Claudio Arnaldo Len

Universidade Federal de São Paulo, San Paulo, São Paulo, Brazil

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Publications (104)184.22 Total impact

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    ABSTRACT: Devic's disease, also known as neuromyelitis optica, is an autoimmune inflammatory demyelinating disorder of the central nervous system that mainly affects the optic nerve and spinal cord. Recently, Devic's disease was demonstrated to be a channelopathy due to the presence of antibodies against the water channel aquaporin-4 in the blood-brain barrier. There have been reports of Devic's disease in infancy, but there are few reported associations of Devic's disease with other diseases. The association of Devic's disease with dermatomyositis has not yet been described in the literature. The aim of this paper is to describe the first case of Devic's disease in an adolescent with juvenile dermatomyositis. Copyright © 2015 Elsevier Editora Ltda. All rights reserved.
    Revista Brasileira de Reumatologia 03/2015; 187. DOI:10.1016/j.rbre.2014.12.004 · 0.99 Impact Factor
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    ABSTRACT: Devic's disease, also known as neuromyelitis optica, is an autoimmune inflammatory demyelinating disorder of the central nervous system that mainly affects the optic nerve and spinal cord. Recently, Devic's disease was demonstrated to be a channelopathy due to the presence of antibodies against the water channel aquaporin‐4 in the blood‐brain barrier. There have been reports of Devic's disease in infancy, but there are few reported associations of Devic's disease with other diseases. The association of Devic's disease with dermatomyositis has not yet been described in the literature. The aim of this paper is to describe the first case of Devic's disease in an adolescent with juvenile dermatomyositis.
    Revista Brasileira de Reumatologia 01/2015; 187. DOI:10.1016/j.rbr.2014.12.004 · 0.99 Impact Factor
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    ABSTRACT: The case of a girl with recurring chorea and a Takayasu's arteritis diagnosis is reported. This clinical manifestation has been reported in only one patient with this vasculitis in the pediatric group.
    11/2014; DOI:10.1016/j.rbre.2013.09.003
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    ABSTRACT: The case of a girl with recurring chorea and a Takayasu's arteritis diagnosis is reported. This clinical manifestation has been reported in only one patient with this vasculitis in the pediatric group.
    Revista Brasileira de Reumatologia 08/2014; DOI:10.1016/j.rbr.2013.09.003 · 0.99 Impact Factor
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    ABSTRACT: We evaluated the concentrations of 25-hydroxyvitamin D [25(OH)D] in children and adolescents with juvenile systemic lupus erythematosus (JSLE) and associated them with disease duration and activity, use of medication (chloroquine and glucocorticoids), vitamin D intake, calcium and alkaline phosphatase levels, and bone mineral density. Thirty patients with JSLE were evaluated and compared to 30 healthy individuals, who were age and gender matched. Assessment was performed of clinical status, disease activity, anthropometry, laboratory markers, and bone mineral density. The 30 patients included 25 (83.3%) females and 16 (53.3%) Caucasians, with a mean age of 13.7 years. The mean age at diagnosis was 10.5 years and mean disease duration was 3.4 years. Mean levels of calcium, albumin, and alkaline phosphatase were significantly lower in patients with JSLE compared with controls (P<0.001, P=0.006, and P<0.001, respectively). Twenty-nine patients (97%) and 23 controls (77%) had 25(OH)D concentrations lower than 32 ng/mL, with significant differences between them (P<0.001). Fifteen patients (50%) had vitamin D levels <20 ng/mL and 14 had vitamin D levels between 20 and 32 ng/mL. However, these values were not associated with greater disease activity, higher levels of parathormone, medication intake, or bone mineral density. Vitamin D concentrations were similar with regard to ethnic group, body mass index, height for age, and pubertal stage. Significantly more frequently than in controls, we observed insufficient serum concentrations of 25(OH)D in patients with JSLE; however, we did not observe any association with disease activity, higher levels of parathormone, lower levels of alkaline phosphatase, use of medications, or bone mineral density alterations.
    Brazilian journal of medical and biological research = Revista brasileira de pesquisas medicas e biologicas / Sociedade Brasileira de Biofisica ... [et al.] 07/2014; DOI:10.1590/1414-431X20143948 · 1.08 Impact Factor
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    ABSTRACT: The aim of the study was (1) to translate the "Pediatric Quality of Life Inventory-Multidimensional Fatigue Scale" (PedsQL-Fatigue) into Brazilian Portuguese language and culture and evaluate its reliability and (2) to measure fatigue among patients with juvenile idiopathic arthritis (JIA): (1) Translation of the PedsQL-Fatigue by two bilingual researchers; (2) Backtranslation into English assessed by the authors of the original version; (3) Pilot study with five patients followed in the Pediatric Rheumatology Outpatient Clinic and their parents; and (4) Field study and assessment of measurement properties (internal consistency, reproducibility, and construct validity). In this stage, the scale was administered to 67 patients with JIA and 63 healthy individuals, aged from 2 to 18 years old, matched by age (from 2 to 4, 5 to 7, 8 to 12, and from 13 to 18 years old). Cronbach's alpha coefficient ranged from 0.6 to 0.8 for children and parents, indicating the instrument's good internal consistency. The scale's construct validity was confirmed by a satisfactory Spearman's coefficient between the PedsQL-Fatigue and the generic PedsQL 4.0 (0.840 for the children and 0.742 for the parents). Reproducibility was also adequate (0.764 for the children and 0.938 for the parents). No differences were found between the scores obtained by the JIA group and control group, though lower scores were observed among patients with clinically active JIA when compared to those without clinical activity. The PedsQL-Fatigue is a valid and reliable tool, and that can be used to measure fatigue among patients with JIA.
    Rheumatology International 06/2014; DOI:10.1007/s00296-014-3064-5 · 1.63 Impact Factor
  • Annals of the Rheumatic Diseases 06/2014; 73(Suppl 2):589-589. DOI:10.1136/annrheumdis-2014-eular.2279 · 9.27 Impact Factor
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    ABSTRACT: Medical treatment of juvenile idiopathic arthritis (JIA) has advanced in the last decade, and improved prognosis is a reality in daily clinical practice. Despite this improvement in the quality of treatment, the outcome can still be compromised by modifiable factors, including delayed referral to a specialist, delayed drug treatment, poor adherence to treatment, and early interruption of drug treatment. In this review we discuss the most relevant aspects related to adherence to treatment in JIA, with emphasis on: factors that affect adherence to treatment; effect of poor adherence to treatment on JIA prognosis; when to suspect and how to assess poor adherence to treatment; and strategies to promote adherence to treatment, with an emphasis on information-reinforcement education. Besides presenting the findings of other authors, we also try to report our experience of this subject, which is still a challenge for health professionals.
    Current Rheumatology Reports 04/2014; 16(4):410. DOI:10.1007/s11926-014-0410-2 · 2.45 Impact Factor
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    ABSTRACT: Background/Purpose:The intra-articular steroids injections (IASI) for treatment of juvenile idiopathic arthritis (JIA) patients are being increasingly used in pediatric rheumatology. Most on knowledge about IASI comes from studies on adults and animal models and there are few reports on children. The aims of this study were to describe and to assess the factors associated with good response to IASI in JIA patients.Methods:A retrospective study of JIA patients who were administered IASI with a minimum follow-up of six months was performed between January 2008 and December 2012. The response to IASI regarding the patients was classified as a good response (complete—all joints remained inactive for at least six months or partial—part of the joints remained inactive for at least six months) or poor response (all joints remained active or reactivated in less than six months). The response to IASI regarding the joints was classified as a good response (the joints remained inactive for at least six months) or poor response (the joints remained active or reactivated in less than six months).Results:A total of 165 IASI were administered to 88 patients. Of the 88 JIA patients, 66 were female, 35.2% were persistent oligoarticular, 19.3% extended oligoarticular, 38.6% polyarticular, 4.5% systemic, 1.1% enthesitis related arthritis and 1.1% psoriatic. Patients were grouped according to disease course in oligoarticular—37.5%, polyarticular—58% and systemic—4.5%. The mean time to diagnosis was 10.5 ± 16 months, the mean age at diagnosis was 6.8 ± 4 years and the mean follow-up time was 7.0 ± 4 years. Sixty-two patients (70.4%) showed good response to IASI, 44.3 % complete and 26.1% partial. There were no associations between the responses to the IASI and gender (p = 0.42), JIA subtype (p = 0.07), disease course (p = 0.08), antinuclear antibodies (p = 0.12), rheumatoid factor (p = 0.27), time to diagnosis (p = 0.73) and mean follow-up time (p = 0.08). There was an association between good response to IASI and presence of uveitis (p = 0.01) and earlier age of diagnosis (p = 0.03). From the 165 joints injected, there were 100 knees, 30 ankles, 15 wrists, 9 elbows, 6 hips, 3 shoulders and 2 hand's proximal interphalangeal. A hundred and four joints (63%) showed good response to IASI, with mean duration of 18.1 ± 13 months. There were no associations between the responses to the IASI and the severity of joint effusion (p = 0.49), use of DMARDs (p = 0.31) or oral steroids (p = 0.55), time of follow-up until the IASI (p = 0.25), age at IASI (p = 0.53), CHAQ (p = 0.53), C reactive protein (p = 0.22), erythrocyte sedimentation rate (p = 0.41) and number of active (p = 0.09) and limited (p = 0.77) joints at the time of IASI. There was an association between good response to IASI and the injected joint (p = 0.001)—knees, wrists and elbows, the use of ultrasonography to guide the IASI (p = 0.02) and the parents (p = 0.01) and physician (p = 0.01) visual analogue scale. Local adverse events occurred in nine (0.05%) IASI (five atrophy and four hypochromia).Conclusion:This study showed that IASI are effective, safe and have a good sustained response. More prospective studies are needed to better define the profile of JIA patients who may benefit from IASI.
    03/2014; 66(S11). DOI:10.1002/art.38462
  • Annals of the Rheumatic Diseases 01/2014; 72(Suppl 3):A496-A496. DOI:10.1136/annrheumdis-2013-eular.1490 · 9.27 Impact Factor
  • Annals of the Rheumatic Diseases 01/2014; 71(Suppl 3):262-263. DOI:10.1136/annrheumdis-2012-eular.2281 · 9.27 Impact Factor
  • Annals of the Rheumatic Diseases 01/2014; 71(Suppl 3):701-701. DOI:10.1136/annrheumdis-2012-eular.1105 · 9.27 Impact Factor
  • Annals of the Rheumatic Diseases 01/2014; 72(Suppl 3):A325-A326. DOI:10.1136/annrheumdis-2013-eular.1005 · 9.27 Impact Factor
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    ABSTRACT: Rheumatic diseases in children are associated with significant morbidity and poor health-related quality of life (HRQOL). There is no health-related quality of life (HRQOL) scale available specifically for children with less common rheumatic diseases. These diseases share several features with systemic lupus erythematosus (SLE) such as their chronic episodic nature, multi-systemic involvement, and the need for immunosuppressive medications. HRQOL scale developed for pediatric SLE will likely be applicable to children with systemic inflammatory diseases. We adapted Simple Measure of Impact of Lupus Erythematosus in Youngsters (SMILEY©) to Simple Measure of Impact of Illness in Youngsters (SMILY©-Illness) and had it reviewed by pediatric rheumatologists for its appropriateness and cultural suitability. We tested SMILY©-Illness in patients with inflammatory rheumatic diseases and then translated it into 28 languages. Nineteen children (79% female, n=15) and 17 parents participated. The mean age was 12±4 years, with median disease duration of 21 months (1-172 months). We translated SMILY©-Illness into the following 28 languages: Danish, Dutch, French (France), English (UK), German (Germany), German (Austria), German (Switzerland), Hebrew, Italian, Portuguese (Brazil), Slovene, Spanish (USA and Puerto Rico), Spanish (Spain), Spanish (Argentina), Spanish (Mexico), Spanish (Venezuela), Turkish, Afrikaans, Arabic (Saudi Arabia), Arabic (Egypt), Czech, Greek, Hindi, Hungarian, Japanese, Romanian, Serbian and Xhosa. SMILY©-Illness is a brief, easy to administer and score HRQOL scale for children with systemic rheumatic diseases. It is suitable for use across different age groups and literacy levels. SMILY©-Illness with its available translations may be used as useful adjuncts to clinical practice and research.
  • Pediatric Rheumatology 12/2013; 11(Suppl 2):P170. DOI:10.1186/1546-0096-11-S2-P170 · 1.62 Impact Factor
  • Pediatric Rheumatology 12/2013; 11(Suppl 2):P251. DOI:10.1186/1546-0096-11-S2-P251 · 1.62 Impact Factor
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    ABSTRACT: The aim of the study was to study the prevalence of juvenile idiopathic arthritis (JIA) in school children in the city of Embu das Artes in São Paulo State. 2880 school children from seven public schools, aged between 6 and 12 years, were evaluated (clinical findings) by a pediatric rheumatologist. A board certified Pediatric Rheumatologist evaluated the subjects with suspected inflammatory arthropathy. Children with higher suspicion were referred to a specialized service. One hundred and forty-one children have presented abnormalities on examination of musculoskeletal system, with isolated pain on palpation the most common finding in the first evaluation (60.9%), with improvement in almost all cases in the second examination. Most of the abnormalities were related to recent injuries or congenital malformations. Six children have clinical findings suggestive of chronic arthropathy and were referred to a specialized pediatric rheumatology clinic. Of these, a 12 year-old girl fulfilled the criteria for JIA. The other diagnoses were aseptic necrosis of the hip (P = 1) of and post-trauma synovitis (P = 4). The prevalence of JIA in children aged between 6 and 12 years was 1/2.880 (or 0.34/1.000).
    Revista Brasileira de Reumatologia 12/2013; 53(6):542-545. DOI:10.1016/j.rbr.2013.04.005 · 0.99 Impact Factor
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    ABSTRACT: This study aimed to translate the 'Pediatric Quality of Life Inventory™ (PedsQL™ 3.0) Cardiac Module' into Portuguese, adapt it to Brazilian culture, and assess its psychometric properties (validity and reproducibility), and to calculate health-related quality of life scores on the PedsQL 4.0 and PedsQL™ 3.0 Cardiac Module Scales for a group of patients 5 to 18 years old with rheumatic heart disease. The methods suggested by the authors of the original version of the questionnaire included 1) translation by an expert panel; 2) translation back into English and revision by the authors of the original version; 3) pilot study with seven children and parents in each of three age ranges (5 to 7, 8 to 12, and 13 to 18 years old); and 4) assessment of the measurement properties. In this stage, the PedsQL™ 3.0 Cardiac Module and the PedsQL 4.0 Generic Scale were applied to a sample comprising 109 children and adolescents with rheumatic heart disease and their parents or caregivers. The version for parents or caregivers was administered separately on the same day. The values of Cronbach's alpha for all scales assessed in the questionnaire (heart problems and treatment [symptoms], problems with perceived physical appearance, treatment anxiety, cognitive problems, and communication problems) varied from 0.6 to 0.8, indicating good internal consistency. Correlation was found between the scores for the Cardiac Module and the Generic Scale (0.36-0.86), demonstrating convergent validity (Spearman's correlation coefficient, p < 0.01). The symptoms, problems with perceived physical appearance, and cognitive and communication problem domains were able to distinguish between groups of patients with mild and moderate/severe heart disease (Student's t-test, p < 0.05). The intraclass correlation of the interobserver reproducibility was adequate (0.76 to 0.94 among the patients [children/adolescents] and 0.76 to 0.84 among their caregivers). The correlation between the patients' scores and their parents' scores varied from 0.50 to 0.86 (Pearson's correlation coefficient, p < 0.01). The Brazilian version of the PedsQL™ 3.0 Cardiac Module was shown to be reliable. The application of this questionnaire in practice will be very useful for all professionals charged with the care of children and adolescents with heart diseases.
    Health and Quality of Life Outcomes 11/2013; 11(1):198. DOI:10.1186/1477-7525-11-198 · 2.10 Impact Factor
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    ABSTRACT: Paediatric rheumatology (PR) is an emerging specialty, practised by a limited number of specialists. Currently, there is neither a record of the profile of rheumatology patients being treated in Brazil nor data on the training of qualified rheumatology professionals in the country. To investigate the profile of PR specialists and services, as well as the characteristics of paediatric patients with rheumatic diseases, for estimating the current state of rheumatology in the state of São Paulo. In 2010, the scientific department of PR of the Paediatric Society of São Paulo administered a questionnaire that was answered by 24/31 accredited specialists in PR practising in state of São Paulo and by 8/21 institutions that provide PR care. Most (91%) of the surveyed professionals practise in public institutions. Private clinics (28.6%) and public institutions (37.5%) reported not having access to nailfold capillaroscopy, and 50% of the private clinics reported not having access to acupuncture. The average duration of professional practise in PR was 9.4 years, and 67% of the physicians had attended postgraduate programmes. Seven (87.5%) public institutions perform teaching activities, in which new paediatric rheumatologists are trained, and five (62.5%) offer postgraduate programmes. Two-thirds of the surveyed specialists use immunosuppressants and biological agents classified as "restricted use" by the Health Secretariat. The disease most frequently reported was juvenile idiopathic arthritis (29.1-34.5%), followed by juvenile systemic lupus erythematosus (JSLE) (11.6-12.3%) and rheumatic fever (9.1-15.9%). The incidence of vasculitis (including Henoch-Schönlein purpura, Wegener's granulomatosis, and Takayasu's arteritis) and autoinflammatory syndromes was higher in public institutions compared to other institutions (P = 0.03, P = 0.04, P = 0.002, and P < 0.0001, respectively). Patients with JSLE had the highest mortality rate (68% of deaths), mainly due to infection. The field of PR in the state of São Paulo has a significant number of specialists with postgraduate degrees who mostly practise at teaching institutions with infrastructures appropriate for the care of high-complexity patients.
    Revista Brasileira de Reumatologia 08/2013; 53(4):346-351. DOI:10.1590/S0482-50042013000400006 · 0.99 Impact Factor
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    ABSTRACT: To determine the effects of Pilates exercises on health-related quality of life (HRQL) in individuals with juvenile idiopathic arthritis (JIA). A randomized, prospective, single-blind trial. Outpatient clinic of pediatric rheumatology and the rehabilitation department. Children (N=50) with JIA according to the International League of Associations for Rheumatology (ILAR) criteria. The participants were randomly assigned into 2 groups. In Group I (N=25), the participants were given a conventional exercise program for 6 months. Patients in Group II (N=25) participated in a Pilates exercise program for 6 months. The primary outcome measure was HRQL, as measured by the Pediatric Quality of Life Inventory (PedsQL 4.0). The secondary outcome measures provided an estimate of the clinical relevance of the primary outcome results and included joint pain intensity [according to a 10-cm visual analog scale (VAS Joint Pain)], disability [according to the Childhood Health Assessment Questionnaire (CHAQ)], joint status [using the pediatric Escola Paulista de Medicina Range of Motion Scale (pEPM-ROM)] and the total PedsQL 4.0 score. All of the participants completed the study. The scores of the PedsQL 4.0 differed significantly between the groups, indicating that the Pilates exercises increased these scores when compared to the conventional exercise program. The Group II participants showed significant improvements in the 10-cm VAS-Joint Pain, CHAQ and pEPM-ROM. The use of Pilates exercises had a positive physical and psychosocial impact on the HRQL of individuals with JIA. Future multicenter studies with a follow-up beyond the period of treatment using more objective parameters will be useful to support the results of the present study.
    Archives of physical medicine and rehabilitation 06/2013; 94(11). DOI:10.1016/j.apmr.2013.05.026 · 2.44 Impact Factor

Publication Stats

500 Citations
184.22 Total Impact Points

Institutions

  • 2000–2015
    • Universidade Federal de São Paulo
      • • Departamento de Pediatria
      • • Escola Paulista de Medicina (EPM)
      San Paulo, São Paulo, Brazil
  • 2013
    • IRCCS Istituto G. Gaslini
      Genova, Liguria, Italy
  • 2008–2012
    • Senac São Paulo
      San Paulo, São Paulo, Brazil
    • University of Iowa Children's Hospital
      Iowa City, Iowa, United States
  • 2009
    • University of São Paulo
      San Paulo, São Paulo, Brazil
  • 2002
    • EPM
      Medellín, Antioquia, Colombia