Clinical, immunological and microbial gingival profile of juvenile systemic lupus erythematosus patients.

Periodontal disease has been associated with rheumatic diseases; however, few studies have evaluated the association with systemic lupus erythematosus (SLE), and its impact on the local inflammatory and microbial profiles. Therefore, this study evaluated the levels of several cytokines in gingival crevicular fluid (GCF) and serum from juvenile SLE (jSLE) patients with gingival inflammation, compared with controls. In addition, we assessed their subgingival microbial profile. Thirty jSLE patients and 29 systemically healthy individuals were recruited. Participants were rheumatologically and periodontally examined, and GCF, serum and intrasulcular biofilm were collected. Cytokines were analysed by bead-based multiplex assays and the bacterial profile by checkerboard DNA-DNA hybridization. jSLE patients presented higher percentages of dental plaque and bleeding than controls, as well as increased mean probing depth and attachment loss. After adjustment for multiple comparisons, GCF levels of interleukin (IL)-1ß, IL-8, granulocyte colony-stimulating factor (G-CSF), interferon-γ and monocyte chemoattractant protein-1 were significantly higher, whereas the levels of granulocyte-macrophage colony-stimulating factor were significantly lower in jSLE patients. In serum, G-CSF levels tended to be higher in jSLE patients (adjusted p-value = 0.06). Intrasulcular counts of Aggregatibacter actinomycetemcomitans were significantly higher in jSLE patients as compared with controls. We conclude that patients with jSLE present a worse periodontal condition associated with altered levels of pro-inflammatory cytokines in GCF and increased counts of A. actinomycetemcomitans in the intrasulcular biofilm.

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.

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.

[Rheumatic diseases in adolescence] / Doenças reumáticas na adolescência.

OBJECTIVE: To present an updated review concerning the most prevalent diseases with musculoskeletal signs and symptoms that make adolescents seek medical care, giving special emphasis to rheumatic diseases. Our aim is to offer physicians and health care providers the possibility of distinct differential diagnoses, thus allowing them to establish a therapeutic approach and, if necessary, refer the patient to a specialist METHODS: Review of literature using Medline database, data obtained at our department, and the authors personal experience. RESULTS: Musculoskeletal pain is characteristic of several diseases and usually urges adolescents to seek medical care. Rheumatic diseases, especially rheumatic fever, account for nearly fifty percent of the cases. In adolescents, it is also important that the aspects regarding the diagnosis and treatment of idiopathic juvenile arthritis, arthritis associated with enthesitis, systemic lupus erythematosus, and vasculitis be considered. Fibromyalgia, reflex sympathetic dystrophy, growing pains, hypermobility syndrome, and psychogenic rheumatism are noninflammatory conditions that frequently mimic rheumatic diseases. CONCLUSIONS: Inflammatory and noninflammatory conditions, and diseases of different etiology (infectious, neoplastic, and orthopedic) are frequently associated with musculoskeletal pain. It is important that health professionals diagnose these diseases as early as possible so that prompt action can be taken and prognosis can be improved.

Clinical significance of anticardiolipin antibodies in juvenile idiopathic arthritis.

OBJECTIVE: Anticardiolipin antibodies (aCL) have been demonstrated in a large spectrum of autoimmune diseases. However, its occurrence in childhood, in particular in juvenile idiopathic arthritis (JIA), is not well established. The present study addressed the frequency and clinical significance of aCL in a group of JIA patients. METHODS: aCL (IgG and IgM isotypes), antinuclear antibodies (ANA), and rheumatoid factor (RF) were determined in 86 children with JIA (33 systemic, 31 polyarticular and 22 oligoarticular onset type). Thirty-two juvenile systemic erythematosus lupus patients (JSLE) and 52 healthy children formed the control groups. The disease activity and functional status of the JIA patients were scored to study their possible associations with the presence of aCL. RESULTS: Serum aCL levels above the normal range were detected in 28/86 JIA patients (32.5%), 12/32 JSLE patients (37.5%), and 3/52 healthy children (6%). Positive aCL levels were slightly or moderately elevated (usually below 30 GPL and 20 MPL). The presence of aCL was not associated with the presence of ANA or RF. Associations between aCL and clinical parameters, such as disease onset, duration, activity or severity could not be established. No JIA patient had vascular thrombosis, thrombocytopenia or "livedo reticularis". CONCLUSION: aCL occurred in low titers in JIA children, in a similar frequency to that observed in JSLE. No association with JIA clinical parameters or the clinical features classically linked to the antiphospholipid antibody syndrome were observed.

Antiperinuclear factor and antibodies to the stratum corneum of rat esophagus in juvenile idiopathic arthritis.

We analyzed the frequency and clinical correlates of antiperinuclear factor (APF) and antibodies to the stratum corneum of rat esophagus in 86 children with juvenile idiopathic arthritis (JIA), 32 children with juvenile systemic lupus erythematosus, and 52 healthy children. Forty-two patients with JIA (49%) were positive for APF. No association was observed between APF and current age, sex, JIA subtype, age at disease onset, or disease duration. APF was found in one patient with juvenile systemic lupus erythematosus and in no healthy child. Antibodies to the stratum corneum of rat esophagus were detected in 3 patients with polyarticular JIA. APF may be a valuable tool in the differential diagnosis of 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.