Characteristics of 1555 childhood-onset lupus in three groups based on distinct time intervals to disease diagnosis: a Brazilian multicenter study.

Objective The objective of this study was to compare demographic data, clinical/laboratorial features and disease activity at diagnosis in three different groups with distinct time intervals between onset of signs/symptoms and disease diagnosis. Methods A multicenter study was performed in 1555 childhood-onset systemic lupus erythematosus (American College of Rheumatology criteria) patients from 27 pediatric rheumatology services. Patients were divided into three childhood-onset systemic lupus erythematosus groups: A: short time interval to diagnosis (<1 month); B: intermediate time interval (≥1 and <3 months); and C: long time interval (≥3 months). An investigator meeting was held to define the protocol. Demographic data, SLICC classification criteria and SLEDAI-2 K were evaluated. Results The number of patients in each group was: A = 60 (4%); B = 522 (33.5%); and C = 973 (62.5%). The median age at diagnosis (11.1 (4.2-17) vs. 12 (1.9-17.7) vs. 12.5 (3-18) years, P = 0.025) was significantly lower in group A compared with groups B and C. The median number of diagnostic criteria according to SLICC (7 (4-12) vs. 6 (4-13) vs. 6 (4-12), P < 0.0001) and SLEDAI-2 K (18 (6-57) vs. 16 (2-63) vs. 13 (1-49), P < 0.0001) were significantly higher in group A than the other two groups. The frequency of oral ulcers in the palate (25% vs. 15% vs. 11%, P = 0.003), pleuritis (25% vs. 24% vs. 14%, P < 0.0001), nephritis (52% vs. 47% vs. 40%, P = 0.009), neuropsychiatric manifestations (22% vs. 13% vs. 10%, P = 0.008), thrombocytopenia (32% vs. 18% vs. 19%, P = 0.037), leucopenia/lymphopenia (65% vs. 46% vs. 40%, P < 0.0001) and anti-dsDNA antibodies (79% vs. 66% vs. 61%, P = 0.01) were significantly higher in group A compared with the other groups. In contrast, group C had a less severe disease characterized by higher frequencies of synovitis (61% vs. 66% vs. 71%, P = 0.032) and lower frequencies of serositis (37% vs. 33% vs. 25%, P = 0.002), proteinuria >500 mg/day (48% vs. 45% vs. 36%, P = 0.002) and low complement levels (81% vs. 81% vs. 71%, P < 0.0001) compared with groups A or B. Conclusions Our large Brazilian multicenter study demonstrated that for most childhood-onset systemic lupus erythematosus patients, diagnosis is delayed probably due to mild disease onset. Conversely, the minority has a very short time interval to diagnosis and a presentation with a more severe and active multisystemic condition.

Electroconvulsive therapy as a treatment for refractory neuropsychiatric lupus with catatonia: three case studies and literature review.

Neuropsychiatric disorders associated with systemic lupus erythematosus are very common. Treatment generally consists of glucocorticoids and immunosuppressive therapy; however, some cases are unresponsive. Electroconvulsive therapy (ECT) is a recognized treatment modality in psychiatry and is an option for refractory cases of neuropsychiatric lupus. This report describes three cases of neuropsychiatric lupus that improved with ECT after failure of antipsychotics and immunosuppressive therapy. All cases met DSM-5 criteria for catatonia (case 1: agitation, stereotypies, and grimacing; case 2: stupor, mutism, and grimacing; case 3: agitation, mutism, and stereotypies); therefore, ECT was indicated. This case series shows that ECT can be a therapeutic option in patients with neuropsychiatric lupus, especially when associated with catatonia and unresponsive to conventional treatment.

Risk factors for amenorrhea in juvenile systemic lupus erythematosus (JSLE): a Brazilian multicentre cohort study.

We evaluated the prevalence and clinical associations of amenorrhea in 298 female juvenile systemic lupus erythematosus (JSLE) patients (ACR criteria) followed in 12 Brazilian Paediatric Rheumatology centres. Amenorrhea was observed in 35 patients (11.7%) with a mean duration of 7.2 +/- 3.6 months. The hormones were performed in 32/35 patients and none of them had FSH and LH levels above and estradiol below the normal range according to pubertal changes. JSLE patients with amenorrhea were younger (15.04 +/- 2.5 versus 17.8 +/- 3.1 years; P = 0.001), and had a shorter period of time between menarche and current age (3.4 +/- 2.9 versus 6.7 +/- 5.4 years; P = 0.001). Interestingly, the frequency, cumulative dose, number of pulses and duration of intravenous cyclophosphamide treatment were alike in patients with and without amenorrhea (P > 0.05). In contrast, patients with amenorrhea had significantly higher SLEDAI (P = 0.01) and SLICC/ACR-DI (P = 0.024) scores compared to those without this condition. Independent risk factors identified by multivariate analysis were higher SLEDAI (OR = 1.059; CI = 1.004-1.116; P = 0.034) and SLICC/ACR-DI (OR = 2.125; IC = 1.373-3.291; P = 0.001) scores. Our data suggest that in spite of immunosuppressive therapy, JSLE patients have an adequate ovarian follicular reserve and amenorrhea is particularly associated with disease activity and damage.

The Brazilian version of the Childhood Health Assessment Questionnaire (CHAQ) and the Child Health Questionnaire (CHQ).

We report the cross-cultural adaptation and validation into Brazilian-Portuguese of the parent's version of two health related quality of life instruments. The Childhood Health Assessment Questionnaire (CHAQ) is a disease specific health instrument that measures functional ability in daily living activities in children with juvenile idiopathic arthritis (JIA). The Child Health Questionnaire (CHQ) is a generic health instrument designed to capture the physical and psychosocial well-being of children regardless the underlying disease. The Brazilian CHAQ was revalidated, while the CHQ has been derived from the Portuguese version. A total of 471 subjects were enrolled: 157 patients with JIA (27% systemic onset, 38% polyarticular onset, 9% extended oligoarticular subtype, and 26% persistent oligoarticular subtype) and 314 healthy children. The CHAQ discriminated clinically healthy subjects from JIA patients, with the systemic, polyarticular and extended oligoarticular subtypes having a higher degree of disability, pain, and lower overall well-being scores when compared to their healthy peers. Also the CHQ discriminated clinically healthy subjects from JIA patients, with the systemic onset, polyarticular onset and extended oligoarticular subtypes having a lower physical and psychosocial well-being score when compared to their healthy peers. In conclusion the Brazilian versions of the CHAQ-CHQ are reliable and valid tools for the combined physical and psychosocial assessment of children with JIA.

[Enthesopathy: a symptom seldom recognized by pediatricians] / Entesopatia: um sintoma pouco reconhecido pelo pediatra.

OBJECTIVE: The disease in the entheses, which are sites of attachment of tendons, ligaments, fascias or joint capsules to bone, may be noted clinically by the onset of tenderness or pain associated to digitopression in certain points. This revision article aims to draw the attention of pediatricians to the enthesopathies or enthesitis (inflammatory disease of these structures) in childhood and adolescence. METHODS: The study reviews concepts about the structure of the enthesis and the clinical, laboratorial and radiological features concerning enthesopathy, as well as differential diagnosis and therapeutic measures. Articles and texts concerning the theme were obtained based on a research in Medline (available data since 1966) and Lilacs (available data since 1981) database, as well as in Pediatric Rheumatology textbooks published after 1990. RESULTS: Enthesopathy in children and adolescents appears more often in the limbs and seems to be associated to the development of spondyloarthropathies, occurring less commonly in other inflammatory diseases or even noninflammatory conditions. CONCLUSIONS: The identification of enthesopathies is important to the early diagnosis of children and adolescents in risk of developing spondyloarthropathies, so that they may be included early in an adequate program of physical and drug therapies.

Estudo clínico multicêntrico de naproxeno suspensäo vs ácido acetilsalicílico no tratamento da artrite reumatóide juvenil / Multicenter clinical trial with naproxen suspension vs acetylcalicylic acid in the treatment of juvenile rheumatoid arthritis

Os autores relatam os resultados obtidos num ensaio clínico multicêntrico, simples-cego, de naproxeno suspensäo vs. ácido acetilsalicílico no tratamento de 52 crianças com artrite reumatóide juvenil. Os pacientes foram divididos em dois grupos de 26 cada um, aos quais foram administrados naproxeno suspensäo (25 mg/ml, na dose de 10 mg/Kg/dia, divididos em duas administraçöes diárias, ou ácido acetilsalicílico, em comprimidos de 500 mg, na dose de 100 mg/Kg/dia divididos em quatro administraçöes diárias. A duraçäo do estudo foi de 60 dias, para ambos os grupos, e a idade das crianças variou de dois a 14 anos. A avaliaçäo final feita pelos investigadores apresentou os seguintes resultados: no grupo naproxeno (69,2%), regular em 5(19,2%) e má em 3(11,5%). No grupo ácido acetilsalicílico os resultados foram bons em 16 pacientes (61,4%), regulares em 5(19,2%) e maus em 5(19,2%). Ao final, 80,7% dos pais, 57,6% das crianças e 69,2% dos investigadores consideraram os resultados do uso do naproxeno entre muito bons ou bons, comparados respectivamente, a 61,4%, 49,9% e 61,4% com o uso do ASS. Quanto aos efeitos colaterais apesar de mais freqüentes no grupo com AAS (vômitos, náuseas, dor epigástrica, cefaléia) do que no grupo com naproxeno (náuseas, diarréia, epistaxe), em geral foram leves e transitórios, com exceçäo de uma criança do grupo naproxeno, que apresentou hematúria macroscópica e foi retirada do estudo, observando-se regressäo completa da lateraçäo em 15 dias. A colaboraçäo das crianças ao esquema terapêutico com o naproxeno suspensäo foi mais facilmente obtida, näo só por necessitar de apenas duas administraçöes diárias, comparadas as quatro do ácido acetilsalicílico, como pelo fato de ser formulaçäo líquida e ter sabor agradável. Teve influência, também, o aspecto cultural negativo que grande parte dos pais tem quanto ao ácido acetilsalicílico, por considerarem-no uma medicaçäo de uso comum, pouco adequada a uma doença täo séria como a artrite reumatóide juvenil e, eventualmente, por ter sido usada anteriormente em algumas crianças...