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BACKGROUND: Increased malignancy frequency is well documented in adult-systemic lupus erythematosus (SLE), but with limited reports in childhood-onset SLE (cSLE) series. We explored the frequency of malignancy associated with cSLE, describing clinical and demographic characteristics, disease activity and cumulative damage, by the time of malignancy diagnosis. METHOD: A retrospective case-notes review, in a nationwide cohort from 27 Pediatric Rheumatology centres, with descriptive biopsy-proven malignancy, disease activity/damage accrual, and immunosuppressive treatment were compiled in each participating centre, using a standard protocol. RESULTS: Of the 1757 cSLE cases in the updated cohort, 12 (0.7%) developed malignancy with median time 10 years after cSLE diagnosis. There were 91% females, median age at cSLE diagnosis 12 years, median age at malignancy diagnosis 23 years. Of all diagnosed malignancies, 11 were single-site, and a single case with concomitant multiple sites; four had haematological (0.22%) and 8 solid malignancy (0.45%). Median (min-max) SLEDAI-2 K scores were 9 (0-38), median (min-max) SLICC/ACR-DI (SDI) score were 1 (1-5) Histopathology defined 1 Hodgkin's lymphoma, 2 non-Hodgkin's lymphoma, 1 acute lymphoblastic leukaemia; 4 gastrointestinal carcinoma, 1 squamous cell carcinoma of the tongue and 1 anal carcinoma; 1 had sigmoid adenocarcinoma and 1 stomach carcinoid; 3 had genital malignancy, being 1 vulvae, 1 cervix and 1 vulvae and cervix carcinomas; 1 had central nervous system oligodendroglioma; and 1 testicle germ cell teratoma. CONCLUSION: Estimated malignancy frequency of 0.7% was reported during cSLE follow up in a multicentric series. Median disease activity and cumulative damage scores, by the time of malignancy diagnoses, were high; considering that reported in adult series.
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Carcinoma , Lúpus Eritematoso Sistêmico , Criança , Feminino , Humanos , Masculino , Adulto Jovem , Idade de Início , Carcinoma/complicações , Lúpus Eritematoso Sistêmico/complicações , Estudos RetrospectivosRESUMO
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.
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Lúpus Eritematoso Sistêmico , Insuficiência Renal Crônica , Criança , Humanos , Feminino , Masculino , Lúpus Eritematoso Sistêmico/complicações , Brasil/epidemiologia , Estudos Retrospectivos , Idade de Início , Fatores de Risco , Insuficiência Renal Crônica/complicaçõesRESUMO
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.
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Antimaláricos , Hipertensão , Lúpus Eritematoso Sistêmico , Nefrite Lúpica , Insuficiência Renal Crônica , Criança , Humanos , Antimaláricos/uso terapêutico , Estudos Retrospectivos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/epidemiologia , Nefrite Lúpica/complicações , Nefrite Lúpica/tratamento farmacológico , Nefrite Lúpica/epidemiologia , Hipertensão/complicações , Insuficiência Renal Crônica/epidemiologia , Insuficiência Renal Crônica/etiologia , Insuficiência Renal Crônica/terapia , Idade de InícioRESUMO
Automated neuropsychiatric batteries have been used in research and clinical practice, including for chronic diseases, such as Systemic Lupus Erythematosus. The Pediatric Automated Neuropsychological Assessment Metrics battery (Ped-ANAM), originally developed for use in American-English speaking individuals, allows tracking of cognitive functions. It can be applied to people over 9 years old. The aim of this study was to translate and present initial validation data from the Ped-ANAM into Brazilian-Portuguese. We translated the battery according to Beaton's guidelines. Psychometric properties were tested, internal consistency was analyzed by Cronbach's alpha coefficient, test-retest reliability by the intraclass correlation coefficient (ICC). Further, we measured the test execution speed at both times as a temporal stability. Principal component analysis (PCA) was used for structural validity. Evidence of construct validity was assessed through assessment of the relationships with the Wechsler Intelligence Scales. All participants prior to the start of study related activities signed an informed consent form approved by the local ethics committee. A sample of 230 individuals [mean (range) of age: 23 (9 to 60) years; 65% females] was included; a subset of 51 individuals [mean (range) of age: 18 (9 to 57) years, 59% female] completed the Ped-ANAM twice to assess test-retest reliability, and another subset of 54 individuals [mean (range) of age: 20.4 (7 to 62) years; 67% female] completed the Wechsler Intelligence Scales for Children and Adult for assessment of the Ped-ANAM's construct validity. Our results suggest that the internal consistency of the Ped-ANAM (Cronbach's α = 0.890) and its subtest test-retest reliability were excellent (ICC: 0.59 to 0.94). There was no clustering in the Principal Components Analysis, suggestive of non-grouping of the evaluated variables. Construct validity assessment to the Wechsler Scales showed expected ranges of low to strong correlations (Spearman correlations: ρ = 0.40 to ρ = 0.69). We concluded that, based on the results of this study, a cross-culturally validated Brazilian-Portuguese version of the Ped-ANAM has been developed and it is a reliable tool for the screening cognitive function.
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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.
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Anticorpos Antifosfolipídeos/imunologia , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/etnologia , Adolescente , Idade de Início , Indígena Americano ou Nativo do Alasca , Povo Asiático , População Negra , Brasil/epidemiologia , Brasil/etnologia , Criança , Pré-Escolar , Etnicidade , Feminino , Humanos , Lactente , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Estudos Retrospectivos , Índice de Gravidade de Doença , População BrancaRESUMO
OBJECTIVE: To evaluate symptomatic polyautoimmunity (PA) at childhood-onset systemic lupus erythematosus(cSLE) diagnosis, and its association with demographic data, disease activity, clinical manifestations and laboratorial abnormalities in a large Brazilian cSLE population. METHODS: A multicenter retrospective study was performed in 1463 cSLE(ACR criteria) patients from 27 Pediatric Rheumatology services. Symptomatic PA was defined according to the presence of more than one concomitant autoimmune disease(AD) and symptomatic multiple autoimmune syndrome(MAS) was defined as three or more AD. An investigator meeting was held to define the protocol. Demographic data, SLICC classification criteria and SLEDAI-2K were evaluated. RESULTS: At cSLE diagnosis symptomatic PA was observed in 144/1463(9.8%) and symptomatic MAS occurred in solely 10/1463(0.7%). In the former group the more frequently observed associated AD were Hashimoto thyroiditis nâ¯=â¯42/144(29%), antiphospholipid syndrome nâ¯=â¯42/144(29%), autoimmune hepatitis nâ¯=â¯26/144(18%) and type 1 diabetes mellitus nâ¯=â¯23/144(15.9%). Further comparisons between cSLE patients with and without PA showed a higher median age(pâ¯=â¯0.016) and lower mean SLICC criteria (pâ¯=â¯0.039) in those with PA. Additionally, these cSLE patients had less renal involvement(35% vs. 44%, pâ¯=â¯0.038) and red blood cell cast(6% vs. 12%, pâ¯=â¯0.042) and more antiphospholipid antibodies(29% vs. 15%, pâ¯<â¯0.0001). CONCLUSIONS: Approximately 10% of cSLE had symptomatic PA at diagnosis, particularly endocrine autoimmune disorders and antiphospholipid syndrome. Lupus was characterized by a mild disease onset and MAS was infrequently evidenced. Further studies are necessary to determine if this subgroup of cSLE patients have a distinct genetic background with a less severe disease and a better long-term outcome.
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Autoimunidade/imunologia , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/diagnóstico , Índice de Gravidade de Doença , Adolescente , Idade de Início , Brasil/epidemiologia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Prevalência , Estudos RetrospectivosRESUMO
OBJECTIVE: To evaluate autoimmune hepatitis (AIH) in a multicenter cohort of childhood-onset systemic lupus erythematosus (cSLE) patients. METHODS: This retrospective multicenter study included 847 patients with cSLE, performed in 10 Pediatric Rheumatology services of São Paulo state, Brazil. AIH was defined according to the International Autoimmune Hepatitis Group criteria (IAHGC). The statistical analysis was performed using the Bonferroni's correction (p < 0.0033). RESULTS: AIH in cSLE patients confirmed by biopsy was observed in 7/847 (0.8%) and all were diagnosed during adolescence. The majority occurred before or at cSLE diagnosis [5/7 (71%)]. Antinuclear antibodies were a universal finding, 43% had concomitantly anti-smooth muscle antibodies and all were seronegative for anti-liver kidney microsomal antibodies. All patients with follow-up ≥18 months (4/7) had complete response to therapy according to IAHGC. None had severe hepatic manifestations such as hepatic failure, portal hypertension and cirrhosis at presentation or follow-up. Further comparison of 7 cSLE patients with AIH and 28 without this complication with same disease duration [0 (0-8.5) vs. 0.12 (0-8.5) years, p = 0.06] revealed that the frequency of hepatomegaly was significantly higher in cSLE patients in the former group (71% vs. 11%, p = 0.003) with a similar median SLEDAI-2 K score [6 (0-26) vs. 7 (0-41), p = 0.755]. No differences were evidenced regarding constitutional involvement, splenomegaly, serositis, musculoskeletal, neuropsychiatric and renal involvements, and treatments in cSLE patients with and without AIH (p > 0.0033). CONCLUSIONS: Overlap of AIH and cSLE was rarely observed in this large multicenter study and hepatomegaly was the distinctive clinical feature of these patients. AIH occurred during adolescence, mainly at the first years of lupus and it was associated with mild liver manifestations.
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Hepatite Autoimune/epidemiologia , Hepatomegalia/epidemiologia , Lúpus Eritematoso Sistêmico/epidemiologia , Adolescente , Idade de Início , Anticorpos Antinucleares/análise , Autoantígenos/análise , Brasil/epidemiologia , Criança , Feminino , Hepatite Autoimune/imunologia , Humanos , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Microssomos/imunologia , Músculo Liso/imunologia , Estudos RetrospectivosRESUMO
Abstract Objectives: To assess clinical digital vasculitis (DV) as an initial manifestation of childhood-onset systemic lupus erythematosus (cSLE) within a large population. Methods: Multicenter cross-sectional study including 852 cSLE patients (ACR criteria) followed in ten Pediatric Rheumatology centers in São Paulo State, Brazil. Results: DV was observed in 25/852 (3%) cSLE patients. Periungual hemorrhage was diagnosed in 12 (48%), periungual infarction in 7 (28%), tip finger ulceration in 4 (16%), painful nodules in 1 (4%) and gangrene in 1 (4%). A poor outcome, with digital resorption, occurred in 5 (20%). Comparison of patients with and without DV revealed higher frequency of malar rash (80% vs. 53%, p = 0.008), discoid rash (16% vs. 4%, p = 0.017), photosensitivity (76% vs. 45%, p = 0.002) and other cutaneous vasculitides (80% vs. 19%, p < 0.0001), whereas the frequency of overall constitutional features (32% vs. 61%, p = 0.003), fever (32% vs. 56%, p = 0.020) and hepatomegaly (4% vs. 23%, p = 0.026) were lower in these patients. Frequency of female gender, severe multi-organ involvement, autoantibodies profile and low complement were alike in both groups (p > 0.05). SLEDAI-2K median, DV descriptor excluded, was significantly lower in patients with DV compared to those without this manifestation [10 (0-28) vs. 14 (0-58), p = 0.004]. Visceral vasculitis or death were not observed in this cSLE cohort. The frequency of cyclophosphamide use (0% vs. 18%, p = 0.014) was significantly lower in the DV group. Conclusion: Our large multicenter study identified clinical DV as one of the rare initial manifestation of active cSLE associated with a mild multisystemic disease, in spite of digital resorption in some of these patients.
Resumo Objetivos: Avaliar a vasculite digital (VD) clínica como uma manifestação inicial do lúpus eritematoso sistêmico de início na infância (LESi) em uma grande população. Métodos: Estudo transversal multicêntrico que incluiu 852 pacientes com LESi (critérios do ACR), acompanhados em dez centros de reumatologia pediátrica do Estado de São Paulo. Resultados: Observou-se VD em 25/852 (3%) pacientes com LESi. Diagnosticaram-se hemorragia periungueal em 12 (48%), infarto periungueal em sete (28%), úlcera de ponta de dígito em quatro (16%), nódulos dolorosos em um (4%) e gangrena em um (4%). Um desfecho ruim, com reabsorção digital, ocorreu em cinco (20%) pacientes. A comparação entre pacientes com e sem VD revelou maior frequência de erupção malar (80% vs. 53%, p = 0,008), erupção discoide (16% vs. 4%, p = 0,017), fotossensibilidade (76% vs. 45% p = 0,002) e outras vasculites cutâneas (80% vs. 19%, p < 0,0001), enquanto a frequência de características constitucionais totais (32% vs. 61%, p = 0,003), febre (32% vs. 56% p = 0,020) e hepatomegalia (4% vs. 23%, p = 0,026) foram menores nesses pacientes. A frequência do gênero feminino, o envolvimento grave de múltiplos órgãos, perfil de autoanticorpos e baixo complemento foram semelhantes nos dois grupos (p > 0,05). A mediana no Sledai-2 K, exclusive o descritor de VD, foi significativamente menor nos pacientes com VD em comparação com aqueles sem essa manifestação [10 (0 a 28) vs. 14 (0 a 58), p = 0,004]. Não foram observadas vasculite visceral nem morte nessa coorte de pacientes com LESi. A frequência de uso de ciclofosfamida (0% vs. 18%, p = 0,014) foi significativamente menor no grupo VD. Conclusão: Este grande estudo multicêntrico identificou a VD clínica como uma rara manifestação inicial do LESi ativo, associada a doença multissistêmica leve, apesar da ocorrência de reabsorção digital em alguns desses pacientes.
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Humanos , Feminino , Criança , Adolescente , Vasculite/epidemiologia , Dedos do Pé , Dedos , Lúpus Eritematoso Sistêmico/epidemiologia , Vasculite/etiologia , Vasculite/fisiopatologia , Índice de Gravidade de Doença , Brasil/epidemiologia , Estudos de Casos e Controles , Estudos Transversais , Estudos Retrospectivos , Idade de Início , Lúpus Eritematoso Sistêmico/fisiopatologiaRESUMO
OBJECTIVES: To assess clinical digital vasculitis (DV) as an initial manifestation of childhood-onset systemic lupus erythematosus (cSLE) within a large population. METHODS: Multicenter cross-sectional study including 852 cSLE patients (ACR criteria) followed in ten Pediatric Rheumatology centers in São Paulo State, Brazil. RESULTS: DV was observed in 25/852 (3%) cSLE patients. Periungual hemorrhage was diagnosed in 12 (48%), periungual infarction in 7 (28%), tip finger ulceration in 4 (16%), painful nodules in 1 (4%) and gangrene in 1 (4%). A poor outcome, with digital resorption, occurred in 5 (20%). Comparison of patients with and without DV revealed higher frequency of malar rash (80% vs. 53%, p=0.008), discoid rash (16% vs. 4%, p=0.017), photosensitivity (76% vs. 45%, p=0.002) and other cutaneous vasculitides (80% vs. 19%, p<0.0001), whereas the frequency of overall constitutional features (32% vs. 61%, p=0.003), fever (32% vs. 56%, p=0.020) and hepatomegaly (4% vs. 23%, p=0.026) were lower in these patients. Frequency of female gender, severe multi-organ involvement, autoantibodies profile and low complement were alike in both groups (p>0.05). SLEDAI-2K median, DV descriptor excluded, was significantly lower in patients with DV compared to those without this manifestation [10 (0-28) vs. 14 (0-58), p=0.004]. Visceral vasculitis or death were not observed in this cSLE cohort. The frequency of cyclophosphamide use (0% vs. 18%, p=0.014) was significantly lower in the DV group. CONCLUSION: Our large multicenter study identified clinical DV as one of the rare initial manifestation of active cSLE associated with a mild multisystemic disease, in spite of digital resorption in some of these patients.
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Dedos , Lúpus Eritematoso Sistêmico/epidemiologia , Dedos do Pé , Vasculite/epidemiologia , Adolescente , Idade de Início , Brasil/epidemiologia , Estudos de Casos e Controles , Criança , Estudos Transversais , Feminino , Humanos , Lúpus Eritematoso Sistêmico/fisiopatologia , Estudos Retrospectivos , Índice de Gravidade de Doença , Vasculite/etiologia , Vasculite/fisiopatologiaRESUMO
Chronic arthritis (CA) is an unusual condition in childhood-onset systemic lupus erythematosus (cSLE) and data in children is very limited. The aim of the study is to assess CA in a large population of cSLE patients, in a multicenter cross-sectional study including 852 cSLE patients followed in ten Pediatric Rheumatology referral services in state of São Paulo, Brazil. CA was observed in 32/852 (3.7 %) cSLE patients mostly in hands and ankles. Chronic monoarthritis was diagnosed in four cSLE patients, oligoarthritis in nine and polyarthritis in 19. In the latter group, six had rhupus syndrome. Two oligoarticular patients had Jaccoud's arthropathy. CA was an isolated manifestation observed at disease onset in 13/32 (41 %) cSLE patients, and juvenile idiopathic arthritis (JIA) was the first diagnosis in 18/32 (56 %). The comparison of last visit of patients with CA and without this manifestation revealed higher frequency of splenomegaly (28 vs. 11 %, p = 0.002). The median of SLICC/ACR-DI score [1(0-9) vs. 0(0-7), p = 0.003] was significantly higher in CA patients compared to patients without this manifestation, likewise the frequency of musculoskeletal damage (31 vs. 9 % p = 0.001). Frequencies of treatment with nonsteroidal anti-inflammatory drugs (75 vs. 26 %, p < 0.0001), hydroxychloroquine sulfate (87 vs. 59 %, p = 0.001) and methotrexate (47 vs. 22 %, p = 0.001) were significantly higher in CA patients. This large multicenter study allowed us to characterize CA as a rare and early manifestation of cSLE, frequently mimicking JIA at disease onset. It is predominantly polyarticular, involving more often hands and ankles and it is associated with significant musculoskeletal accrual damage.
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Artrite/epidemiologia , Lúpus Eritematoso Sistêmico/epidemiologia , Adolescente , Adulto , Idade de Início , Artrite/diagnóstico , Brasil/epidemiologia , Criança , Pré-Escolar , Comorbidade , Estudos Transversais , Feminino , Humanos , Masculino , Prevalência , Estudos Retrospectivos , Índice de Gravidade de Doença , Adulto JovemRESUMO
ABSTRACT OBJECTIVE: Describe the clinical and laboratory profile, follow-up, and outcome of a series of cases of acute viral myositis. METHOD: A retrospective analysis of suspected cases under observation in the emergency department was performed, including outpatient follow-up with the recording of respiratory infection and musculoskeletal symptoms, measurement of muscle enzymes, creatine phosphokinase (CPK), lactate dehydrogenase (LDH), transaminases (AST and ALT), blood count, C-reactive protein, and erythrocyte sedimentation rate in the acute phase and during follow-up until normalization. RESULTS: Between 2000 and 2009, 42 suspected cases were identified and 35 (27 boys) were included. The median age was 7 years and the diagnosis was reported in 89% in the first emergency visit. The observed respiratory symptoms were cough (31%), rhinorrhea (23%), and fever (63%), with a mean duration of 4.3 days. Musculoskeletal symptoms were localized pain in the calves (80%), limited ambulation (57%), gait abnormality (40%), and muscle weakness in the lower limbs (71%), with a mean duration of 3.6 days. There was significant increase in CPK enzymes (5507 ± 9180 U/L), LDH (827 ± 598 U/L), and AST (199 ± 245 U/L), with a tendency to leukopenia (4590 ± 1420) leukocytes/mm3. The complete recovery of laboratory parameters was observed in 30 days (median), and laboratory and clinical recurrence was documented in one case after 10 months. CONCLUSION: Typical symptoms with increased muscle enzymes after diagnosis of influenza and self-limited course of the disease were the clues to the diagnosis. The increase in muscle enzymes indicate transient myotropic activity related to seasonal influenza, which should be considered, regardless of the viral identification, possibly associated with influenza virus or other respiratory viruses.
RESUMO OBJETIVO: Descrever o perfil clínico-laboratorial, o acompanhamento e o desfecho de uma série de casos de miosite aguda viral. MÉTODO: Foi conduzida uma análise retrospectiva de casos suspeitos, em observação em unidade de emergência, e seguimento ambulatorial com o registro de sintomas de infecção respiratória, sintomas músculo-esqueléticos, determinação de enzimas musculares, creatina-fosfoquinase (CPK), desidrogenase lática (DHL), transaminases (AST e ALT), hemograma, proteína C reativa e velocidade de hemossedimentação, na fase aguda e no acompanhamento, até a normalização. RESULTADOS: Entre 2000 e 2009, 42 casos suspeitos foram identificados e 35 (27 meninos) foram incluídos. A mediana de idade foi de sete anos e o diagnóstico relatado em 89%, na primeira visita de emergência. Os sintomas respiratórios observados foram: tosse (31%), coriza (23%) e febre (63%), com duração média de 4,3 dias. Os sintomas músculo-esqueléticos foram: dor localizada nas panturrilhas (80%), deambulação limitada (57%), marcha anormal (40%) e fraqueza muscular nos membros inferiores (71%), com duração média de 3,6 dias. Observou-se elevação importante das enzimas CPK (5.507 ± 9.180) U/l, DHL (827 ± 598) U/l e AST (199 ± 245) U/l e tendência a leucopenia (4.590 ± 1.420) leucócitos/mm3. A recuperação completa dos parâmetros laboratoriais foi observada em 30 dias (mediana) e a recaída clínica e laboratorial em um caso após 10 meses. CONCLUSÃO: Os sintomas típicos com enzimas musculares elevadas após diagnóstico de influenza e o curso autolimitado foram os indícios para o diagnóstico. A elevação de enzimas musculares indica a atividade miotrópica transitória relacionada à influenza sazonal que deve ser considerada, a despeito da identificação viral, possivelmente associada com o vírus influenza ou outros vírus respiratórios.
Assuntos
Criança , Pré-Escolar , Feminino , Humanos , Masculino , Miosite/virologia , Viroses/complicações , Doença Aguda , Proteína C-Reativa/análise , Ensaios Enzimáticos Clínicos/métodos , Creatina Quinase Forma MB/sangue , Creatina Quinase/sangue , Influenza Humana/complicações , Debilidade Muscular/etiologia , Miosite/diagnóstico , Estudos Retrospectivos , Distribuição por Sexo , Fatores de Tempo , Transaminases/sangue , Viroses/diagnósticoRESUMO
BACKGROUND: Data regarding the prevalence of chronic spontaneous urticaria (CSU) in childhood-onset systemic lupus erythematosus (cSLE) patients and possible associated factors are limited to a few case reports. The objectives of this study were to assess CSU in a large cSLE population, in order to evaluate the demographic data, clinical manifestations, disease activity/damage, laboratory abnormalities and treatment. METHODS: A retrospective multicenter cohort study (Brazilian cSLE group) was performed in 10 Pediatric Rheumatology services and included 852 cSLE patients. CSU was diagnosed according to the guidelines of the European Academy of Allergy and Clinical Immunology, the Global Allergy and Asthma European Network, the European Dermatology Forum and the World Allergy Organization. Patients with CSU (evaluated at urticaria diagnosis) and patients without CSU (evaluated at the last visit) were assessed for lupus clinical/laboratory features and treatment. RESULTS: CSU was observed in 10/852 cSLE patients (1.17%). The median of cSLE duration at urticaria diagnosis was 0 (-3 to 5) years. Comparison of cSLE patients with and without CSU revealed a greater frequency of constitutional symptoms (40 vs. 8%, p = 0.006), reticuloendothelial system involvement (30 vs. 3%, p = 0.003), mucocutaneous (90 vs. 28%, p < 0.0001) and musculoskeletal manifestations (50 vs. 6%, p < 0.0001) and methylprednisolone pulse therapy use (60 vs. 9%, p < 0.0001) in the former group. The frequency of immunosuppressive treatment was lower in patients with CSU (p = 0.018). The median SLE Disease Activity Index 2000 (12 vs. 2, p < 0.0001) and erythrocyte sedimentation rate (40 vs. 19 mm/1st hour, p = 0.024), was higher in patients with CSU. CONCLUSIONS: To our knowledge, this is the first study with evidence that CSU may be linked to cSLE. We also demonstrated that this particular skin manifestation occurs predominantly at disease onset and is associated with lupus moderate/high disease activity without major organ involvement.
Assuntos
Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/epidemiologia , Urticária/epidemiologia , Urticária/etiologia , Adolescente , Idade de Início , Brasil/epidemiologia , Criança , Pré-Escolar , Doença Crônica , Feminino , Humanos , Lactente , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Masculino , Estudos Retrospectivos , Urticária/diagnóstico , Urticária/tratamento farmacológicoRESUMO
OBJECTIVE: Describe the clinical and laboratory profile, follow-up, and outcome of a series of cases of acute viral myositis. METHOD: A retrospective analysis of suspected cases under observation in the emergency department was performed, including outpatient follow-up with the recording of respiratory infection and musculoskeletal symptoms, measurement of muscle enzymes, creatine phosphokinase (CPK), lactate dehydrogenase (LDH), transaminases (AST and ALT), blood count, C-reactive protein, and erythrocyte sedimentation rate in the acute phase and during follow-up until normalization. RESULTS: Between 2000 and 2009, 42 suspected cases were identified and 35 (27 boys) were included. The median age was 7 years and the diagnosis was reported in 89% in the first emergency visit. The observed respiratory symptoms were cough (31%), rhinorrhea (23%), and fever (63%), with a mean duration of 4.3 days. Musculoskeletal symptoms were localized pain in the calves (80%), limited ambulation (57%), gait abnormality (40%), and muscle weakness in the lower limbs (71%), with a mean duration of 3.6 days. There was significant increase in CPK enzymes (5507±9180U/L), LDH (827±598U/L), and AST (199±245U/L), with a tendency to leukopenia (4590±1420) leukocytes/mm(3). The complete recovery of laboratory parameters was observed in 30 days (median), and laboratory and clinical recurrence was documented in one case after 10 months. CONCLUSION: Typical symptoms with increased muscle enzymes after diagnosis of influenza and self-limited course of the disease were the clues to the diagnosis. The increase in muscle enzymes indicate transient myotropic activity related to seasonal influenza, which should be considered, regardless of the viral identification, possibly associated with influenza virus or other respiratory viruses.
Assuntos
Miosite/virologia , Viroses/complicações , Doença Aguda , Proteína C-Reativa/análise , Criança , Pré-Escolar , Ensaios Enzimáticos Clínicos/métodos , Creatina Quinase/sangue , Creatina Quinase Forma MB/sangue , Feminino , Humanos , Influenza Humana/complicações , Masculino , Debilidade Muscular/etiologia , Miosite/diagnóstico , Estudos Retrospectivos , Distribuição por Sexo , Fatores de Tempo , Transaminases/sangue , Viroses/diagnósticoRESUMO
BACKGROUND: Muscle atrophy and asymmetric extremity growth is a common feature of linear scleroderma (LS). Extra-cutaneous features are also common and primary neurologic involvement, with sympathetic dysfunction, may have a pathogenic role in subcutaneous and muscle atrophy. The aim was investigate nerve conduction and muscle involvement by electromyography in pediatric patients with LS. METHODS: We conducted a retrospective review of LS pediatric patients who had regular follow up at a single pediatric center from 1997-2013. We selected participants if they had consistently good follow up and enrolled consecutive patients in the study. We examined LS photos as well as clinical, serological and imaging findings. Electromyograms (EMG) were performed with bilateral symmetric technique, using surface and needle electrodes, comparing the affected side with the contralateral side. Abnormal muscle activity was categorized as a myopathic or neurogenic pattern. RESULTS: Nine LS subjects were selected for EMG, 2 with Parry-Romberg/Hemifacial Atrophy Syndrome, 7 linear scleroderma of an extremity and 2 with mixed forms (linear and morphea). Electromyogram analysis indicated that all but one had asymmetric myopathic pattern in muscles underlying the linear streaks. Motor and sensory nerve conduction was also evaluated in upper and lower limbs and one presented a neurogenic pattern. Masticatory muscle testing showed a myopathic pattern in the atrophic face of 2 cases with head and face involvement. CONCLUSION: In our small series of LS patients, we found a surprising amount of muscle dysfunction by EMG. The muscle involvement may be possibly related to a secondary peripheral nerve involvement due to LS inflammation and fibrosis. Further collaborative studies to confirm these findings are needed.
Assuntos
Deformidades Adquiridas do Pé/prevenção & controle , Deformidades Adquiridas da Mão/prevenção & controle , Músculo Esquelético , Atrofia Muscular , Esclerodermia Localizada , Pré-Escolar , Estudos Transversais , Diagnóstico Precoce , Intervenção Médica Precoce , Eletromiografia/métodos , Feminino , Deformidades Adquiridas do Pé/etiologia , Deformidades Adquiridas da Mão/etiologia , Humanos , Masculino , Músculo Esquelético/inervação , Músculo Esquelético/patologia , Músculo Esquelético/fisiopatologia , Atrofia Muscular/diagnóstico , Atrofia Muscular/etiologia , Atrofia Muscular/fisiopatologia , Condução Nervosa/fisiologia , Avaliação de Resultados em Cuidados de Saúde , Estudos Retrospectivos , Esclerodermia Localizada/complicações , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/fisiopatologia , Esclerodermia Localizada/terapiaRESUMO
OBJECTIVES: Evaluate damage in oligoarticular JIA, estimating its frequency, risks and probability over time. METHODS: A cross-sectional and retrospective analysis of Juvenile Arthritis Damage Index (JADI) scoring, with both articular and extraarticular components, active joint count, disability index by CHAQ and Steinbrocker class, physician's global assessment, child's pain and overall well-being visual analogue scale (VAS), was conducted in patients with oligoarticular JIA. Damage risk factors were estimated by univariate analysis and by generalised linear model. The probability of damage over time was estimated by survival analysis and damage progression rates were calculated by hazard function. RESULTS: Seventy-five JIA cases were assessed, 89.3% persistent and 10.7% extended oligoarthritis, with median follow-up duration 1.7 years (IQR 1.3-3.1). Damage occurred in 38.7%. JADI-A correlated moderately only with the number of limited joints (rs= 0.50, p<0.0001). Female sex (OR 3.5, 95% CI 1.0-11.6), DMARD use (OR 3.9, 95%CI 1.0-15.0) and knee involvement (OR 4.2, 95%CI 1.3-13.5) were significantly associated with joint damage, whereas only joint steroid injection was associated with extraarticular damage (OR 5.9, 95% CI 1.8-19.3). Damage probability at 5 years was 50% for JADI-A, and 57% for JADI-E. Calculated hazard rates each year were 16.1% and 16.3%, for JADI-A and JADI-E, respectively. CONCLUSIONS: Sex, DMARD use and knee involvement were associated with joint damage, whereas only joint steroid injection was associated with extraarticular damage, which progressed at stable rates over ten years.