Risk of chronic kidney disease in 260 patients with lupus nephritis-analysis of a nationwide multicentre cohort with up to 35 years of follow-up.

OBJECTIVES: To compare proliferative (PLN) and membranous (MLN) lupus nephritis (LN) regarding clinical and laboratory presentation and long-term outcomes; To investigate predictors of progression to chronic kidney disease (CKD). METHODS: Multicentre observational study, with retrospective analysis of a prospective cohort, using data from the Rheumatic Diseases Portuguese Registry-Reuma.pt. Patients with biopsy-proven PLN, MLN and mixed LN were included. Cox regression survival analysis was used to investigate predictors of CKD. RESULTS: 260 patients were included. Median follow-up was 8 years (IQR 11; minimum 1, maximum 35 years). MLN patients presented with significantly lower serum creatinine (0.70 (IQR 0.20; minimum 0.50, maximum 1.30) mg/dl vs 0.80 (IQR 0.31; minimum 0.26, maximum 2.60) in PLN, p= 0.003). Proteinuria levels did not differ between groups (p= 0.641). Levels of complement were reduced in PLN but nearly normal in MLN patients, and there were fewer patients with positive anti-dsDNA antibodies in the MLN group (p< 0.001). One year after the beginning of treatment, 62% of the patients achieved EULAR/ERA-EDTA complete response, with further 5% achieving partial response. Patients with lower proteinuria at diagnosis were more likely to achieve a complete renal response at one year, however, proteinuria at diagnosis or at one year did not predict long term CKD. Estimated glomerular filtration rate (eGFR) ≤75 mL/min/1.73 m2 at one year was the strongest predictor of progression to CKD (HR 23 [95% CI 8-62], p< 0.001). Other possible predictors included the use of azathioprine for induction of remission, older age at diagnosis and male sex. CONCLUSION: Proteinuria levels did not predict LN histologic class in our cohort. eGFR cutoff of 75 mL/min/1.73 m2 after one year of treatment was strongly predictive of progression to CKD.

Anti-SSA Ro52 and anti-Ro60 autoantibodies: association with clinical phenotypes.

OBJECTIVES: Anti-SSA autoantibodies can be differentiated according to their antigenic target proteins as anti-Ro60 (60 kDa) or anti-Ro52 (52 kDa). Anti-SSA(Ro60) antibodies are clearly associated with connective tissue diseases (CTD), but the clinical significance of anti-SSA(Ro52) antibodies remains unclear. The aim of the present study was to analyse the disease phenotype of patients with anti-Ro52 and/or anti-Ro60 antibodies. METHODS: A multicentre, cross-sectional study was carried out of positive anti-Ro52 and/or Ro60 antibodies patients followed at 10 Rheumatology centres from January 2018 until December 2021. Patients were categorised into 3 groups: group 1 (Ro52+/Ro60-); group 2 (Ro52-/Ro60+); group 3 (Ro52+/Ro60+). Antinuclear antibodies were evaluated by indirect immunofluorescence assay and further screened for anti-extractable nuclear antigen (ENA) antibodies. Demographicsand clinical data were compared between the 3 groups, by patients' medical chart review. Univariate analysis was performed and subsequently logistic regression was used to identify intergroup differences and calculate the odds ratio with a 95% confidence interval (95% CI). RESULTS: We included 776 patients [female: 83.1%; median age: 59 (46-71) years]. Groups 1, 2, and 3 comprised 31.1%, 32.6%, and 36.3% of the patients, respectively. Anti-Ro52 antibody alone was more frequently associated with non-rheumatic diseases, older age, and men (p<0.05). Among patients with CTD, the diagnosis of systemic lupus erythematosus is 3 and 2 times more prevalent in groups 2 and 3, respectively, than in group 1 [OR 2.8 (95% CI 1.60, 4.97), p<0.001; OR 2.2 (95% CI 1.28, 3.86), p<0.01]. In group 2, the diagnosis of undifferentiated CTD is more frequent than in the other groups. Group 1 was more frequently associated with inflammatory myositis than group 2 [OR 0.09 (95% CI 0.01, 0.33), p<0.001] or group 3 [OR 0.08 (95% CI 0.01, 0.29), p<0.001]. Group 1 was also more frequently associated with arthritis (p<0.01), interstitial lung disease (p<0.01), and myositis (p<0.01). CONCLUSIONS: Anti-Ro52+ antibody alone is frequently found in patients with non-rheumatic diseases. In addition, anti-Ro52+ antibody is also prevalent in patients with CTD and associated with clinical phenotypes that are different from anti-Ro60+ antibody.

A one-point increase in the Damage Index for Antiphospholipid Syndrome (DIAPS) predicts mortality in thrombotic antiphospholipid syndrome.

OBJECTIVES: To determine whether early damage and its kinetics measured by the Damage Index for Antiphospholipid Syndrome (DIAPS) predicts mortality. METHODS: We carried out a single-centre retrospective analysis of thrombotic APS patients (2006 Sydney criteria), using the DIAPS for damage assessment. Early damage was considered to be at six months after disease onset; early damage increase (delta-DIAPS) was deemed to be at least a one-point rise in DIAPS within the first five years of illness. Groups were compared using appropriate statistical tests. Survival was analysed by the Kaplan-Meier method. Cox regression analysis was performed to investigate predictors of mortality. RESULTS: A total of 197 patients (71.1% female; 65.9% primary APS; 72.4% Caucasian) were followed for up to 43 years (median 10). Damage developed in 143 (73.6%) patients. Twenty-three patients (12%) died. Secondary APS (HR 3.07, 95%CI 1.32-7.12, p=0.009), male sex (HR 3.14, 95%CI 1.35-7.33, p=0.008) and age at APS onset ≥40 years (HR 5.34, 95%CI 1.96-14.53, p=0.001) were risk factors for death. Early damage (n=69, 35.0%) was not associated with death (p=0.231). Having a first arterial event was associated with early damage (p<0.001), but not with delta-DIAPS (p=0.539) nor with the risk of death (p=0.151). Delta-DIAPS (n=53/181, 29.3%) predicted mortality (HR 5.40, 95%CI 2.33-12.52, p<0.001), even after adjusting individually for APS category (secondary), sex (male), early damage and age at APS onset (≥40 years) (all p<0.005). CONCLUSIONS: Evolving damage in the first five years of illness, but not early damage, predicted mortality regardless of the nature of the first thrombotic event, sex, APS category and age.

Major determinants of prolonged remission in systemic lupus erythematosus: retrospective study over a 41+ year period.

OBJECTIVES: To investigate predictors of sustained complete remission (CR) for 3 and 5 years, minimum. METHODS: Retrospective observational study from January 1978 to December 2019, including systemic lupus erythmatosus (SLE) patients who attended the Lupus Clinic in a tertiary hospital, for at least 3 years. We used the BILAG score and serological profile to classify patients into CR, serologically active clinically quiescent (SACQ) and serological remission (SR). Multivariable Cox regression analysis was performed to investigate predictors of CR and Kaplan-Meier curves were obtained. RESULTS: We included 564 patients; 15% achieved CR, 7% SACQ and 15% SR. Some 63% attained no remission. In the CR group, 73% sustained the remission for 5 years or more. Patients who did not reach any kind of sustained remission died significantly earlier (P < 0.001). Cumulative survival figures at 5, 10, 20 and 30 years were 100, 100, 94 and 90%, respectively, for CR patients and 96, 93, 77 and 58%, respectively, for patients in the no-remission group. Significant predictors of CR were White ethnicity [adjusted hazard ratio (HR) 2.16 (95% CI 1.30-3.59); P = 0.003]; older age at diagnosis (>32 years) [HR 1.92 (1.24-2.97); P = 0.003]; absence of renal involvement [HR 2.55 (1.39-4.67); P = 0.002]; and of antiphospholipid syndrome (APS) [HR 4.92 (1.55-15.59); P = 0.007]. CONCLUSION: Patients not achieving any kind of sustained remission have a higher risk of early mortality. White ethnicity, older age at diagnosis, absence of renal involvement and of APS were significantly associated with CR. Predictors for sustained CR do not change whether a 3-year or 5-year period is applied.

Survival analysis of mortality and development of lupus nephritis in patients with systemic lupus erythematosus up to 40 years of follow-up.

OBJECTIVES: Patients with SLE have increased mortality compared with age- and sex-matched controls. LN is a severe manifestation of SLE and an important cause of death. We carried out a retrospective survival analysis to investigate factors that could influence the risk of mortality and LN in a large multi-ethnic cohort of patients with SLE. METHODS: By careful review of medical records, we identified 496 patients with SLE for whom we had complete information regarding the period of observation and occurrence of death and nephritis. Patients were stratified into groups according to sex, ethnicity, age at start of follow-up and time period of diagnosis. Kaplan-Meier analysis was used to investigate differences between the groups. RESULTS: Of the 496 patients in the study, 91 (18.3%) died, 165 (33.3%) developed LN and 33 (6.7%) developed end-stage renal failure. There was no difference between men and women in either mortality or development of LN. Caucasian patients were significantly less likely to develop LN than other ethnic groups (P < 0.0001) but not less likely to die. Patients diagnosed before the median age of 28 years were significantly more likely to develop LN (P < 0.0001) but significantly less likely to die (P = 0.0039) during the period of observation. There has been a significant improvement in survival in patients diagnosed between 1978 and 1989 and those diagnosed between 2006 and 2011 (P = 0.019). CONCLUSION: In our cohort, non-Caucasian ethnicity and younger age at diagnosis are associated with the risk of developing LN. There is evidence of improvement in survival of patients with SLE over time.

Extent of vascular plaque predicts future cardiovascular events in patients with systemic lupus erythematosus.

OBJECTIVE: Patients with SLE have increased prevalence of clinical cardiovascular disease (CVD) and subclinical atherosclerosis. Although 30-40% of patients with SLE have vascular plaque on ultrasound scanning, this study is the first to consider the relationship between total burden of plaque and subsequent CVD risk. METHODS: One hundred patients with SLE and without any previous clinical CVD underwent vascular ultrasound scans of both carotid and both common femoral bifurcations between 2011 and 2013. Clinical, serological, demographic and treatment data were collected at baseline. Patients were followed till 2020 to identify those who developed new onset coronary disease or stroke. Statistical analysis to identify factors associated with increased risk of developing CVD events was carried out. RESULTS: Thirty-six patients had plaque at baseline. During follow-up five patients (all had baseline plaque) developed coronary disease and two, without baseline plaque, developed lacunar strokes. Mean (s.d.) age of these patients was 46.5 (4.5) years. Patients with three or more baseline bifurcations with plaque were 10 times more likely to develop CVD than those with 0-2 bifurcations with plaques (OR 9.9, P = 0.009). TPA > 16mm2 was associated with six-fold increased risk of CVD (OR = 6.44, P = 0.028). Patients with disease duration > 14 years were more likely than those with disease duration < 14 years to develop CVD (OR 8.3 P = 0.043). CONCLUSIONS: The number of bifurcations with plaque and TPA in patients with SLE may be valuable in assessing risk of CVD and deciding on clinical measures to reduce this risk.

Serum Metabolomic Signatures Can Predict Subclinical Atherosclerosis in Patients With Systemic Lupus Erythematosus.

[Figure: see text].

Assessing outcomes in a lupus nephritis cohort over a 40-year period.

OBJECTIVES: To characterize a LN cohort over 40 years, assessing its evolution, analysing two major outcomes: the development of end-stage renal disease and mortality rates in the first 5 years after LN diagnosis. METHODS: An observational retrospective study of patients with LN, followed up from 1975 at University College Hospital. Patients were divided into four groups, depending on the decade of LN diagnosis: 1975-1985 (D1), 1986-1995 (D2), 1996-2005 (D3) and 2006-2015 (D4). Comparison between groups was performed with respect to demographic, clinical, serological and histological characteristics and outcome. RESULTS: Two hundred and nineteen patients with LN were studied. There was a change in ethnic distribution, with a decreasing proportion of Caucasians (58.6% in D1 to 31.3% in D4, P = 0.018) and increase in African-ancestry (17.2% in D1 to 39.6% in D4, P = 0.040). Serological and histological patterns changed throughout time, with a reduction in class IV nephritis (51.7% in D1 to 27.1% in D4, P = 0.035), and increase in class II nephritis (10% in D2 to 18.8% in D4, P = 0.01) and anti-extractable nuclear antigen antibody positivity (17.2% in D1 to 83.3% in D4, P = 0.0001). The 5-year mortality rates decreased from D1 (24.1%) to D2 (4%), stabilizing for the next 30 years. The 5-year progression to end-stage renal disease remained stable over the decades. CONCLUSION: Despite the changes in treatment of LN in the past 20 years, we have reached a plateau in 5-year mortality and progression to end-stage renal disease rates, suggesting that new therapeutic and management approaches, and strategies to enhance adherence, are needed to improve outcomes further in LN patients.

Total plaque area and plaque echogenicity are novel measures of subclinical atherosclerosis in patients with systemic lupus erythematosus.

OBJECTIVES: Patients with SLE have an increased risk of developing cardiovascular disease (CVD). Multiple studies have shown that these patients have increased numbers of carotid plaques and greater intima-media thickness (IMT) than healthy controls. Measures such as total plaque area (TPA) and plaque echogenicity may be more sensitive and more relevant to cardiovascular risk than presence of plaque and IMT alone. Our objective was to produce the first report of TPA and echogenicity in a population of patients with SLE. METHODS: One hundred patients with SLE and no history of clinical CVD were recruited. Clinical, serological and treatment variables were recorded and serum was tested for antibodies to apolipoprotein A-1 and high-density lipoprotein. Both carotid and both femoral artery bifurcations of each patient were scanned to determine IMT, TPA and echogenicity of plaques. Univariable and multivariable statistical analyses were carried out to define factors associated with each of these outcomes. RESULTS: Thirty-six patients had carotid and/or femoral plaque. Increasing age was associated with presence of plaque and increased IMT. Triglyceride levels were associated with presence of plaque. Mean (s.d.) TPA was 60.8 (41.6) mm2. Patients taking prednisolone had higher TPA. Most plaques were echolucent, but increased echogenicity was associated with prednisolone therapy and persistent disease activity. CONCLUSION: TPA and plaque echogenicity in patients with SLE are associated with different factors than those associated with presence of plaque and IMT. Longitudinal studies may show whether these outcome measures add value in the management of cardiovascular risk in SLE.

Fracture risk in systemic lupus erythematosus patients over 28 years.

OBJECTIVES: Chronic glucocorticoid use is complicated by osteoporosis and increases the risk of fragility fractures. EULAR guidelines on SLE management recommend reducing chronic glucocorticoid dosage to ≤7.5 mg/day to minimize this risk. We examined the relationship of glucocorticoid dose to fragility fracture risk in a cohort of SLE patients. METHODS: Retrospective analysis of SLE patients attending University College Hospital over 28 years was undertaken. Collected data included consecutive steroid dose, dual-energy X-ray absorptiometry scans and fragility fractures. RESULTS: We collected data on 250 patients with a median of 17 years' follow-up. Fragility fractures were diagnosed in 28 (11.2%) patients and the mean ± s.d. age of first fracture was 51 ± 16 years. A total of 94% received glucocorticoids, the average dose being 6.20 mg/day. Patients with fragility fractures had a lower average daily dose (5.36 vs 6.23 mg/day) but a higher median cumulative dose (25.19 vs 20.96 g). These differences were not significant (P = 0.127 and 0.229, respectively). Some 93% of patients received vitamin D, and 85% received calcium. Cox regression analysis showed older age at SLE diagnosis, osteoporosis and secondary hyperparathyroidism were associated with fragility fractures. Glucocorticoid dose was not significantly associated with the occurrence of fragility fractures. Twenty-two patients with fractures were treated with bisphosphonates, two with denosumab and two with teriparatide. CONCLUSIONS: We found no significant association between glucocorticoid treatment and fragility fractures in our group of patients; however, a prospective study including more patients not treated with CS would be necessary to confirm these results.

Outcomes of membranous and proliferative lupus nephritis - analysis of a single-centre cohort with more than 30 years of follow-up.

OBJECTIVES: To compare membranous lupus nephritis (MLN) and proliferative lupus nephritis (PLN) with respect to survival, demographic, clinical and laboratory characteristics; and to investigate predictors of renal and patient survival. METHODS: Single-centre retrospective observational study. Patients with biopsy-proven PLN, MLN and mixed lupus nephritis were included. Groups were compared using appropriate statistical tests and survival was analysed through the Kaplan-Meier method. Cox regression analysis was performed to investigate predictors of renal and patient survival. RESULTS: A total of 187 patients with biopsy-proven lupus nephritis (135 with PLN, 38 with MLN and 14 with mixed LN) were followed for up to 42 years (median 12 years). There was a higher proportion of MLN amongst Afro-Caribbeans than amongst Caucasians (31% vs 15%, P = 0.010). Patients with MLN had significantly lower anti-dsDNA antibodies (P = 0.001) and higher C3 levels (P = 0.018) at diagnosis. Cumulative renal survival rates at 5, 10, 15 and 20 years were 91, 81, 75 and 66% for PLN and 100, 97, 92 and 84% for MLN, respectively (P = 0.028). Cumulative patient survival at 5, 10, 15 and 20 years was 94, 86, 80 and 76%, with no difference between PLN and MLN. Urinary protein-creatinine ratio above 42 mg/mmol and eGFR below 76 ml/min/1.73 m2, one year after the diagnosis of LN, were the strongest predictors of progression to end-stage renal disease. eGFR below 77 ml/min/1.73 m2, at one year, development of end-stage renal disease and Afro-Caribbean ethnicity were associated with higher mortality. CONCLUSION: Patients with MLN and PLN differ significantly regarding serological profiles and renal survival, suggesting different pathogenesis. Renal function at year one predicts renal and patient survival.

Comparing pregnancy outcomes in patients with Systemic Lupus Erythematosus (SLE) and Undifferentiated Connective Tissue Disease (UCTD): a descriptive cohort study.

BACKGROUND: Females diagnosed with systemic lupus erythematosus (SLE) face an elevated risk of adverse pregnancy outcomes (APOs). However, the evidence regarding whether a similar association exists in patients with undifferentiated connective tissue disease (UCTD) is inconclusive. METHODS: We conducted a retrospective review (2006-2019) of pregnancy outcomes among patients with SLE (n = 51) and UCTD (n = 20) within our institution. We examined the occurrence of various APOs, encompassing miscarriage, stillbirth, termination, preterm birth, pre-eclampsia, eclampsia, HELLP syndrome, intrauterine growth restriction, abruption placentae, congenital heart block, or other cardiac abnormalities. RESULTS: The mean age at pregnancy was 35 ±â€¯7.0 years for patients with SLE and 35 ±â€¯6.8 years for those with UCTD (p = 0.349). The proportion of Caucasian women was 47% in SLE and 80% in UCTD. Pregnancies in both groups were planned (81% in SLE and 77% in UCTD), and patients presented with inactive disease at conception (96% in SLE and 89% in UCTD). Hydroxychloroquine at conception was utilized by 86% of women with SLE, in contrast to 36% in the UCTD group. Both, SLE and UCTD cohorts exhibited low rates of disease flares during pregnancy and/or puerperium (14% vs. 10%). The incidence of APOs was 15.6% in SLE patients compared to 5% in those with UCTD (Risk difference 19.5%; 95% confidence interval: -3.9 to 43.1; p = 0.4237). CONCLUSION: Our study underscores the importance of strategic pregnancy planning and the maintenance of appropriate treatment throughout pregnancy to ensure optimal disease management and minimize adverse outcomes in both SLE and UCTD pregnancies.

Contactin-1 links autoimmune neuropathy and membranous glomerulonephritis.

Membranous glomerulonephritis (MGN) is a common cause of nephrotic syndrome in adults, mediated by glomerular antibody deposition to an increasing number of newly recognised antigens. Previous case reports have suggested an association between patients with anti-contactin-1 (CNTN1)-mediated neuropathies and MGN. In an observational study we investigated the pathobiology and extent of this potential cause of MGN by examining the association of antibodies against CNTN1 with the clinical features of a cohort of 468 patients with suspected immune-mediated neuropathies, 295 with idiopathic MGN, and 256 controls. Neuronal and glomerular binding of patient IgG, serum CNTN1 antibody and protein levels, as well as immune-complex deposition were determined. We identified 15 patients with immune-mediated neuropathy and concurrent nephrotic syndrome (biopsy proven MGN in 12/12), and 4 patients with isolated MGN from an idiopathic MGN cohort, all seropositive for IgG4 CNTN1 antibodies. CNTN1-containing immune complexes were found in the renal glomeruli of patients with CNTN1 antibodies, but not in control kidneys. CNTN1 peptides were identified in glomeruli by mass spectroscopy. CNTN1 seropositive patients were largely resistant to first-line neuropathy treatments but achieved a good outcome with escalation therapies. Neurological and renal function improved in parallel with suppressed antibody titres. The reason for isolated MGN without clinical neuropathy is unclear. We show that CNTN1, found in peripheral nerves and kidney glomeruli, is a common target for autoantibody-mediated pathology and may account for between 1 and 2% of idiopathic MGN cases. Greater awareness of this cross-system syndrome should facilitate earlier diagnosis and more timely use of effective treatment.

Impact of pegloticase on patient outcomes in refractory gout: current perspectives.

Gout is currently the most frequent cause of inflammatory arthritis worldwide and is responsible for poor health-related quality of life and loss of work productivity. It is caused by high levels of serum urate, leading to the deposition of monosodium urate crystals in joints and soft tissues. This condition is associated with acute flares and, if untreated or refractory, chronic and potentially destructive arthritis and tophi formation. Pegloticase is a recombinant, pegylated uricase used in the treatment of gout patients who fail conventional urate-lowering therapy. In this review, we discuss the impact of pegloticase on patient outcomes in refractory gout. We analyze different parameters, such as plasma uric acid concentration, frequency of flares, tophi reduction, pain, function, quality of life, and safety.

Concerns of patients with systemic lupus erythematosus and adherence to therapy - a qualitative study.

PURPOSE: The objectives of this study were 1) to identify the impact of systemic lupus erythematosus (SLE) on patients' lives and their reactions to this, as well as their main concerns and expectations regarding their disease and treatments; and 2) to assess the relationship between these concerns and the adherence to treatments, medical visits, and diagnostic tests. PATIENTS AND METHODS: Qualitative study, using a convenient sample of SLE patients attending an outpatient rheumatology clinic. Semistructured interviews were conducted and audiotaped. The full transcripts were analyzed by two different coders using content analysis methodology. RESULTS: Fifteen participants were included. SLE had a major impact on these patients' lives. Their main concerns were fear of disease worsening and becoming dependent on other people, fear of not being able to take care of their children or provide for the family, and the possibility of transmitting SLE to their offspring. The main reasons for adherence to therapy were the wish to avoid manifestations of SLE and trust in the rheumatologist and routine. Nonadherence was more common in the beginning of the treatment because of the difficulty in accepting a chronic disease that requires lifelong therapy. CONCLUSION: Our data underlined the important interplay between adherence to medication and the possibility to gather accurate information and proper support during the treatment process. Good communication and efficient patient education strategies, focused on improving their knowledge about the disease and its treatments, may be important to improve adherence to therapy in SLE.

Shoulder giant schwannoma - a diagnosis to be considered in painless shoulder masses.

Tumours formed from peripheral nerve sheaths are rare in the shoulder. We present the case of a 80-year-old female referred to an orthopaedic surgery clinic because of a large painless mass of the right shoulder, with one year of evolution. There was no history of trauma, involvement of any other joint or systemic complaints. Given the clinical and imagiological suspicion of a giant synovial cyst, the patient underwent complete surgical resection of the mass, which took place without any complications or functional impairment. Histological diagnosis - schwannoma - was unexpected given the lesion characteristics and the low incidence of these benign tumours. They usually present as a slow growth mass, where pain and neurological deficits are uncommon. Among the complementary tests available, MRI is the gold standard. Still, the diagnosis is made preoperatively only in a minority of cases. We emphasize the need to consider this rare clinical entity in the differential diagnosis of slow evolution tumourations of the shoulder, in order to avoid unnecessary surgery and to minimize the risk of iatrogenic injury of the involved nerve.

[The Epidemiology of Dementia and Alzheimer Disease in Portugal: Estimations of Prevalence and Treatment-Costs]. / Epidemiologia da Demência e da Doença de Alzheimer em Portugal: Estimativas da Prevalência e dos Encargos Financeiros com a Medicação.

INTRODUCTION: The incidence and prevalence of global dementia and Alzheimer's disease (AD) increase with age, almost doubling every five years after the sixth decade of life. Demographic aging is a reality in Portugal, being expectable that the number of dementia cases also increases. Even so, dementia-epidemiological data in Portugal is scarce and cost-of-illness studies are almost inexistent. Our aims were to obtain up-to-date information about the prevalence of dementia/ Alzheimer's disease in Portugal, to estimate the number of cases effectively diagnosed as Alzheimer's disease and to determine illness-costs with specific dementia treatment. MATERIAL AND METHODS: The numbers of age-adjusted prevalence of dementia obtained for Occidental Europe (Alzheimer's Disease International study), where applied to the resident population in Portugal (2013). Estimations related to diagnosis and treatment-costs were based in data provided by the Intercontinental Marketing Services Health (IMSH)-2013. RESULTS: The estimated number of Portuguese people with dementia among those aged ≥60 years, is 160,287, representing 5.91% of this population-stratum. Knowing Alzheimer's disease is responsible for 50-70% of all cases, we might conclude there are between 80,144 and 112,201 patients. According to IMSH-data, 76250 receive anti-dementia drugs and the costs of this kind of medication is 37 M€¬/year. CONCLUSIONS: As a consequence of the demographic aging, also the number of dementia cases increases. Apparently, not all Alzheimer's disease patients receive the recommended medication, suggesting this condition is still under-diagnosed. However, figures indicate a positive progression with an increment of treated cases and a reduction of medication-costs.

Introdução: A incidência e prevalência de demência e de Doença de Alzheimer aumentam com a idade, duplicando a cada cinco anos após a sexta década de vida. Portugal é um país envelhecido, previsivelmente com um número crescente de casos de demência. No entanto, os dados epidemiológicos são escassos e os estudos sobre os custos da doença praticamente inexistentes. Propomo-nos apresentar uma estimativa actualizada da prevalência de demência/ Doença de Alzheimer em Portugal e inferir, a partir da prescrição específica para demência, o número de diagnósticos efectivos e os encargos financeiros com esses medicamentos.Material e Métodos: ÃÄ população residente em Portugal (2013), aplicámos os valores de prevalência de demência para a Europa Ocidental (estudo da AlzheimerâÄôs Disease International). A estimativa dos diagnósticos efectivos de Doença de Alzheimer e dos encargos financeiros com medicação específica baseou-se nas informações do Intercontinental Marketing Services Health (IMSH) âÄì 2013.Resultados: O número estimado de Portugueses com mais de 60 anos e com demência foi 160287, o que corresponde a 5,91% deste universo populacional. Sabendo que a Doença de Alzheimer representa 50-70% dos casos, inferimos que existirão entre 80144 e 112201 doentes. Por outro lado, os dados da IMSH indicam que estarão diagnosticados e a receceber anti-demenciais 76250 doentes, representando um encargo financeiro de 37 MâǬ/ano.Conclusão: O envelhecimento da população incrementa o número de casos de demência. Aparentemente, nem todos os doentes com Doença de Alzheimer recebem a medicação aconselhada, sugerindo que esta condição ainda está sub-diagnosticada. A evolução tem sido positiva, com incremento do número de doentes tratados e redução dos custos com fármacos específicos.