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1.
Lupus ; 32(6): 781-790, 2023 May.
Article in English | MEDLINE | ID: mdl-37070932

ABSTRACT

OBJECTIVES: To identify associations between mortality in cSLE patients and their characteristics: clinical and laboratory features, disease activity and damage scores, and treatment; to evaluate risk factors associated with mortality in cSLE; and to determine the most frequent causes of death in this group of patients. METHODS: We performed a multicenter retrospective cohort using data from 1,528 cSLE patients followed in 27 pediatric rheumatology tertiary centers in Brazil. Patients' medical records were reviewed according to a standardized protocol, in which information regarding demographic and clinical features, disease activity and damage scores, and treatment were collected and compared between deceased cSLE patients and survivors. Univariate and multivariate analyses by Cox regression model were used to calculate risk factors for mortality, whereas survival rates were analyzed by Kaplan-Meier plots. RESULTS: A total of 63/1,528 (4.1%) patients deceased, 53/63 were female (84.1%), median age at death was 11.9 (9.4-13.1) years and median time interval between cSLE diagnosis and death was 3.2 (0.5-5.3) years. Sepsis was the main cause of death in 27/63 (42.8%) patients, followed by opportunistic infections in 7/63 (11.1%), and alveolar hemorrhage in 6/63 (9.5%) patients. The regression models resulted in neuropsychiatric lupus (NP-SLE) (HR = 2.56, 95% CI = 1.48-4.42) and chronic kidney disease (CKD) (HR = 4.33, 95% CI = 2.33-4.72), as risk factors significantly associated with mortality. Overall patient survival after cSLE diagnosis at 5, 10, and 15 years were 97%, 95.4%, and 93.8%, respectively. CONCLUSIONS: This study confirmed that the recent mortality rate in cSLE in Brazil is low, but still of concern. NP-SLE and CKD were the main risk factors for mortality, indicating that the magnitude of these manifestations was significantly high.


Subject(s)
Lupus Erythematosus, Systemic , Renal Insufficiency, Chronic , Child , Humans , Female , Male , Lupus Erythematosus, Systemic/complications , Brazil/epidemiology , Retrospective Studies , Age of Onset , Risk Factors , Renal Insufficiency, Chronic/complications
2.
Pediatr Nephrol ; 38(6): 1843-1854, 2023 06.
Article in English | MEDLINE | ID: mdl-36409367

ABSTRACT

BACKGROUND: Lupus nephritis (LN) is a frequent manifestation of childhood-onset systemic lupus erythematosus (cSLE) with a potential risk for kidney failure and poor outcomes. This study aimed to evaluate stages III, IV, and V of chronic kidney disease (CKD) and investigate risk factors for CKD in cSLE patients. METHODS: We performed a nationwide observational cohort study in 27 pediatric rheumatology centers, including medical charts of 1528 cSLE patients. Data were collected at cSLE diagnosis, during follow-up, and at last visit or death, between September 2016 and May 2019. RESULTS: Of 1077 patients with LN, 59 (5.4%) presented with CKD, 36/59 (61%) needed dialysis, and 7/59 (11.8%) were submitted for kidney transplantation. After Bonferroni's correction for multiple comparisons (p < 0.0013), determinants associated with CKD were higher age at last visit, urinary biomarker abnormalities, neuropsychiatric involvement, higher scores of disease activity at last visit and damage index, and more frequent use of methylprednisolone, cyclosporine, cyclophosphamide, and rituximab. In the regression model analysis, arterial hypertension (HR = 15.42, 95% CI = 6.12-38.83, p ≤ 0.001) and biopsy-proven proliferative nephritis (HR = 2.83, 95%CI = 1.70-4.72, p ≤ 0.001) increased the risk of CKD, while children using antimalarials had 71.0% lower CKD risk ((1.00-0.29) × 100%) than children not using them. The Kaplan-Meier comparison showed lower survival in cSLE patients with biopsy-proven proliferative nephritis (p = 0.02) and CKD (p ≤ 0.001). CONCLUSIONS: A small number of patients manifested CKD; however, frequencies of dialysis and kidney transplantation were relevant. This study reveals that patients with cSLE with hypertension, proliferative nephritis, and absence of use of antimalarials exhibited higher hazard rates of progression to CKD. A higher resolution version of the Graphical abstract is available as Supplementary information.


Subject(s)
Antimalarials , Hypertension , Lupus Erythematosus, Systemic , Lupus Nephritis , Renal Insufficiency, Chronic , Child , Humans , Antimalarials/therapeutic use , Retrospective Studies , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/drug therapy , Lupus Erythematosus, Systemic/epidemiology , Lupus Nephritis/complications , Lupus Nephritis/drug therapy , Lupus Nephritis/epidemiology , Hypertension/complications , Renal Insufficiency, Chronic/epidemiology , Renal Insufficiency, Chronic/etiology , Renal Insufficiency, Chronic/therapy , Age of Onset
3.
Lupus ; 30(14): 2286-2291, 2021 Dec.
Article in English | MEDLINE | ID: mdl-34689652

ABSTRACT

OBJECTIVE: To evaluate if the 2019-European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) criteria at diagnosis of childhood-onset systemic lupus erythematosus (cSLE) are associated with higher rates of early damage scored by Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index (SDI). METHODS: This retrospective multicenter study included 670 cSLE patients with ≤5 years of disease duration. All patients fulfilled both 2019-EULAR/ACR and 1997-ACR classification criteria. Total score of 2019-EULAR/ACR criteria and each of its specific domains were assessed at diagnosis as predictors of damage accrual at the last visit, according to the presence of any organ damage (defined by SDI ≥ 1). RESULTS: Median disease duration was 2.8 (IQR 1.8-3.8) years and 200 (29.9%) patients had at least one organ damage (SDI ≥ 1). The most frequent domains were neuropsychiatric (12%), renal (7%), and musculoskeletal (6%). There was a higher frequency of renal (58% vs 43%, p = 0.0004) and neuropsychiatric domain (21% vs 7%, p < 0.0001) of 2019-EULAR/ACR criteria in patients with damage (SDI ≥ 1) compared to those without damage (SDI = 0). Patients scoring renal or neuropsychiatric domains of the 2019-EULAR/ACR criteria at diagnosis were associated with renal damage (odds ratio 9.701, 95% confidence interval 3.773-24.941, p < 0.001) or neuropsychiatric damage (OR 9.480, 95% CI 5.481-16.399, p<0.0001) at latest visit, respectively. cSLE patients with positive anti-dsDNA at diagnosis were also associated with renal damage by the latest visit (OR 2.438, 95% CI 1.114-5.3381, p = 0.021). Constitutional, hematologic, mucocutaneous, serosal, and musculoskeletal domains and specific criteria as well as other immunologic criteria were not associated with damage accrual. Median of SLEDAI-2K was significantly higher in patients with global damage (19.5 (2-51) vs 14 (0-51), p<0.001). 2019-EULAR/ACR score >25 was associated with more overall (SDI ≥ 1) (38% vs 25%, p = 0.0002) and renal damage (11% vs 5%, p = 0.023). CONCLUSIONS: The 2019-EULAR/ACR criteria at diagnosis were associated with a higher rate of early damage in cSLE patients, especially for renal and neuropsychiatric damage. Of note, damage was particularly associated with high disease activity at diagnosis and 2019-EULAR/ACR score >25.


Subject(s)
Lupus Erythematosus, Systemic , Rheumatic Diseases , Rheumatology , DNA , Humans , Lupus Erythematosus, Systemic/diagnosis , Retrospective Studies , Severity of Illness Index
4.
J Periodontal Res ; 56(6): 1132-1140, 2021 Dec.
Article in English | MEDLINE | ID: mdl-34510434

ABSTRACT

OBJECTIVE: To evaluate how chronic gingivitis treatment impacts the oral and circulating cytokine expressions after six-month follow-up in patients with juvenile systemic lupus erythematosus (jSLE) and also to evaluate the circulating expression of anti-Porphyromonas gingivalis peptidylarginine deiminase antibodies (anti-PPAD) before and after treatment. BACKGROUND: Juvenile systemic lupus erythematosus patients present a worse periodontal condition associated with higher gingival crevicular fluid (GCF) levels of interleukin (IL)-1ß, IL-8, granulocyte colony-stimulating factor (G-CSF), interferon-γ and monocyte chemoattractant protein (MCP)-1. MATERIALS AND METHODS: Twenty-one adolescents with jSLE (mean age: 16.2 ± 1.5 years) were recruited. Participants were rheumatologically and periodontally examined. All individuals were clinically diagnosed with gingival inflammation. Chronic gingivitis treatment consisted of supragingival scaling, prophylaxis and oral hygiene instructions. The cytokine levels were determined by bead-based multiplex assays and the anti-PPAD levels by ELISA. Gingival crevicular fluid (GCF) and serum samples were collected at baseline and 6 months after treatment. RESULTS: We observed a reduction in attachment loss, SLE Disease Activity Index (SLEDAI), IL-1ß, IL-10 and MCP-1 GCF levels, and the IL-4 and IL-5 serum levels 6 months after periodontal treatment. On the contrary, a significant increase in GCF expression of IL-4, IL-12, IL-17, IFN-γ and serum levels of anti-PPAD antibody was observed. CONCLUSION: Juvenile systemic lupus erythematosus patients seem to positively benefit from periodontal treatment by a significantly reduced CAL, a GCF reduction of pro-inflammatory cytokines and an increasing of anti-inflammatory ones. However, an increase in the GCF expression of IL-17 and the serum expression of anti-PPAD antibody 6 months after periodontal treatment might negatively affect the treatment outcome of such patients in the long term.


Subject(s)
Gingivitis , Lupus Erythematosus, Systemic , Adolescent , Cytokines/analysis , Follow-Up Studies , Gingival Crevicular Fluid/chemistry , Gingivitis/therapy , Humans , Interleukin-12 , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/therapy
5.
Autoimmun Rev ; 19(12): 102693, 2020 Dec.
Article in English | MEDLINE | ID: mdl-33164791

ABSTRACT

OBJECTIVE: To assess childhood-onset systemic lupus erythematosus-related antiphospholipid syndrome(cSLE-APS) in a large Brazilian population. METHODS: A retrospective observational cohort study was carried-out in 27 Pediatric Rheumatology university centers, including 1519 cSLE patients. RESULTS: cSLE-APS was observed in 67/1519 (4%) and was diagnosed at disease onset in 39/67 (58%). The median disease duration was 4.9 (0-17) years. Thrombosis recurrences were evidenced in 18/67 (27%) cSLE-APS patients. The most frequent thrombosis sites in cSLE-APS patients were: venous thrombosis in 40/67 (60%), especially deep vein thrombosis in 29/40 (72%); arterial thrombosis in 35/67 (52%), particularly stroke; small vessels thrombosis in 9/67 (13%) and mixed thrombosis in 3/67 (4%). Pregnancy morbidity was observed in 1/67 (1%). Non-thrombotic manifestation associated to cSLE-APS occurred in 21/67 (31%), mainly livedo reticularis in 14/67 (21%), valvar thickening in 4/67 (6%) and valvar vegetations not related to infections in 2/67 (3%). None of them had catastrophic APS. Further analysis demonstrated that the median of SLICC/ACR-DI [1(0-5) vs. 0(0-7),p < 0.0001] was significantly higher in cSLE-APS patients compared to cSLE without APS. The frequencies of cerebrovascular disease (40% vs. 1%,p < 0.0001), polyneuropathy (9% vs. 1%,p < 0.0001), SLICC/ACR-DI ≥1 (57% vs. 27%, p < 0.0001) and intravenous cyclophosphamide use (59% vs. 37%, p < 0.0001) were significantly higher in the former group. CONCLUSIONS: Our large multicenter study demonstrated that cSLE-APS was a rare condition, occurring during disease course with a high accrual damage. Central and peripheral neuropsychiatric involvements were distinctive features of this autoimmune thrombosis.


Subject(s)
Antiphospholipid Syndrome , Lupus Erythematosus, Systemic , Pregnancy Complications , Adult , Age of Onset , Antiphospholipid Syndrome/complications , Antiphospholipid Syndrome/diagnosis , Antiphospholipid Syndrome/epidemiology , Brazil/epidemiology , Child , Cohort Studies , Female , Humans , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/epidemiology , Morbidity , Pregnancy , Retrospective Studies
6.
Clin Rheumatol ; 38(10): 2857-2863, 2019 Oct.
Article in English | MEDLINE | ID: mdl-31209708

ABSTRACT

OBJECTIVE: To evaluate the influence of ethnicity in presentation of childhood-onset systemic lupus erythematosus (cSLE) patients. METHODS: This multicenter study included cSLE patients (American College of Rheumatology criteria) followed in 27 Pediatric Rheumatology services of Brazil. Ethnicities were classified in four groups according to the parents' and all four grandparents' self-reported ethnicity. The statistical analysis was performed using the Bonferroni's correction (p < 0.0027). RESULTS: According to ethnic groups, 1537 cSLE patients were classified in Caucasian (n = 786), African-Latin American (n = 526), Asian (n = 8), and others/unknown (n = 217). Comparisons between 1312 African-Latin American and Caucasian revealed similar median age at cSLE diagnosis [12.2(2.6-18) vs. 12.1(0.3-18) years, p = 0.234], time interval to diagnosis [0.25(0-12) vs. 0.3(0-10) years, p = 0.034], and SLEDAI-2K score [14(0-55) vs. 14(0-63), p = 0.781] in both groups. The mean number of diagnostic criteria according to SLICC (6.47 ± 1.911 vs. 5.81 ± 1.631, p < 0.0001) and frequencies of maculopapular lupus rash (8% vs. 3%, p < 0.0001), palate oral ulcers (17% vs. 11%, p = 0.001), tongue oral ulcers (4% vs. 1%, p = 0.001), and nonscarring alopecia (29% vs. 16%, p < 0.0001) were significantly higher in African-Latin American, whereas malar rash (45% vs. 58%, p < 0.0001) was more frequent in Caucasian. The presence of anti-phospholipid antibody (23% vs. 12%, p < 0.0001), low complement levels (58% vs. 41%, p < 0.0001), and isolated direct Coombs test (10% vs. 5%, p = 0.001) was also significantly higher in the former group. CONCLUSIONS: Our study demonstrated that disease presentation severity of African-Latin American cSLE patients is comparable with Caucasian. Mucocutaneous manifestations and autoantibodies profile were the only distinctive features of the former group. The unique mixed background of Brazilian patients probably minimized race diversity spectrum of these patients. Key Points • Our study demonstrated that disease presentation severity of African-Latin American cSLE patients is comparable with Caucasian. • Mucocutaneous manifestations and autoantibodies profile were the only distinctive features of African-Latin American cSLE patients. • African-Latin American cSLE patients had more often anti-phospholipid antibodies and hypocomplementemia. • The unique mixed background of Brazilian patients probably minimized race diversity spectrum of these patients.


Subject(s)
Antibodies, Antiphospholipid/immunology , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/ethnology , Adolescent , Age of Onset , American Indian or Alaska Native , Asian People , Black People , Brazil/epidemiology , Brazil/ethnology , Child , Child, Preschool , Ethnicity , Female , Humans , Infant , Lupus Erythematosus, Systemic/immunology , Male , Retrospective Studies , Severity of Illness Index , White People
7.
Rheumatol Int ; 33(7): 1873-8, 2013 Jul.
Article in English | MEDLINE | ID: mdl-22210275

ABSTRACT

Relapsing polychondritis (RP) is a rare autoimmune systemic disease, especially in childhood. To report three new pediatric RP cases, to provide a literature review and to compare with adulthood disease, retrospective data collection from three childhood RP cases was observed in a Brazilian Pediatric Rheumatology Division. A literature review based on a MEDLINE database search was performed. Arthritis and auricular chondritis were present in our three patients. Two cases presented with early and severe laryngotracheal chondritis, besides initial and symptomatic costochondritis. The other case developed prominent epiphyseal plate involvement. Two patients were refractory to corticosteroids and immunosuppressants and required the use of TNF-alpha inhibitors to improve the symptoms, while corticosteroids plus methotrexate induced remission in the other patient. The literature review showed 44 cases of pediatric-onset disease in English language. Arthritis and ear chondritis are the most common initial and cumulative manifestations of RP in children and adults. Nasal and laryngotracheobronchial chondritis are also common manifestations observed during follow-up in childhood. There is also an early severity of respiratory chondritis in childhood, requiring aggressive treatment with corticosteroids, immunosuppressants and biologic agents. The data presented by those 3 children, considered in conjunction with the data from the 44 published cases, may reflect some distinguishing childhood RP features, such as more severe and frequent respiratory tract involvement, symptomatic costochondritis and the atypical pattern of persistent and destructive arthritis with epiphyseal plate involvement. Response to immunosuppressants and biologic agents is anecdotal, but steroids remain the main drug during the flares.


Subject(s)
Polychondritis, Relapsing , Adolescent , Adrenal Cortex Hormones/therapeutic use , Adult , Age of Onset , Biopsy , Brazil , Child , Child, Preschool , Drug Resistance , Drug Substitution , Drug Therapy, Combination , Female , Humans , Immunosuppressive Agents/therapeutic use , Male , Middle Aged , Polychondritis, Relapsing/complications , Polychondritis, Relapsing/diagnosis , Polychondritis, Relapsing/drug therapy , Remission Induction , Retrospective Studies , Severity of Illness Index , Tomography, X-Ray Computed , Treatment Outcome
8.
Rheumatol Int ; 31(8): 1037-43, 2011 Aug.
Article in English | MEDLINE | ID: mdl-20306266

ABSTRACT

To identify the underlying mechanism of amenorrhea in juvenile systemic lupus erythematosus (JSLE) patients, thirty-five (11.7%) JSLE patients with current or previous amenorrhea were consecutively selected among the 298 post-menarche patients followed in 12 Brazilian pediatric rheumatology centers. Pituitary gonadotrophins [follicle-stimulating hormone (FSH) and luteinizing hormone (LH)] and estradiol were evaluated in 32/35 patients, and prolactin and total testosterone in 29/35 patients. Patient's medical records were carefully reviewed according to demographic, clinical and therapeutic findings. The mean duration of amenorrhea was 7.2 ± 3.6 months. Low FSH or LH was observed in 7/32 (22%) JSLE patients and normal FSH or LH in 25 (78%). Remarkably, low levels of FSH or LH were associated with higher frequency of current amenorrhea (57% vs. 0%, P = 0.001), higher median disease activity (SLEDAI) and damage (SLICC/ACR-DI) (18 vs. 4, P = 0.011; 2 vs. 0, P = 0.037, respectively) and higher median current dose of prednisone (60 vs. 10 mg/day, P = 0.0001) compared to normal FSH or LH JSLE patients. None of them had decreased ovarian reserve and premature ovarian failure. Six of 29 (21%) patients had high levels of prolactin, and none had current amenorrhea. No correlations were observed between levels of prolactin and SLEDAI, and levels of prolactin and SLICC/ACR-DI scores (Spearman's coefficient). We have identified that amenorrhea in JSLE is associated with high dose of corticosteroids indicated for active disease due to hypothalamic-pituitary-ovary axis suppression.


Subject(s)
Amenorrhea/blood , Hormones/blood , Lupus Erythematosus, Systemic/blood , Adolescent , Amenorrhea/diagnosis , Child , Child, Preschool , Estradiol/blood , Female , Follicle Stimulating Hormone/blood , Glucocorticoids/therapeutic use , Humans , Lupus Erythematosus, Systemic/drug therapy , Lupus Erythematosus, Systemic/pathology , Luteinizing Hormone/blood , Menarche , Prednisone/therapeutic use , Retrospective Studies , Testosterone/blood , Young Adult
9.
J Pediatr (Rio J) ; 85(3): 183-93, 2009.
Article in English, Portuguese | MEDLINE | ID: mdl-19492166

ABSTRACT

OBJECTIVES: To present an updated review concerning new assays for diagnosing tuberculosis based on in vitro interferon-gamma production by host T cells, and compare them with tuberculin skin test. METHODS: A literature review was carried out based on Medline and LILACS databases (2000-2008) searching for the following keywords: tuberculosis, interferon-gamma, quantiFERON, ELISPOT and T-SPOT.TB. RESULTS: These new assays proved to have, in general, equal or superior sensitivity and specificity than the tuberculin skin test not only in adults but also in children and immunosuppressed patients for the diagnosis of both latent tuberculosis infection or active disease, with some advantages such as less cross-reactivity as a result of previous BCG vaccination, less influence of anergy and better accuracy in small children. In the United States, these assays have been used instead of the tuberculin skin test and, although still very expensive, the World Health Organization will be making its economic viability a priority. CONCLUSIONS: Always having in mind the importance of clinical and epidemiological histories, these new assays based on interferon-gamma release present promising results and should be considered in tuberculosis investigation procedures for all patients, however with a special concern in the risk groups (i.e., children and immunosuppressed patients).


Subject(s)
Enzyme-Linked Immunosorbent Assay/methods , Interferon-gamma/biosynthesis , T-Lymphocytes/immunology , Tuberculosis/diagnosis , Child , Humans , Interferon-gamma/blood , Sensitivity and Specificity , Tuberculin Test/methods
10.
J. pediatr. (Rio J.) ; J. pediatr. (Rio J.);85(3): 183-193, maio-jun. 2009. tab
Article in English, Portuguese | LILACS | ID: lil-517865

ABSTRACT

OBJETIVOS: Apresentar uma revisão atualizada sobre os novos métodos para o diagnóstico da tuberculose baseados na produção in vitro de interferon-gama (IFN-γ) por células T dos pacientes sob investigação, comparando-os com a tradicional prova tuberculínica. FONTES DOS DADOS: Revisão de literatura utilizando os bancos de dados MEDLINE e LILACS (2000-2008) utilizando as palavras-chave tuberculose, interferon-gama, quantiFERON, ELISPOT e T-SPOT.TB. SÍNTESE DOS DADOS: Esses novos testes mostraram-se, de um modo geral, tão ou mais sensíveis e específicos que a prova tuberculínica, tanto em adultos como em crianças e imunossuprimidos, para o diagnóstico da infecção latente e da doença ativa, apresentando vantagens como a menor interferência da vacinação prévia pelo BCG, menor influência de estados anérgicos e melhor acurácia em crianças menores. Nos Estados Unidos, já estão sendo utilizados em substituição à prova tuberculínica, e apesar dos custos ainda elevados, a Organização Mundial de Saúde vai priorizar a sua viabilidade econômica. CONCLUSÕES: Sempre levando em conta a importância da história clínica e epidemiológica, os novos testes baseados na produção de IFN-γ apresentam resultados promissores e deverão ser considerados na investigação de tuberculose em qualquer paciente, mas especialmente nos grupos de risco, como as crianças e os imunossuprimidos.


OBJECTIVES: To present an updated review concerning new assays for diagnosing tuberculosis based on in vitro interferon-gamma production by host T cells, and compare them with tuberculin skin test. SOURCES: A literature review was carried out based on Medline and LILACS databases (2000-2008) searching for the following keywords: tuberculosis, interferon-gamma, quantiFERON, ELISPOT and T-SPOT.TB. SUMMARY OF THE FINDINGS: These new assays proved to have, in general, equal or superior sensitivity and specificity than the tuberculin skin test not only in adults but also in children and immunosuppressed patients for the diagnosis of both latent tuberculosis infection or active disease, with some advantages such as less cross-reactivity as a result of previous BCG vaccination, less influence of anergy and better accuracy in small children. In the United States, these assays have been used instead of the tuberculin skin test and, although still very expensive, the World Health Organization will be making its economic viability a priority. CONCLUSIONS: Always having in mind the importance of clinical and epidemiological histories, these new assays based on interferon-gamma release present promising results and should be considered in tuberculosis investigation procedures for all patients, however with a special concern in the risk groups (i.e., children and immunosuppressed patients).


Subject(s)
Child , Humans , Enzyme-Linked Immunosorbent Assay/methods , Interferon-gamma/biosynthesis , T-Lymphocytes/immunology , Tuberculosis/diagnosis , Interferon-gamma/blood , Sensitivity and Specificity , Tuberculin Test/methods
11.
Oral Health Prev Dent ; 6(1): 75-81, 2008.
Article in English | MEDLINE | ID: mdl-18399311

ABSTRACT

PURPOSE: Our aim was to evaluate the expression of interleukin-18 (IL-18), interleukin-l-beta (IL-1beta) and the amount of elastase activity in gingival crevicular fluid (GCF) from inflamed gingival sites in patients with juvenile systemic lupus erythematosus (JSLE), and compare these to the expression in GCF from inflamed sites in generally healthy controls. In addition, the local inflammation in periodontal tissues was related to systemic inflammation by the assessment of IL-18 levels in plasma. MATERIALS AND METHODS: GCF from 16 patients with JSLE and 14 controls were collected using a washing device. Elastase activity was measured with a specific substrate, and IL-18 and 11-1 were measured by ELISA. RESULTS: The percentage of visible plaque index, gingival bleeding index and attachment level were similar in JSLE and controls, while the percentage of probing depth greater or equal to 3 mm was significantly higher in the controls. The total amount of IL-1beta and IL-18 in GCF were significantly decreased in JSLE, while the total amount and the percentage of free elastase activity were significantly higher in JSLE when compared with the controls. The plasma levels of I1-18 and the erythrocyte sedimentation rate were significantly higher in JSLE patients. CONCLUSION: We found more active elastase in GCF from inflamed sites in JSLE patients even in the presence of significantly lower levels of IL-18 and IL-13. The increased elastase activity suggests a hyperactivity of neutrophils in JSLE, possibly generated by a priming effect caused by the higher plasma levels of IL-18 found in these JSLE patients.


Subject(s)
Gingival Crevicular Fluid/chemistry , Gingivitis/metabolism , Interleukin-18/biosynthesis , Leukocyte Elastase/metabolism , Lupus Erythematosus, Systemic/metabolism , Adolescent , Case-Control Studies , Female , Gingivitis/complications , Humans , Interleukin-18/analysis , Interleukin-18/blood , Interleukin-1beta/analysis , Interleukin-1beta/biosynthesis , Lupus Erythematosus, Systemic/complications , Male , Statistics, Nonparametric
12.
Rev. bras. reumatol ; Rev. bras. reumatol;42(2): 85-90, mar.-abr. 2002. tab, graf
Article in Portuguese | LILACS | ID: lil-413698

ABSTRACT

Objetivo: estudar as características clínicas e demográficas de crianças e adolescentes brasileiros com lúpus eritematoso sistêmico juvenil (LESj), seguidos em centros de referência em Reumatologia Pediátrica. Métodos: o estudo foi multicêntrico retrospectivo (análise de prontuários) e envolveu 12 centros de São Paulo, Rio de Janeiro, Goiás, Rio Grande do Norte e Rio Grande do Sul. Foram analisados 280 prontuários de crianças e adolescentes com LESj dos quais 234 eram do sexo feminino (83,6%). A idade de início variou de 1 a 16 anos, com média de 11,4 anos. Foram analisados os dados clínicos e laboratoriais iniciais e de seguimento. Para o cálculo da taxa de mortalidade foram excluídos os pacientes que abandonaram o seguimento. Resultados: a presença de anticorpos antinucleares (AAN) foi descrita em 259 pacientes (93%). As manifestações iniciais mais freqüentes foram: artrite (73%), citopenia (67%), presença de anti-DNA (59%), fotossensibilidade (50%) e nefrite (45%). O comprometimento do sistema nervoso central foi observado em 15% dos pacientes, sendo que destes 50% apresentaram convulsões e 38% psicose lúpica. Vinte e sete pacientes (9,7%) abandonaram o acompanhamento médico. Quinze dos 253 pacientes restantes (5,9% evoluíram para óbito durante o seguimento, sendo as principais causas a infecção (73%) e a falência renal (33%), isoladas ou associadas. A mortalidade foi relativamente maior no sexo masculino (11:210 meninas [5,2%] e 4:43 meninos [9,3%]). Não se observou correlação entre a mortalidade e a idade de início da doença, o tempo de seguimento ou o número de critérios presentes na primeira consulta. Conclusão: as crianças e adolescentes brasileiros com LESj apresentam características clínicas semelhantes às observadas em outras populações. As infecções e a insuficiência renal são as principais causas de óbito.


Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Adolescent , Infections , Lupus Erythematosus, Systemic , Mortality , Multicenter Studies as Topic , Rheumatic Diseases
13.
J. pediatr. (Rio J.) ; J. pediatr. (Rio J.);77(supl.2): S234-S244, nov. 2001. ilus, tab
Article in Portuguese | LILACS | ID: lil-315119

ABSTRACT

Objetivo: apresentar uma revisão sobre as doenças com manifestações músculo-esqueléticas que, mais freqüentemente, levam o adolescente a procurar os serviços médicos, com ênfase nas doenças reumáticas. Com isso, pretendemos oferecer ao médico e à equipe que atende esses adolescentes a possibilidade de pensar em diferentes diagnósticos diferenciais e, daí, traçar o plano diagnóstico, iniciar a abordagem terapêutica e, caso seja necessário, encaminhar para um especialista. método: foram utilizados revisão bibliográfica na base de daos Medline, dados dos serviços, cujos autores participam dessa revisão, e experiência pessoal dos mesmos. Resultados: as manifestações músculo-esqueléticas ocorrem em um número muito grande de enfermidades e constituem motivo freqüente da consulta do adolescente, sendo que as doenças reumáticas constituem cerca de metade dessas doenças. Destas, a mais freqüente em nosso meio é a febre reumática. Na adolescência, são também importantes os pontos de vista de diagnóstico e de tratamento: artrites idiopáticas juvenis, destacando-se a artrite associada à entesite, lúpus eritemetoso sistêmico e vasculites. A fibromialgia, distrofia simpático-reflexa, dores de crescimento, síndrome da hipermobilidade articular e reumatismos psicogênicos constituem condições não-inflamatórias, freqüentemente, simulam doenças reumáticas. Conclusão: condições inflamatórias, e não-inflamatórias, doenças de etiologias diversas como infecciosas, neoplásicas e ortopédicas, dentre outras, constituem causas freqüentes de queixas músculo-esqueléticas, e há necessidade de seu reconhecimento precoce para que o médico que cuida do adolescente possa atuar prontamente, de modoa melhorar o prognóstico de seu paciente


Subject(s)
Humans , Adolescent , Rheumatic Diseases , Rheumatic Fever
14.
J. pediatr. (Rio J.) ; J. pediatr. (Rio J.);75(5): 321-6, set.-out. 1999. ilus
Article in Portuguese | LILACS | ID: lil-251405

ABSTRACT

Objetivo: O acometimento patológico das enteses, que representam o sítio de uniäo de tendöes, ligamentos, fáscias e cápsulas articulares ao osso, pode ser avaliado clinicamente pelo surgimento de dor espotânea ou à digitopressäo em certas localizaçöes. Este artigo de revisäo tem por objetivo chamar a atençäo dos pediatras para a existência de entesopatias ou entesites (acometimento inflamatório dessas estruturas) na faixa etária infanto-juvenil. MétodosO estudo faz uma revisäo de conceitos em relaçäo à estrutura das enteses e dos quadros clínico, radiológico e laboratorial das entosepatias, assim como dos diagnósticos diferenciais e da terapêutica. A partir da revisäo sistemática baseada em pesquisa nos bancos de referências bibliográficas Medline (dados disponíveis a partir de 1966) e Lilacs (dados disponíveis a partir de 1981), bem como em livros-texto de Reumatologia Pediátrica publicados a partir de 1990, foram selecionados artigos e textos pertinentes ao assunto. Resultados: as entesopatias na faixa etária infanto-juvenil localizam-se principalmente em membros inferiores. Parecem estar associadas ao desenvolvimento de espondiliartropatias, ocorrendo, com menor freqüência, em outras doenças inflamatórias ou, mesmo, em algumas condiçöes näo inflamatórias. Conclusäo: a identificaçäo das entesopatias é importante para o diagnóstico precoce de crianças em risco para o desenvolvimento de espondiloartropatias. Assim, elas poderäo ser incluídas, precocemente, em um programa adequado de terapias física e medicamentosa


Subject(s)
Humans , Child , Adolescent , Arthritis, Juvenile , Rheumatic Diseases , Adrenal Cortex Hormones
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