Melissa Mariti Fraga

Features of 847 Childhood-Onset Systemic Lupus Erythematosus Patients in Three Age Groups at Diagnosis: A Brazilian Multicenter Study

To evaluate demographic data and clinical and laboratory features at disease diagnosis in 3 different age groups of childhood‐onset systemic lupus erythematosus (SLE): group A, early‐onset (<6 years); group B, school age (≥6 to <12 years); and group C, adolescent (≥12 to <18 years).

Sleep, stress, neurocognitive profile and healthrelated quality of life in adolescents with idiopathic musculoskeletal pain

OBJECTIVES: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Dor musculoesquelética idiopática difusa na infância e na adolescência

Objective: Persistent or intermittent diffuse idiopathic musculoskeletal pain is defined by the presence of pain over three months with no apparent etiology. The diagnosis of this condition in children and adolescents is increasing. The objective of this study was to report the cases of two patients with disabling pain that received multidisciplinary treatment and to review the current literature about this subject. Case description: The first patient is a nine-year old girl with history of headache, musculoskeletal pain and abdominal pain for two years. During this period she had four episodes of absence of gait with no apparent organic cause. The second patient is a 14-year girl with severe daily low back pain for 14 months, followed by headache and fatigue. Both patients presented a normal physical examination, except for allodynia (case 1) and fibromyalgia tender points (case 2). All tests resulted negative regarding the search for organic reasons for pain complaints. Significant clinical improvement was observed with a multidisciplinary approach offered by pediatricians, psychologists, physical therapists, and nutritionists. Both patients resumed their daily regular activities. Comments: Team integrated performance in a short-term period can provide the attenuation of pain symptoms since emotional, physical and nutritional factors are related to the pain expressed by the patients.

Alterações oftalmológicas decorrentes do tratamento do lúpus eritematoso sistêmico juvenil

OBJECTIVE: To assess retrospectively the ocular changes in children and adolescents with juvenile systemic lupus erythematosus (JSLE) in a tertiary pediatric rheumatology service. METHODS: This study assessed 117 JSLE patients (85.5% female, 60.7% non-Caucasian), who met at least four criteria of the 1997 SLE classification of the American College of Rheumatology. Their mean age was 10.4 years, and their mean time of disease progression was 5.4 years. A protocol containing clinical and demographic data, ophthalmologic complaints and changes, age of onset, duration of medication use, and cumulative medication dose was applied. RESULTS: Of the 117 patients, 24 (20.5%) had ocular changes. Sixteen of them had abnormal fundoscopy associated with systemic hypertension and/or use of chloroquine; four had cataract; two had glaucoma; and two had cataract and glaucoma. The mean age of ocular change onset was 14.1 years. Patients with ocular changes received statistically higher and longer doses of glucocorticoid pulse therapy as compared with patients without ocular changes [1.5 (0.4 to 1.6) versus 1 (0.2 to 1.6) mg/kg, P = 0.003; 25.7 (2-99) versus 17.8 (1-114) months, P = 0.0001, respectively]. CONCLUSION: A high prevalence of ocular changes relating mainly to the treatment of JSLE was observed. This demonstrates the need for regular ophthalmologic examinations even in asymptomatic patients, aiming at the early diagnosis and intervention, and at decreasing the ocular morbidity related to that disease.

Rituximab use in young adults diagnosed with juvenile idiopathic arthritis unresponsive to conventional treatment: report of 6 cases

Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood. Without an effective therapy, patients may progress quickly to functional disability. Recently, depletion of B cells emerged as a new approach for the treatment of autoimmune diseases, including JIA. We describe six cases of JIA patients followed at a referral center for Rheumatology and Pediatric Rheumatology, submitted to treatment with rituximab (RTX) after refractoriness to three anti-TNF agents. Patients received RTX cycles with two infusions every six months. Response to treatment was assessed by DAS28, HAQ/CHAQ, and an overall assessment by the doctor and the patient. Of our six patients, four were girls (mean age at onset of disease: 6.1 years; mean disease evolution time: 15.1 years; mean age upon receiving RTX: 21.6 years). Four patients belonged to polyarticular subtype (1 rheumatoid factor [RF]-negative, 3 FR-positive), a patient with systemic JIA subtype with a polyarticular course and arthritis related to enthesitis. Of our six patients, five responded to treatment; and during the course of 12 months, the clinical response was maintained, although not sustained. However, discontinuation by infusion reactions caused the withdrawal of RTX in two patients. The use of RTX in JIA is restricted to cases refractory to other biological agents and, even considering that this study was held in a small number of advanced patients, RTX proved to be an effective therapeutic option.

PERCEPÇÃO E ENFRENTAMENTO DA DOR EM CRIANÇAS E ADOLESCENTES COM FIBROMIALGIA JUVENIL E ARTRITE IDIOPÁTICA JUVENIL POLIARTICULAR

ABSTRACT Objective: To measure and compare musculoskeletal pain in patients with juvenile fibromyalgia (JFM) and polyarticular juvenile idiopathic arthritis (JIA), and to evaluate and compare pain perception and pain coping mechanisms in these patients. Methods: In this cross sectional study, we evaluated 150 children and adolescents, and their respective parents, from 3 different groups: JFM, polyarticular JIA, and healthy controls. Pain intensity and pain coping mechanisms were measured using specific questionnaires. Pain perception was evaluated according to three illustrations simulating situations that might cause pain: a shot, a bicycle fall, and social isolation. The patients’ parents also filled out the questionnaires and provided a pain score that matched their child’s perception of pain for each illustration. Results: The highest pain scores, the lowest pain coping strategy scores, the highest pain perception scores for all three illustrations, and the worse health related to quality of life indicators were observed in the JFM group, when compared to the JIA and control groups. The same pattern was observed with their parents. Conclusions: Patients with JIA and JFM behave differently in relation to pain perception and the development pain coping mechanisms. Pain should be evaluated from different perspectives for an individualized and efficient treatment of patients.

Symptomatic polyautoimmunity at diagnosis of 1463 childhood-onset lupus: A Brazilian multicenter study

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 n = 42/144(29%), antiphospholipid syndrome n = 42/144(29%), autoimmune hepatitis n = 26/144(18%) and type 1 diabetes mellitus n = 23/144(15.9%). Further comparisons between cSLE patients with and without PA showed a higher median age(p = 0.016) and lower mean SLICC criteria (p = 0.039) in those with PA. Additionally, these cSLE patients had less renal involvement(35% vs. 44%, p = 0.038) and red blood cell cast(6% vs. 12%, p = 0.042) and more antiphospholipid antibodies(29% vs. 15%, p < 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.

[Devic's disease in an adolescent girl with juvenile dermatomyositis].

Devics disease, also known as neuromyelitis optica, is an autoimmune inflammatory demyelinating disorder of the central nervous system that mainly affects the optic nerve and spinal cord. Recently, Devics disease was demonstrated to be a channelopathy due to the presence of antibodies against the water channel aquaporin-4 in the blood-brain barrier. There have been reports of Devics disease in infancy, but there are few reported associations of Devics disease with other diseases. The association of Devics disease with dermatomyositis has not yet been described in the literature. The aim of this paper is to describe the first case of Devics disease in an adolescent with juvenile dermatomyositis.

Psychosocial profile of children and adolescents followed in a pediatric musculoskeletal pain clinic

Amplified pain syndrome preferentially affects girls between the ages of 10-17 years. It is a disease with multiple causes that could be associated with major psychosocial disorders of patients and caregivers, affecting their quality of life. These psychosocial aspects can interfere intensifying the pain.

PReS-FINAL-2261: Prevalence of orofacial symptoms and signs in patients with juvenile fibromyalgia

Fibromyalgia may coexist with other clinical conditions such temporomandibular disorders (TMD). Temporomandibular disorder is a term embracing clinical disorders that involve the masticatory musculature, the temporomandibular joints, and associated structures.