[Show abstract][Hide abstract] 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.
[Show abstract][Hide abstract] 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.
[Show abstract][Hide abstract] 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.
[Show abstract][Hide abstract] 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.
[Show abstract][Hide abstract] 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. · 0.86 Impact Factor
[Show abstract][Hide abstract] 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. · 0.86 Impact Factor
[Show abstract][Hide abstract] 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; · 2.18 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: OBJECTIVE: To evaluate the quality of life in children and adolescents with sickle cell disease attending a blood reference center, and to assess the quality of life of their relatives. METHODS: Cross-sectional study that included 100 patients with sickle cell disease, which were divided into three subgroups according to age: 5 to 7 (n=18), 8 to 12 (n=32), and 13 to 18 years-old (n=50), and their parents. The Control Group included 50 healthy children and adolescents from a public local school, also divided into the same three age subgroups and their caregivers. The Pediatric Quality of life Inventory (PedsQL), version 4.0, was applied in both groups. The generic questionnaire Medical Outcomes Study 36 - Item Short-Form Health Survey (SF-36) was applied to the relatives. The answers were linearly transformed into a score and compared by non-parametric tests. RESULTS: The PedsQL scores of patients were significantly lower than those obtained in the Control Group (p<0.0001) in all studied areas (physical, emotional, social skills, and school activities). Similarly, SF-36 scores applied to the patients' parents were lower than those obtained in the Control Group in all studied aspects (p<0.0001). CONCLUSIONS: Sickle cell disease affects the quality of life of children, adolescents, and their families. Patients sense restrictions in the emotional, social, family and physical aspects, among others.
Revista paulista de pediatria : orgao oficial da Sociedade de Pediatria de Sao Paulo. 03/2013; 31(1):24-29.
[Show abstract][Hide abstract] ABSTRACT: Our objective was to evaluate the concentrations of serum 25-hydroxyvitamin D [25(OH)D], serum calcium, serum phosphorus, alkaline phosphatase, and parathormone (PTH) in patients with polyarticular juvenile idiopathic arthritis (JIA) and to associate them with disease duration and activity, bone mineral density and use of medications. In a cross-sectional and controlled study, 30 patients with polyarticular JIA were evaluated and compared to 30 healthy individuals matched for age and gender. Clinical status, anthropometry, laboratory markers in both patients and controls, and bone mineral density, only in the patients, were measured. Of the 30 patients included in the study, 23 (76.7%) were female and 16 (53.3%) non-Caucasian; mean age was 14 years (range = 4 to 20 years). Mean disease duration was 5 years (range = 1 to 12 years). The mean concentrations of serum albumin-corrected calcium (9.04 ± 0.41 mg/dL) and alkaline phosphatase (153.3 ± 100.1 IU) were significantly lower in patients with JIA than in controls (P < 0.0001 and P = 0.001, respectively). No differences in 25(OH)D, PTH or serum phosphorus were observed between JIA and control subjects. Regarding 25(OH)D concentration, 8 patients (26.7%) and 5 controls (16.7%) had 25(OH)D concentrations compatible with deficiency (lower than 20 ng/mL) and 14 patients (46.7%) and 18 controls (60%) had concentrations compatible with insufficiency (20-32 ng/mL). These values were not associated with disease activity, use of medications or bone mineral density. We observed a high frequency of 25(OH)D insufficiency and deficiency in the study sample. The compromised bone metabolism emphasizes the importance of follow-up of JIA patients.
Brazilian journal of medical and biological research = Revista brasileira de pesquisas medicas e biologicas / Sociedade Brasileira de Biofisica ... [et al.] 01/2013; · 1.08 Impact Factor
[Show abstract][Hide abstract] 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 01/2013; 11(1):198. · 2.27 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: In this study, we measured the health-related quality of life (HRQOL) and fatigue of the children of health professionals, aged between two and eleven years, and assessed the daytime and sleep habits of these children and their parents. The study included children from a public school. Data regarding demographics and daily habits were collected. The HRQOL, sleep habits and fatigue were measured using questionnaires. A total of 249 parents participated - 63.5% reported getting an adequate amount of sleep, while 47.4% woke up feeling tired. The children's mean age was 5.6 years - 62.2% watched television in their rooms, 50% used the computer (> 4 hours/day) and 27.8% engaged in extracurricular physical exercise. The sleep score was 45.8 ± 12.2. The HRQOL scores were higher in the physical and lower in the emotional aspects. We found that poorer sleep on the part of both children and parents may be related to the children's lower HRQOL. We conclude that the inadequate habits of parents as well as children, are related to a decrease in HRQOL, particularly regarding the emotional aspect.
Revista da Escola de Enfermagem da U S P 12/2012; 46(6):1313-1319. · 0.39 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: To assess eating disorders, nutritional status, body composition, and food intake in adolescents presenting with fibromyalgia.
In a cross-sectional study, we evaluated the nutritional status (z score of body mass index [ZBMI]), waist circumference, body fat percentage by bioelectrical impedance analysis, symptoms of disordered eating, and possible eating disorders (Kids' Eating Disorders Survey [KEDS]) of 23 female adolescents with fibromyalgia and 23 matched healthy control subjects.
Median age for both groups was 15 years. In the fibromyalgia group, the median time for diagnosis was 13.5 months. We did not observe a statistically significant difference between the control and fibromyalgia groups in relation to ZBMI, fat mass percentage, food intake, and symptoms of disordered eating (KEDS). In the fibromyalgia group, there was a significant correlation between fat mass percentage and the total KEDS score (r = .587, p = .003); the same correlation was observed for ZBMI (r = .0778, p < .001).
This study verified an absence of nutritional and eating disorders in adolescents recently diagnosed with fibromyalgia that, in addition to the correlation between adiposity indexes and KEDS total score, emphasizes the importance of nutritional and body composition assessment, allowing an early and adequate nutritional intervention.
Journal of Adolescent Health 11/2012; 51(5):524-7. · 2.97 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: There are several factors that contribute to poor adherence to treatment in children and adolescents with chronic rheumatic diseases, worsening their quality of life and prognosis. Our aim was to assess the rates of adherence to treatment and to identify the socioeconomic and clinical factors associated.
The sample included 99 patients with juvenile idiopathic arthritis, systemic erythematosus lupus, dermatomyositis or juvenile scleroderma. All patients were followed at the outpatient pediatric rheumatology for a minimum period of 6 months. To assess adherence, a questionnaire was administered to the providers, which included three blocks: 1) demographic, clinical and laboratory data; 2) medication adherence; and 3) attending follow-up appointments, examinations and use of orthoses. A value lower than or equal to 80% of the prescribed was considered poor adherence.
A total of 53% of patients showed good overall adherence, observed when the caregiver lived in a stable union marital status (p = 0.006); 20 patients (20.2%) presented poor medication adherence, related to the use of three or more medications daily (p = 0.047). The causes of poor adherence were forgetfulness, refusal, incorrect dose or lack of medication, personal problems, and financial difficulties.
We observed good overall treatment adherence in patients whose providers lived in stable union and poor adherence to medication in patients who used more than three types of medication daily. There was no association between the adherence rates and sex, age, time since diagnosis and disease activity.
Jornal de pediatria 11/2012; 88(6):483-8. · 1.07 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: OBJECTIVE: To assess nailfold capillaroscopy in children and adolescents with autoimmune rheumatic diseases (juvenile idiopathic arthritis, systemic lupus erythematosus, juvenile dermatomyositis, scleroderma and mixed connective tissue disease) and relate it to clinical and laboratory findings and disease activity. METHODS: Cross-sectional study assessing 147 patients by use of nailfold capillaroscopy as follows: 60 with juvenile idiopathic arthritis; 30 with systemic lupus erythematosus; 30 with juvenile dermatomyositis; 20 with localized scleroderma; four with systemic sclerosis; and three with mixed connective tissue disease. Clinical and laboratory tests and nailfold capillaroscopy were performed in all patients. The nailfold capillaroscopy was performed with an optical microscope (at 10- and 16-time magnifications) by the same observer. RESULTS: Most patients (76.2%) had normal nailfold capillaroscopy. The major changes in nailfold capillaroscopy, characterizing the scleroderma pattern, were observed in patients with juvenile dermatomyositis, systemic scleroderma and mixed connective tissue disease. There was no association between nailfold capillaroscopy and disease activity in patients with juvenile idiopathic arthritis, systemic lupus erythematosus and localized scleroderma. Disease activity and capillaroscopy were associated in patients with juvenile dermatomyositis. CONCLUSION: Nailfold capillaroscopy is a useful method to diagnose autoimmune rheumatic diseases and monitor disease activity.
Revista Brasileira de Reumatologia 10/2012; 52(5):727-732. · 0.86 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The aims of this study were to measure levels of sleep, stress, and depression, as well as health-related quality of life, and to assess the neurocognitive profiles in a sample of adolescents with idiopathic musculoskeletal pain.
Nineteen adolescents with idiopathic musculoskeletal pain and 20 age-matched healthy control subjects were evaluated regarding their levels of sleep and stress, as well as quality of life, and underwent neurocognitive testing.
The sample groups consisted predominantly of females (84%), and the socioeconomic status did not differ between the two groups. In addition, the occurrence of depressive symptoms was similar between the two groups; specifically, 26% of the idiopathic musculoskeletal pain patients and 30% of the control subjects had scores indicative of depression. Teenagers in the group with idiopathic musculoskeletal pain reported poorer quality of life and sleep scores than those in the control group. Regarding stress, patients had worse scores than the control group; whereas 79% of the adolescents with idiopathic musculoskeletal pain met the criteria for a diagnosis of stress, only 35% of the adolescents in the control group met the criteria. In both groups, we observed scores that classified adolescents as being in the resistance phase (intermediate) and exhaustion phase (pathological) of distress. However, the idiopathic musculoskeletal pain group more frequently reported symptomatic complaints of physical and emotional distress. The neurocognitive assessment showed no significant impairments in either group.
Adolescents with idiopathic musculoskeletal pain did not exhibit cognitive impairments. However, adolescents with idiopathic musculoskeletal pain did experience intermediate to advanced psychological distress and lower health-related quality of life, which may increase their risk of cognitive dysfunction in the future.
Clinics (São Paulo, Brazil) 10/2012; 67(10):1139-44. · 1.59 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: To assess the frequency of calcinosis in patients with juvenile dermatomyositis, and the possible risk factors for that manifestation.
Medical record review of 34 patients, with an emphasis on the following characteristics: demographic, clinical and laboratory data; type of treatment; adherence to treatment; disease course (monocyclic, chronic and polycyclic); and disease severity. Patients were divided into two groups as follows: those who developed calcinosis (up to the sixth month of follow-up and after six months of follow-up) and those who did not develop calcinosis. Twenty-seven patients underwent two nailfold capillaroscopies (NFC), which were considered altered when the scleroderma pattern was found.
The mean age of symptom onset of the 34 patients was 6.5 years, the time until diagnosis was 1.2 years, and 70% were females. Half of the patients had a monocyclic disease course, and only 14.7% had severe vasculitis. Almost 90% of the patients undergoing NFC showed a change on the first assessment, 74% showed a change on the second assessment, and the mean interval between both assessments was 1.6 year. Calcinosis was evidenced in 16 (47.1%) patients. No association was observed between the variables analyzed and the development of calcinosis.
No risk factors for calcinosis were identified in this study, although that complication was found in half of the patients with juvenile dermatomyositis studied.
Revista Brasileira de Reumatologia 08/2012; 52(4):549-53. · 0.86 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: OBJECTIVES: Juvenile idiopathic arthritis (JIA) is a chronic inflammatory joint disease affecting children. Even if remission is successfully induced, about half of the patients experience a relapse after stopping anti-inflammatory therapy. The present study investigated whether patients with JIA at risk of relapse can be identified by biomarkers even if clinical signs of disease activity are absent. METHODS: Patients fulfilling the criteria of inactive disease on medication were included at the time when all medication was withdrawn. The phagocyte activation markers S100A12 and myeloid-related proteins 8/14 (MRP8/14) were compared as well as the acute phase reactant high-sensitivity C reactive protein (hsCRP) as predictive biomarkers for the risk of a flare within a time frame of 6 months. RESULTS: 35 of 188 enrolled patients experienced a flare within 6 months. Clinical or standard laboratory parameters could not differentiate between patients at risk of relapse and those not at risk. S100A12 and MRP8/14 levels were significantly higher in patients who subsequently developed flares than in patients with stable remission. The best single biomarker for the prediction of flare was S100A12 (HR 2.81). The predictive performance may be improved if a combination with hsCRP is used. CONCLUSIONS: Subclinical disease activity may result in unstable remission (ie, a status of clinical but not immunological remission). Biomarkers such as S100A12 and MRP8/14 inform about the activation status of innate immunity at the molecular level and thereby identify patients with unstable remission and an increased risk of relapse.
Annals of the rheumatic diseases 06/2012; · 8.11 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: To evaluate the prevalence of genetic defects in clinically suspected autoinflammatory syndromes (AIS) in a Brazilian multicenter study.
The study included 102 patients with a clinical diagnosis of Cryopyrin Associated Periodic Syndromes (CAPS), TNF Receptor Associated Periodic Syndrome (TRAPS), Familial Mediterranean Fever (FMF), Mevalonate Kinase Deficiency (MKD) and Pediatric Granulomatous Arthritis (PGA). One of the five AIS-related genes (NLRP3, TNFRSF1A, MEFV, MVK and NOD2) was evaluated in each patient by direct DNA sequencing, based on the most probable clinical suspect.
Clinical diagnoses of the 102 patients were: CAPS (n = 28), TRAPS (n = 31), FMF (n = 17), MKD (n = 17) and PGA (n = 9). Of them, 27/102 (26 %) had a confirmed genetic diagnosis: 6/28 (21 %) CAPS patients, 7/31 (23 %) TRAPS, 3/17 (18 %) FMF, 3/17 (18 %) MKD and 8/9 (89 %) PGA.
We have found that approximately one third of the Brazilian patients with a clinical suspicion of AIS have a confirmed genetic diagnosis.
Journal of Clinical Immunology 05/2012; 32(5):922-32. · 3.38 Impact Factor