% respectively. No association was seen with any SNP in LN-PEP, which flanks ERAP1 and was associated with AS in the British population. No association was seen with clinical manifestations of AS. These results show that IL23R and ERAP1 genes are also associated with susceptibility to AS in the Portuguese population, and that they contribute a significant proportion of the population risk for this disease.
    Full-text · Article · Jan 2009 · Clinical and experimental rheumatology
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    ABSTRACT: Eosinophilic fasciitis is a rare rheumatic condition characterized by inflammatory thickening of the skin and fascia, peripheral eosinophilia, elevated erythrocyte sedimentation rate and hypergammaglobulinemia. Internal organ involvement is uncommon. It is often difficult to diagnose eosinophilic fasciitis and its course may be variable. Glucocorticoids are most commonly used in the treatment but in many cases they are ineffective, requiring combined immunosuppressive treatment. Several cases of eosinophilic fasciitis and serious haematological disorders such as immune thrombocytopenia, Hodgkin's disease and aplastic anaemia have been described. The authors report an atypical severe case of eosinophilic fasciitis complicated by aplastic anaemia non responsive to treatment.
    No preview · Article · Jan 2009 · Acta reumatologica portuguesa
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    ABSTRACT: To verify if the response to TNFalpha inhibitors is influenced by the presence of IgM rheumatoid factor (RF), in patients with RA. In this study, the patients with the diagnosis of RA treated with TNFa inhibitors followed in our hospital were recruited. A protocol was applied including demographic, clinical and laboratory data, in order to calculate DAS 28. The presence/absence of IgM RF and associated therapies were record. Fifty-seven patients, 52 female, with a mean duration of anti-TNFa treatment of 30,9+/-15,9 months were studied. Twenty-four patients were being treated with infliximab, 17 with adalimumab and 16 with etanercept. Forty-one patients had IgM RF detectable in serum (RF positive group). In the RF positive group, the variation of DAS 28 was -1,75 +/- 1,53 vs -1,04 +/- 1,76 in the RF negative group (p=0,135). The mean duration of anti-TNFalpha treatment was similar in both groups (31,9+/-15,9 vs 29,5+/-16,16 months). Patients who were treated with methotrexate presented a higher variation of DAS 28 (-1,87 +/- 1,70 vs -0,80 +/- 1,09; p=0,041) and this variation was dose dependent (p=0,056). Despite needing a replication in a larger cohort, our results suggest that the presence of IgM RF in the serum did not interfere with the response to treatment with TNFalpha inhibitors.
    No preview · Article · Oct 2008 · Acta reumatologica portuguesa

Publication Stats

236 Citations
118.53 Total Impact Points

Institutions

  • 2009-2015
    • Centro Hospitalar de Lisboa Ocidental
      Lisboa, Lisbon, Portugal
  • 2005-2014
    • Hospital Egas Moniz
      • Department of Rheumatology
      Lisboa, Lisbon, Portugal
  • 2013
    • University of Lisbon
      Lisboa, Lisbon, Portugal
  • 2009-2013
    • New University of Lisbon
      • Faculty of Medical Sciences
      Lisboa, Lisbon, Portugal
  • 2006
    • Santa Maria Goretti Hospital
      Littoria, Latium, Italy