Early detection of interstitial lung disease in rheumatic diseases: A joint statement from the Portuguese Pulmonology Society, the Portuguese Rheumatology Society, and the Portuguese Radiology and Nuclear Medicine Society.

INTRODUCTION: Interstitial lung disease (ILD) contributes significantly to morbidity and mortality in connective tissue disease (CTD). Early detection and accurate diagnosis are essential for informing treatment decisions and prognosis in this setting. Clear guidance on CTD-ILD screening, however, is lacking. OBJECTIVE: To establish recommendations for CTD-ILD screening based on the current evidence. METHOD: Following an extensive literature research and evaluation of articles selected for their recency and relevance to the characterization, screening, and management of CTD-ILD, an expert panel formed by six pulmonologists from the Portuguese Society of Pulmonology, six rheumatologists from the Portuguese Society of Rheumatology, and six radiologists from the Portuguese Society of Radiology and Nuclear Medicine participated in a multidisciplinary discussion to produce a joint statement on screening recommendations for ILD in CTD. RESULTS: The expert panel achieved consensus on when and how to screen for ILD in patients with systemic sclerosis, rheumatoid arthritis, mixed connective tissue disease, Sjögren syndrome, idiopathic inflammatory myopathies and systemic lupus erythematous. CONCLUSIONS: Despite the lack of data on screening for CTD-ILD, an expert panel of pulmonologists, rheumatologists and radiologists agreed on a series of screening recommendations to support decision-making and enable early diagnosis of ILD to ultimately improve outcomes and prognosis in patients with CTD.

Effects of a respiratory functional training program on pain and sleep quality in patients with fibromyalgia: A pilot study.

OBJECTIVE: To evaluate the effect of 8-week respiratory functional training program on pain tolerance, sleep, and urinary antioxidant and cortisol levels in 18 patients with fibromyalgia. METHODS: Participants underwent a 12-week intervention: 4 weeks as control and 8 weeks of breathing exercises. Pain tolerance assay was done by using an algometer, whereas sleep quality was evaluated by actigraphy and by the Pittsburgh Sleep Quality Index. Cortisol and antioxidant levels were determined using commercial assay kits. RESULTS: Increases in the pain tolerance threshold were detected in the occiput point after one month of intervention as well as in the low cervical and second rib points after one and two months. Actigraphy revealed a decrease in sleep latency, whereas sleep questionnaire showed improvements in sleep quality, sleep duration and sleep efficiency. No changes in cortisol and antioxidant levels were detected. CONCLUSION: The 8-week breathing exercise intervention reduced pain and improved sleep quality.

The economic impact of early retirement attributed to rheumatic diseases: results from a nationwide population-based epidemiologic study.

OBJECTIVES: To measure early retirement due to self-reported rheumatic diseases (RDs) and to estimate the respective indirect costs and years of working life lost (YWLL). METHODS: We used individual level data from the national, cross-sectional, population-based EpiReumaPt study (September 2011-December 2013) where 10,661 inhabitants were randomly surveyed in order to capture and characterize all cases of RD within a representative sample of the Portuguese population. In this analysis, we used all participants aged between 50 and 64 years, near the official retirement age. A national database was used to calculate productivity values by gender, age and region, using the human capital approach. YWLL were estimated as the difference between each participant's current age and the respective retirement age, while the potential years of working life lost (PYWLL) were given by the difference between official and actual retirement ages. We also calculated the percentage of time in inactivity (inactivity ratio = YWLL/Active age-range [15-64 years old]). RESULTS: 29.9% of the Portuguese population with ages between 50 and 64 years were retired with 13.1% self-reporting retirement due to RD. The estimated annual indirect cost following premature retirement attributed to RD was €910 million (€555 per capita; €1625 per self-reported RD patient and €13,592 per early retiree due to RD). Females contributed with 84% for these costs (€766 million; €882 per capita vs €187 from males). We observed a total number of 389,939 accumulated YWLL (228 per 1000 inhabitants) and 684,960 PYWLL (401 per 1000 inhabitants). The mean YWLL and PYWLL inactivity ratios were 12% and 21%, respectively. RD patients with higher values of disability have the highest risk of early retirement. CONCLUSIONS: Early retirement attributed to self-reported RD amounts to approximately 0.5% of the national gross domestic product (GDP) in 2013, due to large YWLL. Both the public health concern and the economic impact highlight the need to prioritize investments in health and social protection policies targeting patients with rheumatic conditions.

Effect of Comedication With Conventional Synthetic Disease-Modifying Antirheumatic Drugs on Retention of Tumor Necrosis Factor Inhibitors in Patients With Spondyloarthritis: A Prospective Cohort Study.

OBJECTIVE: To evaluate whether use of comedication with conventional synthetic disease-modifying antirheumatic drugs (csDMARDs) influences the retention of tumor necrosis factor inhibitors (TNFi) in patients with spondyloarthritis (SpA). METHODS: Patients with SpA from the Rheumatic Diseases Portuguese Register who started treatment with their first TNFi between 2001 and 2014 were included in this study. Cox regression analysis was used to estimate the effect of comedication with csDMARDs on TNFi retention in 2 types of models: a model in which baseline (time-fixed) variables were included, and a second model incorporating time-varying variables, including sociodemographic features, measures of disease activity, measures of physical function, and cotreatment with other drugs (nonsteroidal antiinflammatory drugs and oral steroids). To control for possible confounding by indication, the effect of csDMARD comedication on TNFi retention was also tested after adjustment for the treatment propensity score. RESULTS: In total, 954 patients were included in the study, of whom 289 (30.3%) discontinued treatment with their first TNFi after a median follow-up time of 2.5 years (range 0.08-13 years). Inefficacy was the most common reason for TNFi discontinuation (55.7% of patients). In the multivariable analyses, comedication with csDMARDs had no measurable effect on TNFi retention, neither in the baseline model (hazard ratio [HR] 0.83, 95% confidence interval [95% CI] 0.59-1.16) nor during follow-up in the model adjusted for time-varying covariates (HR 1.07, 95% CI 0.68-1.68). The effect of csDMARD comedication remained nonsignificant after propensity score adjustment. CONCLUSION: Comedication with csDMARDs does not prolong TNFi retention in patients with SpA in clinical practice, suggesting that there is no benefit conferred by the concomitant use of these drugs.

Childhood trauma, family history, and their association with mood disorders in early adulthood.

OBJECTIVE: To assess the prevalence of childhood trauma and types of trauma on mood disorders among young adults in a population-based sample. We further gathered data on family history of mood disorders to test the hypothesis that childhood trauma is a mediating factor for the association between family history of mood disorder and mood disorder in adulthood. METHOD: This is a cross-sectional study, including young adults with bipolar disorder, major depressive disorder, and matched controls without any mood disorder. Childhood trauma was assessed using the Childhood Trauma Questionnaire (CTQ). The Hicks and Tingley implementation was employed to assess whether trauma is a mediator of the effect of family history on diagnosis of any mood disorder. RESULTS: All types of trauma were associated with both major depression and bipolar disorder, with the exception of sexual abuse, which was only associated with bipolar disorder. Moreover, family history of psychiatric illness was also associated with mood disorder in adulthood and with childhood trauma. Using the presence of any mood disorder as the outcome, a third of the effect of having any family history of mood disorder was mediated via childhood trauma. CONCLUSION: This investigation provides further support, in a population-based sample of young adults, of the association between childhood trauma and mood disorders, with sexual abuse being specifically linked with bipolar disorder. The hypothesis that childhood trauma would function as a partial mediator of the association between family history of mood disorder and mood disorder in adulthood was also confirmed.

[Relationships between widespread pain and thresholds pain tolerance on tender points in Portuguese women with fibromyalgia: impact on daily life]. / Relação entre a dor generalizada e o limiar de tolerância à dor nos pontos sensíveis em mulheres portuguesas com fibromialgia: impacto sobre a vida diária.

OBJECTIVES: to establish a relationship between widespread pain subjectively perceived and threshold pain tolerance on tender points, and to determine whether there are differences in threshold pain tolerance on tender points between the upper and lower body, as well as between the dominant and non-dominant side, and whether these differences have an impact on the daily life of Portuguese women with fibromyalgia (FM). MATERIAL & METHODS: thirty-one women with FM aged between 34 and 67 years volunteered for the study. Threshold pain tolerance was assessed at critical points using a digital algometer pressure; the widespread pain index (WPI) was constructed by the addition of 19 painful body regions; and the impact on the daily life assessed by the Portuguese version of the Fibromyalgia Impact Questionnaire (FIQ), with individual interviews. RESULTS: significant differences between the percentage of threshold pain tolerance of the whole body and the scales of widespread pain subjectively perceived were observed, showing that the widespread pain subjectively perceived by patients was between +25.9% and +27.5%. Also, significant differences between threshold pain tolerance of tender points located on the upper and lower body (1.9 ± 0.5 kg/cm2 vs. 2.6 ± 0.7 kg/cm2; respectively) were observed. However, no significant differences were found between threshold pain tolerance of tender points located on dominant and non-dominant sides (2.1 ± 0.5 kg/cm2 e 2.1 ± 0.6 kg/cm2; respectively). Additionally, the analysis showed significant correlations between pain and patient`s daily life in: FIQ total score, physical function, feel good, job ability and fatigue. CONCLUSIONS: the women with FM show higher widespread pain subjectively perceived than threshold pain tolerance on tender points. Furthermore, the pain suffered by the patients with FM, especially that located on the upper body, either on the dominant or on the non-dominant side, has a negative influence on physical function, job ability, fatigue and feel good, affecting daily life.

[Epidemiology of hip fractures and its social and economic impact. An update for 2014]. / ACTUALIZAÇÃO SOBRE O IMPACTO EPIDEMIOLÓGICO E SOCIOECONÓMICO DAS FRACTURAS DA EXTREMIDADE PROXIMAL DO FÉMUR.

Hip fractures (HF) following low-impact trauma are the most visible and dramatic consequences of osteoporosis (OP). It is estimated that within one year after HF, 20 to 30% of the patients die, 50-60% become disabled and only 30-40% fully recover their previous functional levels. Therefore, its medical, societal and economic impact is huge and it is not fully delivered by the event itself, but rather by its consequences. This paper aims to update data published by Branco et al. 2009 about epidemiology and burden of HF, specifically to revise the national data. In Portugal there have been 77,083 HF reported between 2000 and 2008 with increasing numbers with ageing along with due associated refracture and mortality rates. Recent results suggest a slight change at clinical level concerning the pharmacological approach for the treatment of HF, however it is still important to stay alert to the patients' needs and to their bone mineral density losses in order to avoid repeating the occurrence of these events, aiming to generate significant benefits in terms of health outcomes and due public expenditure.

Portuguese recommendations for the diagnosis and management of gout.

OBJECTIVE: To develop Portuguese evidence-based recommendations for the Diagnosis and Management of Gout. METHODS: As part of the 3e Initiative (Evidence, Expertise and Exchange), a panel of 78 international rheumatologists developed 10 relevant clinical questions which were investigated with systematic literature reviews. MEDLINE, EMBASE, Cochrane CENTRAL and abstracts from 2010-2011 EULAR and ACR meetings were searched. Based on the evidence found in the published literature, rheumatologists from 14 countries developed national recommendations that were merged and voted into multinational recommendations. We present the Portuguese recommendations for the Diagnosis and Management of Gout which were formulated and voted by Delphi method in April 2012, in Lisbon. The level of agreement and potential impact in clinical practice was also assessed. RESULTS: Twelve national recommendations were elaborated from 10 international and 2 national questions. These recommendations addressed the diagnosis of gout; the treatment of acute flares and urate-lowering therapy; monitoring of gout and comorbidity screening; the influence of comorbidities in drug choice; lifestyle; flare prophylaxis; management of tophi and asymptomatic hyperuricaemia; the role of urine alkalinization; and the burden of gout. The level of agreement with the recommendations ranged from 6.8 to 9.0 (mean 7.7) on a 1-10 point visual analogue scale, in which 10 stands for full agreement. CONCLUSION: The 12 Portuguese recommendations for the Diagnosis and Management of Gout were formulated according to the best evidence and endorsed by a panel of 42 rheumatologists, enhancing their validity and practical use in daily clinical practice.

HLA alleles and HLA-B27 haplotypes associated with susceptibility and severity of ankylosing spondylitis in a Portuguese population.

Human leukocyte antigen (HLA)-B27 is the mostly known major histocompatibility complex (MHC) gene associated with ankylosing spondylitis (AS). Nonetheless, there is substantial evidence that other MHC genes appear to be associated with the disease, although it has not yet been established whether these associations are driven by direct associations or by linkage disequilibrium (LD) mechanisms. We aimed to investigate the contributions of HLA class I and II alleles and B27-haplotypes for AS in a case-control study. A total of 188 HLA-B27 AS cases and 189 HLA-B27 healthy controls were selected and typed for HLA class I and II by the Luminex polymerase chain reaction-sequence specific oligonucleotide probe (PCR-SSOP) method. Allelic and haplotypic distributions were estimated by maximum likelihood method using Arlequin v3.11 and statistical analysis were performed by Stata10.1. No associations were found between non-HLA-B27 loci and AS susceptibility, but several associations were observed for phenotypic features of the disease. DRB1*08 was identified as a risk factor for uveitis and DQB1*04 seems to provide protection for AS severity (functional, metrological and radiological indexes). A*02/B27/C*02/DRB1*01/DQB1*05 [P<0.0001; odds ratio (OR) = 39.06; 95% confidence interval (CI) (2.34-651)] is the only haplotype that seems to confer susceptibility to AS. Moreover, the haplotype A*02/B27/C*01/DRB1*08/DQB1*04 seems to provide protection for disease functional and radiological repercussions. Our findings are compatible with the hypothesis that other genes within the HLA region besides HLA-B27 might play some role in AS susceptibility and severity.

Childhood hypophosphatasia with myopathy: clinical report with recent update.

Hypophosphatasia is a rare genetic disease with low tissue nonspeficic alkaline phosphatase activity (TNSALP), due to ALPL gene mutation. There are 6 clinical forms. Childhood form is caractherized by short stature, premature loss of decidous teeth and diffuse bone pain associated with a pathological bone fracture in the past. Laboratory findings present low serum level of alkaline phosphatase and high levels of serum and urinary extracelular metabolytes. It is described a case report of a 34 years old woman with previous diagnosis of childhood hypophosphatasia, caryotype 46,XX, and molecular screening for the gene ALPL with a c.1426>A p.E476K mutation, who complained of proximal muscular weakness intensified with the cold weather, exercise, and a waddling gait. The electromyography was compatible with myopathy but the muscle biopsy was normal. The serum creatine kinase levels were normal, as well as the others muscle enzymes. Clinical and laboratory/ /imaging dissociation is frequent in other metabolic bone diseases as osteomalacia. The rarity of this case of childhood hypophosphatasia with "de novo" non-progressive myopathy of the lower limbs, justified a case report with literature revision.

Portuguese version of the bath indexes for ankylosing spondylitis patients: a cross-cultural adaptation and validation.

The Bath Ankylosing Spondylitis Activity Index (BASDAI), Functional Index (BASFI), Metrology Index (BASMI), and Global Score (BASG) are commonly used to assess patients with ankylosing spondylitis (AS). The aim of this study was to cross-culturally adapt and validate these indexes into the Portuguese language. Seventy-eight patients were included in the study. After forward and backward translations, the questionnaires were administered and tested for internal consistency, test-retest reliability, face validity, content validity, and construct validity. The outcome measures HAQ, EQ-5D, and SF-36 were also implemented. Metrological parameters (BASMI components) and chest expansion were evaluated. Correlation coefficients for test-retest were 0.875, 0.937, 0.831, and 0.961 for BASDAI, BASFI, BASMI, and BASG, respectively. Internal consistency coefficients were between 0.747 and 0.953. The adapted and translated questionnaires demonstrated an acceptable comprehensibility by a panel of patients, and face validity was assured by the cognitive debriefing performed. Content validity was assured by comparing the scores obtained by the questionnaires when age and gender, age of symptoms onset, and disease duration were considered. Construct validity was assured by significant correlations established between the Bath scores and generic health status HAQ, EQ-5D and SF-36, morning stiffness duration, chest expansion, and physician disease activity assessment. The Portuguese version of the BASDAI, BASFI, BASG, and BASMI showed adequate reliability and validity in patients with AS. The measurement properties were comparable to versions in other languages, indicating that the indexes can be used for evaluation of Portuguese-speaking AS patients.

[Assessment of erythrocyte sedimentation rate (ESR) and C- reactive protein (CRP) on rheumatoid arthritis activity prediction]. / Velocidade de sedimentação ou proteína c reactiva, que variáveis utilizar na avaliação clínica dos doentes com artrite reumatóide?

OBJECTIVE: Rheumatoid Arthritis (RA) disease activity plays an important role in patients disability. A standardized approach to measure it was achieved by using disease activity score (DAS) based on erythrocyte sedimentation rate (ESR) and more recently C-reactive protein (CRP). In this study we will assess the role and influence of ESR and CRP in evaluating and assessing the disease activity. METHODS: This is a retrospective, longitudinal study, whose data was obtained from the national RA patient clinical database BioreportAR (following ACR criteria) under biological disease modifying anti-rheumatic drugs (BDMARDs), involving 71 patients from CHLO-Hospital Egas Moniz. The ESR and CRP of each patient were recorded from 2 separated visits. The swollen joint counts (SJC), tender joint counts (TJC), visual analog scale (VAS) for pain, patient global assessment and physician global assessment were also recorded. DAS 28-ESR, DAS 28-CRP, simplified disease activity index (SDAI) and clinical disease activity index (CDAI) were calculated. The relevant Pearson correlations were established between variables. RESULTS: A sample of 71 patients with an average age of 55 years old and an average RA duration of 9,8 years, was analysed. ESR and CRP correlated poorly between themselves (r=0,31, p<0,001) and they were shown not to be significant predictors of SJC (ESR r=0,29 p< 0,001 and CRP r=0,089 p<0,001) or TJC (ESR r=0,28 p<0,001 and CRP r=0,072 p<0,001). However DAS-ESR and DAS-CRP were highly correlated (r=0,88, p<0,001) as also as, DAS ESR-CDAI (r=0,89, p<0,001), DAS RCP-CDAI (r= 0,88 p< 0,001) and SDAI-CDAI (r=0,89 p<0,001). DAS-CRP values were lower than those in DAS-ESR, but in 84,7% of the visits disease activity status were. CONCLUSIONS: The significant correlation between DAS-ESR and DAS-CRP, indicated that it will not be necessary to perform both evaluations. DAS-CRP yielded a better activity score more often than DAS-ESR, but with 84,7% of concordance in the disease activity status, indicating that both measures are useful for assessing disease activity in RA. Furthermore the correlation between DAS scores and CDAI, and also between SDAI-CDAI may enable physicians to easily assess the disease activity without ESR or CRP values.

[Fibrous dysplasia and McCune-Albright syndrome: case-report and differential diagnose with Paget bone disease]. / Fibrodisplasia óssea e síndrome de McCune-Albright: descrição de um caso clínico e diagnóstico diferencial com doença óssea de Paget.

Fibrous dysplasia of bone or Jaffe Lischtenstein's disease is a genetic, non-inheritable disease of bone development, characterized by bone pain, deformities and fracture, mainly observed in yo ung adults. The frequency is equal between sexes. Mutations in the gene coding the Gsα, GNAS complex, results in osteoblastic differentiation defects, and bone resorption. The disease can have a monostotic or polyostotic form, or be associated with café-au-lait skin spots and precocious puberty (McCune-Al bright syndrome). The normal bone and bone marrow is replaced with abnormal benign intramedullary fibro-osseous tissue, and can involve any bone in the body. The vertebral involvement is rare. Radiological and pathological findings can be diagnostic. Biphosphonates and calcium, vitamin D and phosphorus supplements have been used in fibrous dysplasia treatment. Osteosarcoma is a rare, but serious malignant complication. We report the case of a 68 year old woman with a history of hypofisectomy, with a progressive low back pain, without systemic or neurological symptoms. The bone scan, the ra di o graphs and the computed tomography findings revealed polyostotic fibrous dysplasia, with vertebral and mandibular involvement. In this paper we compared fibrous dysplasia of bone with Paget bone disease.

Association of IL23R and ERAP1 genes with ankylosing spondylitis in a Portuguese population.

OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

EULAR evidence-based recommendations for the management of fibromyalgia syndrome.

OBJECTIVE: To develop evidence-based recommendations for the management of fibromyalgia syndrome. METHODS: A multidisciplinary task force was formed representing 11 European countries. The design of the study, including search strategy, participants, interventions, outcome measures, data collection and analytical method, was defined at the outset. A systematic review was undertaken with the keywords "fibromyalgia", "treatment or management" and "trial". Studies were excluded if they did not utilise the American College of Rheumatology classification criteria, were not clinical trials, or included patients with chronic fatigue syndrome or myalgic encephalomyelitis. Primary outcome measures were change in pain assessed by visual analogue scale and fibromyalgia impact questionnaire. The quality of the studies was categorised based on randomisation, blinding and allocation concealment. Only the highest quality studies were used to base recommendations on. When there was insufficient evidence from the literature, a Delphi process was used to provide basis for recommendation. RESULTS: 146 studies were eligible for the review. 39 pharmacological intervention studies and 59 non-pharmacological were included in the final recommendation summary tables once those of a lower quality or with insufficient data were separated. The categories of treatment identified were antidepressants, analgesics, and "other pharmacological" and exercise, cognitive behavioural therapy, education, dietary interventions and "other non-pharmacological". In many studies sample size was small and the quality of the study was insufficient for strong recommendations to be made. CONCLUSIONS: Nine recommendations for the management of fibromyalgia syndrome were developed using a systematic review and expert consensus.

[Genetic basis of ankylosing spondylitis]. / Espondilite Anquilosante e sua base genética.

Ankylosing spondylitis (AS) is a common rheumatic condition, highly heritable. Much of the genetic contribution to the disease lies in the major histocompatibility complex (MHC). The association with the allele group HLA-B*27 has been described worldwide for 30 years. On the other hand, genome wide scans have provided some interesting results showing that other MHC and non-MHC genes could be implicated either in disease susceptibility and phenotypic manifestations. Different hypothesis for disease pathophysiology have been investigated which contribute for a better understanding of the genetic basis of AS. This review aims to summarize the status of the knowledge in this exciting area. New data may, in a near future, change the screening of patients and generate new insights for the emergence of novel therapies.

[Systemic lupus erythematosus and anaemia]. / Lúpus eritematoso sistiémico e anemia.

The authors report the case of a 48-years-old Caucasian women, with a previous diagnosis of systemic lupus erythematosus characterized by asthenia, fever, skin rash, alopecia, Raynaud's phenomenon, arthritis, pericardial effusion, interstitial pulmonary involvement, diffuse proliferative glomerulonephritis with crescents and anemia. The presence of severe anemia refractory to high doses of glucocorticoids (1 mg/ /Kg/day), iron therapy and blood transfusions, associated with a low reticulocyte count determined the execution of a bone marrow aspiration, biopsy and immunophenotyping, which were compatible with the diagnosis of Myelodysplastic Syndrome. The treatment with erythropoietin (5.000U 3x/week) and cyclophosphamide pulses (1 gr/m(2) month) induced complete regression of morphologic bone marrow changes and anemia. The main causes of anemia in lupus patients are discussed.