Major improvements in health-related quality of life during the use of etanercept in patients with previously refractory juvenile idiopathic arthritis.

OBJECTIVE: To evaluate changes in health-related quality of life (HRQoL) in patients with refractory juvenile idiopathic arthritis (JIA) who are being treated with etanercept. METHODS: 53 patients with JIA from seven Dutch centres were included. HRQoL was measured by the Childhood Health Assessment Questionnaire (CHAQ), Child Health Questionnaire (CHQ) and Health Utilities Index mark 3 (HUI3) at the start and after 3, 15 and 27 months of treatment. At the same time points the following JIA disease activity variables were collected; physician's global assessment through the visual analogue scale (VAS), number of active and limited joints and erythrocyte sedimentation rate. A statistical method linear mixed models was used to assess outcomes over time. RESULTS: During etanercept treatment both disease-specific and generic HRQoL outcomes improved dramatically. Significant improvements were shown after 3 months and these improvements continued at least up to 27 months of treatment. The disease-specific CHAQ, including VAS pain and wellbeing, showed a significant improvement in all domains. The generic health-profile measure CHQ improved for all the health concepts except for "family cohesion", which was normal. The generic preference-based HUI3 showed impairment and, subsequently, significant improvement in the more specific domains ("pain", "ambulatory", "dexterity"). In accordance disease activity variables also improved significantly over time. CONCLUSION: This study shows that the HRQoL of patients with refractory JIA can be substantially improved by the use of etanercept for all aspects impaired by JIA. Information on HRQoL is crucial to understand the complete impact of etanercept treatment on patients with JIA and their families.

Disease activity patterns in juvenile systemic lupus erythematosus and its relation to early aggressive treatment.

This study aimed to determine disease activity patterns in juvenile systemic lupus erythematosus (jSLE) and its relation to early treatment. All jSLE patients who visited the outpatient departments of three Dutch university hospitals for at least 6 months were included. Data were retrospectively collected from each patient visit and hospitalization. Patient characteristics, clinical and laboratory findings categorized in organ systems, flare rate, medication use and disease course were analysed. Included were 35 patients (female 77%; White 47%) with a total follow-up of 142 years. Median age at diagnosis was 12.8 years. Flare rate was 0.45/ patient-year. An organ system not earlier involved was affected in 34% of flares. Identifiable disease activity patterns were: chronic active (49%), relapse remitting (14%) and long quiescence (37%), with no significant difference in organ involvement at diagnosis. Positive anti-Sm and non-White ethnicity were significantly associated with a chronic active pattern. In 14 patients with severe symptoms at diagnosis, treatment with intravenous cyclophosphamide and/or biologics and/or intravenous methylprednisone in the first 6 months resulted in a long quiescence pattern in seven patients. In conclusion, distinct disease activity patterns are identifiable in children. Suppression of disease with early aggressive treatment may decrease the rate of progression.

Long-term follow-up on effectiveness and safety of etanercept in juvenile idiopathic arthritis: the Dutch national register.

OBJECTIVE: We undertook an observational study to obtain a complete overview of the long-term effectiveness and safety of etanercept in patients with different juvenile idiopathic arthritis (JIA) subtypes. METHODS: At baseline we collected patient and disease characteristics of all Dutch patients with JIA who started treatment with etanercept. Disease activity was evaluated (at start of the study, after 3 months and then yearly) according to the JIA core set of the American College of Rheumatology paediatric definition for 30, 50 and 70% improvement (ACR Pedi 30, 50 and 70). Use of etanercept and concomitant drugs was monitored. Adverse events were recorded. RESULTS: We included 146 patients with JIA with a median follow-up of 2.5 years per patient (range 0.3-7.3). JIA subtypes represented: 27% systemic, 8% polyarticular rheumatoid factor positive, 38% polyarticular rheumatoid factor negative, 19% oligoarticular extended, 3% enthesitis-related and 5% psoriatica. Most patients (77%) met the criteria of the ACR Pedi 30 in the first 3 months of treatment. For the majority of patients this improvement was sustained; 53 (36%) of all patients met the remission criteria. No other second-line agents were needed in 43 patients. Although patients with systemic JIA responded initially less to etanercept therapy than patients from other subtypes, those who did respond showed equal effectiveness in the long term. Serious adverse events rate was low (0.029 per patient year). CONCLUSIONS: Etanercept is effective and safe in JIA, even for a large proportion of the patients with systemic JIA. The greatest improvement occurred in the first 3 months of treatment, and was sustained for a long time in most patients (up to 75 months).

No radiographic wrist damage after treatment to target in recent-onset juvenile idiopathic arthritis.

BACKGROUND: To evaluate radiographic progression of patients with new-onset juvenile idiopathic arthritis (JIA) in response to an early, tightly-controlled, treatment-to-target. METHODS: Patients with JIA participating in the BeSt-for-Kids-study, randomized to 3 treatment strategy arms, were eligible if at least 1 conventional wrist-radiograph was available. Bone damage as reflected by carpal length was assessed using the Poznanski-score. The BoneXpert-method was used to determine the Bone Age (BA, > 5 years) and bone mineral density (BMD) of the wrist. These scores were evaluated over time and compared between the treatment arms and mean JADAS10-score using linear mixed models corrected for age and symptom duration. RESULTS: In 60 patients, 252 radiographs were analysed. Baseline age and symptom duration were different between the arms. No difference in comparison to the healthy reference population was found at baseline for the Poznanski-score (IQR varying from - 0,82; 0.68), nor for BA (varying from - 0.88 to 0.74). Baseline BMD was statistically significantly lower in arm 3 (initial treatment with etanercept and methotrexate) (- 1.48; - 0.68) compared to arm 1 (- 0.84; - 0.04) and arm 2 (- 0.93; 0.15). After treatment to target inactive disease, the Poznanski-scores and the BA remained clinically unchanged, while the BMD in arm 3 improved (p < 0.05 vs arm 1). CONCLUSIONS: Recent-onset JIA patients, treated-to-target aimed at inactive disease, showed no signs of radiographic wrist damage (Poznanski-score, BA or BMD) either at baseline or at follow-up, irrespective of treatment arm. A lower BMD at baseline in arm 3, initially treated with methotrexate and etanercept, improved significantly after treatment. TRIAL REGISTRATION: NTR, NL1504 (NTR1574). Registered 01-06-2009.

Development of a web-based register for the Dutch national study on biologicals in JIA: www.ABC-register.nl.

OBJECTIVES: Most clinical studies use paper case record forms (CRFs) to collect data. In the Dutch multi-centre observational study on biologicals we encountered several disadvantages of using the paper CRFs. These are delay in data collection, lack of overview in collected data and difficulties in obtaining up-to-date interim reports. Therefore, we wanted to create a more effective method of data collection compared with CRFs on paper in a multi-centre study. METHODS: We designed a web-based register with the intention to make it easy to use for participating physicians and at the same time accurate and up-to-date. Security demands were taken into account to secure the safety of the patient data. RESULTS: The web-based register was tested with data from 161 juvenile idiopathic arthritis patients from nine different centres. Internal validity was obtained and user-friendliness guaranteed. To secure the completeness of the data automatically generated e-mail alerts were implemented into the web-based register. More transparency of data was achieved by including the option to automatically generate interim reports of data in the web-based register. The safety was tested and approved. CONCLUSIONS: By digitalizing the CRF we achieved our aim to provide easy, rapid and safe access to the database and contributed to a new way of data collection. Although the web-based register was designed for the current multi-centre observational study, this type of instrument can also be applied to other types of studies. We expect that especially collaborative study groups will find it an efficient tool to collect data.

A comparison of three treatment strategies in recent onset non-systemic Juvenile Idiopathic Arthritis: initial 3-months results of the BeSt for Kids-study.

BACKGROUND: Combination therapy with prednisone or etanercept may induce earlier and/or more improvement in disease activity in Disease Modifying Anti Rheumatic Drug (DMARD) naïve non-systemic Juvenile Idiopathic Arthritis (JIA) patients. Here we present three months clinical outcome of initial treatments of the BeSt-for-Kids study. METHODS: Included patients were randomized to either: 1. initial DMARD-monotherapy (sulfasalazine (SSZ) or methotrexate (MTX)), 2. Initial MTX / prednisolone-bridging, 3. Initial combination MTX/etanercept. Percentage inactive disease, adjusted (a) ACR Pedi30, 50 and 70 and JADAS after 6 and 12 weeks of treatment (intention to treat analysis) and side effects are reported. RESULTS: 94 patients (67% girls, 32 (arm 1), 32 (arm 2) and 30 (arm 3) with median (InterQuartileRange) age of 9.1 (4.7-12.9) years were included. 38% were ANA positive, 10 had oligo-articular disease, 68 polyarticular JIA and 16 psoriatic arthritis. Baseline median (IQR) ACRpedi-scores: VAS physician 49 (40-58) mm, VAS patient 54 (37-70) mm, ESR 6.5 (2-14.8)mm/hr, active joint count 8 (5-12), limited joint count 3 (1-5), CHAQ score 0.88 (0.63-1.5). In arm 1, 17 started with MTX, 15 with SSZ. After 3 months, aACR Pedi 50 was reached by 10/32 (31%), 12/32(38%) and 16/30 (53%) (p = 0.19) and aACR Pedi 70 was reached by 8/32 (25%), 6/32(19%) and 14/30(47%) in arms 1-3 (p = 0.04). Toxicity was similar. Few serious adverse events were reported. CONCLUSION: After 3 months of treatment in a randomized trial, patients with recent-onset JIA achieved significantly more clinical improvement (aACRPedi70) on initial combination therapy with MTX / etanercept than on initial MTX or SSZ monotherapy. TRIAL REGISTRATION: NTR1574 . Registered 3 December 2008.

Interventions for cutaneous molluscum contagiosum.

BACKGROUND: Molluscum contagiosum is a common skin infection, caused by a virus, which will usually resolve within months in people with a normal immune system. Many treatments have been promoted for molluscum contagiosum but a clear evidence base supporting them is lacking. OBJECTIVES: To assess the effects of management strategies (including waiting for natural resolution) for cutaneous, non-genital molluscum contagiosum in healthy people. SEARCH STRATEGY: We searched the Skin Group Specialised Register (March 2004), the Cochrane Central Register of Controlled Trials (2004, Issue 2), MEDLINE (from 1966 to March 2004), EMBASE (from 1980 to March 2004) and LILACS (from 1982 to March 2004) databases. We also searched reference lists and contacted pharmaceutical companies and experts in the field. SELECTION CRITERIA: Randomised controlled trials for treatment of molluscum contagiosum were investigated. Trials on sexually transmitted molluscum contagiosum and in people with lowered immunity (including those with HIV infection) were excluded. DATA COLLECTION AND ANALYSIS: Study selection and assessment of methodological quality were carried out by two independent authors. As similar comparisons between two interventions were not made in more than one study, statistical pooling was not performed. MAIN RESULTS: Five studies, with a total number of 137 participants, examined the effects of topical (three studies), systemic and homoeopathic interventions (one study each). Limited evidence was found for sodium nitrite co-applied with salicylic acid compared to salicylic acid alone (risk ratio (RR) 3.50, 95% confidence interval (CI) 1.23 to 9.92). No statistically significant differences were found for topical povidone iodine plus salicylic acid compared to povidone iodine alone (RR of cure 1.67, 95% CI 0.81 to 3.41) or compared to salicylic acid alone. Also no statistically significant differences were found for potassium hydroxide compared to placebo; systemic treatment with cimetidine versus placebo or systemic treatment with calcarea carbonica, a homoeopathic drug, versus placebo (RR 5.57, 95% CI 0.93 to 33.54). Study limitations included no blinding (two studies), many dropouts (three studies) and no intention-to-treat analysis (two studies); small study sizes may have led to important differences being missed. None of the evaluated treatment options were associated with serious adverse effects. AUTHORS' CONCLUSIONS: No single intervention has been shown to be convincingly effective in treating molluscum contagiosum.

[Two children with skin rash and muscle weakness: juvenile dermatomyositis]. / Twee kinderen met huiduitslag en spierzwakte: juveniele dermatomyositis.

Two girls aged 1.5 and 3 years, presented with a skin rash, loss of proximal muscle power and malaise. The younger girl recovered quickly after a short course of corticosteroids but the elder girl proved more difficult to treat effectively with corticosteroids, methotrexate, ciclosporin, intravenous immunoglobulins and hydroxychloroquine. This combination of symptoms should make one consider the diagnosis of juvenile dermatomyositis (JDM). To make the diagnosis of JDM, a characteristic skin rash, proximal muscle weakness, elevated muscle enzymes and, possibly, an abnormal EMG or muscle biopsy should be present. Treatment consists, of steroids and, if necessary, immunosuppressive agents or intravenous immunoglobulins. If cutaneous lesions are serious or persistent, hydroxychloroquine may also be prescribed.

Interventions for impetigo.

BACKGROUND: Impetigo is a common superficial bacterial skin infection, most frequently encountered in children. There is no standard therapy and guidelines for treatment differ widely. Treatment options include many different oral and topical antibiotics as well as disinfectants. OBJECTIVES: To assess the effects of treatments for impetigo, including waiting for natural resolution. SEARCH STRATEGY: We searched the Skin Group Specialised Trials Register (March 2002), Cochrane Central Register of Controlled Trials (CENTRAL, Issue 1 2002), the National Research Register (2002), MEDLINE (from 1966 to January 2003), EMBASE (from 1980 to March 2000) and LILACS (November 2001). We handsearched the Yearbook of Dermatology (1938-1966), the Yearbook of Drug Therapy (1949-1966), used reference lists of articles and contacted pharmaceutical companies. SELECTION CRITERIA: Randomised controlled trials of treatments for non-bullous and bullous, primary and secondary impetigo. DATA COLLECTION AND ANALYSIS: All steps in data collection were done by two independent reviewers. We performed quality assessments and data collection in two separate stages. MAIN RESULTS: We included 57 trials including 3533 participants in total which studied 20 different oral and 18 different topical treatments. CURE OR IMPROVEMENT: Topical antibiotics showed better cure rates than placebo (pooled odds ratio (OR) 6.49, 95% confidence interval (CI) 3.93 to 10.73), and no topical antibiotic was superior (pooled OR of mupirocin versus fusidic acid 1.76, 95% CI 0.69 to 2.16). Topical mupirocin was superior to oral erythromycin (pooled OR 1.22, 95% CI 1.05 to 2.97). In most other comparisons, topical and oral antibiotics did not show significantly different cure rates, nor did most trials comparing oral antibiotics. Penicillin was inferior to erythromycin and cloxacillin and there is little evidence that using disinfectant solutions improves impetigo. SIDE EFFECTS: The reported number of side effects was low. Oral antibiotic treatment caused more side effects, especially gastrointestinal ones, than topical treatment. REVIEWERS' CONCLUSIONS: Data on the natural course of impetigo are lacking. Placebo controlled trials are scarce. There is little evidence about the value of disinfecting measures. There is good evidence that topical mupirocin and topical fusidic acid are equally, or more effective than oral treatment for people with limited disease. It is unclear if oral antibiotics are superior to topical antibiotics for people with extensive impetigo. Fusidic acid and mupirocin are of similar efficacy. Penicillin was not as effective as most other antibiotics. Resistance patterns against antibiotics change and should be taken into account in the choice of therapy.

Bone mineral density and body composition and influencing factors in children with rheumatic diseases treated with corticosteroids.

In rheumatic diseases the use of corticosteroids (CS), immobility, or the disease itself, may cause osteoporosis and growth retardation. We evaluated bone mineral density (BMD) by dual energy X-ray absorptiometry (DXA), growth and physical activity in 27 children with rheumatic disease, all treated with high dose CS for at least one year, in a cross-sectional design. BMD SDS was significantly lower than zero: -1.02 for total body and -1.49 for lumbar spine measurement. SDS for fat mass was higher than zero. In multiple regression analysis the Child Health Assessment Questionnaire score significantly correlated with BMD of the lumbar spine. There was no significant correlation with cumulative dose or duration of CS treatment. Height SDS decreased during treatment to -1.57 (p <0.001 compared to 0). In conclusion, BMD and body composition in children with rheumatic disease treated with CS are influenced by physical activity, as well as corticosteroid treatment and type of rheumatic disease.

[A flow diagram for the diagnosis of acute, non-traumatic joint complaints during childhood, retrospectively applied to 115 children]. / Een stroomdiagram voor de diagnostiek van acute, niet-traumatische gewrichtsklachten op de kinderleeftijd, retrospectief toegepast bij 115 kinderen.

Acute, non-traumatic joint complaints during childhood can be caused by conditions which require a quick and adequate recognition and treatment as well as by conditions in which an expectant policy can be pursued. On the basis of certain data from the anamnesis, supplemented with findings from the physical examination it is often possible to arrive at a (probable) diagnosis. An algorithm was designed, the differential steps of which were: fever, C-reactive protein titre, involvement of the hip joint, the presence of extra-articular manifestations and the results of a full blood count, erythrocyte sedimentation rate and imaging techniques. When this algorithm was retrospectively applied to the disease data of 115 children with acute, non-traumatic joint complaints, for whom the diagnosis in the status was taken as the gold standard, the correct diagnosis was established for every single child: for 98 (85.2%) by the shortest route and for 17 (14.8%) indirectly. In the case of 4 children, use of this algorithm would have led to unnecessary laboratory investigations and/or treatment. None of the diseases requiring immediate treatment were missed.

Clinical course and prognostic value of disease activity in the first two years in different subtypes of juvenile idiopathic arthritis.

OBJECTIVE: Juvenile idiopathic arthritis (JIA) is a heterogeneous disease involving chronic arthritis. The clinical course is characterized by a fluctuating pattern of active and inactive disease. We have described in detail the clinical course in different JIA subtypes during the first 2 years after diagnosis and studied its relationship to disease activity in the following years. METHODS: Detailed clinical data on different parameters describing the disease activity in sequential time periods covering the first 2 years after diagnosis were retrieved from the charts of 311 patients with JIA and compared between subtypes. In a cohort of 146 patients, the relation of these different clinical variables to the course of disease in the following 3 years was evaluated. RESULTS: The percentage of time with active disease in the first 2 years differed significantly between subtypes. In all subtypes, a broad spectrum of activity was observed. The time with active disease in the first 2 years was the most significant factor associated with the duration of active disease in the following years. CONCLUSION: Different percentages of time with active disease have been observed between JIA subtypes in the first 2 years. The cumulative duration of activity varied widely within each subtype. Regarding the prognosis of the individual patient, the clinical course in the first 2 years appears to be predictive of the clinical course in the following years. Patients that have less time with active disease in the first 2 years are not likely to develop an unremitting clinical course later on.

Incidence of temporomandibular involvement in juvenile idiopathic arthritis.

OBJECTIVE: Temporomandibular joint (TMJ) involvement is a frequent feature in cross-sectional prevalence studies among juvenile idiopathic arthritis (JIA) patients. The cross-sectional design makes it almost impossible to study the incidence. Follow-up data on TMJ involvement are sparse. In this study patients were reviewed with an interval of a minimum of 1 year and a maximum of 2 years to study the yearly incidence of TMJ involvement and to obtain follow-up data on TMJ involvement and orthopantomogram (OPT) alterations. METHODS: Children with JIA from a previous study on TMJ involvement were included. OPTs were scored according to Rohlin's grading system (grade 0-5). A paediatric rheumatologist measured the level of disease activity during the interval. RESULTS: Eighty-nine of the 97 patients were included in this study with a mean follow-up of 14 months. The yearly incidence of TMJ involvement was 7.1% in patients with JIA. Improvement on the OPT was seen in 27 patients (66%), and 19 of these patients no longer showed any signs of TMJ involvement. Worsening on the OPT was seen in four patients (10%). Disease activity was significantly lower in the improved patients than in the patients with worsening. CONCLUSION: Condylar lesions due to arthritis can improve over time, indicating a regenerative capacity of the mandibular condyle. As condylar improvement seems to be associated with low disease activity, it is important to consider the TMJ when deciding on a therapeutic regimen.

Reported incidence and treatment of dermatophytosis in children in general practice: a comparison between 1987 and 2001.

INTRODUCTION: Dermatophytosis is a common skin infection in children. Although the epidemiology is relatively unknown it is becoming a major health problem in some countries. We determine the incidence and management of dermatophytosis in Dutch general practice in 1987 and 2001. METHODS: We used data of all children aged 0-17 years derived from two national surveys performed in Dutch general practice in 1987 and 2001 respectively. All diagnoses, prescriptions and referrals were registered over a 12 months period by the participating general practitioners (GPs), 161 and 195 respectively. Data were stratified for socio-demographic characteristics. RESULTS: Compared to 1987, in 2001 the total reported incidence rate of dermatophytosis in children in general practice increased from 20.8 [95%CI 18.9-22.8] to 24.6 [95%CI 23.5-25.7] per 1,000 person years. Infants (<1 year), girls, children in rural areas and children of non-western immigrants more often consulted the GP for dermatophytosis in 2001. In both surveys GPs treated the majority of children with dermatophytosis with topical drugs, especially with azoles. CONCLUSIONS: The reported incidence rate of dermatophytosis in children in general practice increased; however it is unclear whether this is a consequence of an increasing prevalence in the population or a changing help seeking behaviour. GPs generally follow the national guideline for the treatment of dermatophytosis in children.

Facioskeletal changes in children with juvenile idiopathic arthritis.

OBJECTIVE: To investigate the facioskeletal morphology in patients with juvenile idiopathic arthritis (JIA) with and without temporomandibular joint (TMJ) involvement. METHODS: Eighty five patients were included. TMJ involvement was defined by orthopantomogram alterations. Lateral cephalograms were used to determine linear and angular measurements and occlusion. RESULTS: Patients regardless of their TMJ status had a 67% chance for retrognathia and a 52% chance for posterior rotation of the mandible and, respectively, 82% and 58% if TMJ involvement were present. Changes were not uniformly distributed among the different subtypes. CONCLUSION: Patients with JIA have an altered facial morphology, especially in the presence of TMJ involvement.

Impetigo: incidence and treatment in Dutch general practice in 1987 and 2001--results from two national surveys.

BACKGROUND: Impetigo is a common skin infection in children. The epidemiology is relatively unknown, and the choice of treatment is subject to debate. OBJECTIVE: The objective of our study was to determine the incidence and treatment of impetigo in Dutch general practice, and to assess trends between 1987 and 2001. METHODS: We used data from the first (1987) and second (2001) Dutch national surveys of general practice. All diagnoses, prescriptions and referrals were registered by the participating general practitioners (GPs), 161 and 195, respectively. RESULTS: The incidence rate of impetigo increased from 16.5 (1987) to 20.6 (2001) per 1000 person years under 18 years old (P < 0.01). In both years, the incidence was significantly higher in summer, in rural areas and in the southern region of the Netherlands, compared with winter, urban areas and northern region, respectively. Socioeconomic status was not associated with the incidence rate. From 1987 to 2001, there was a trend towards treatment with a topical antibiotic (from 43% to 64%), especially fusidic acid cream and mupirocin cream. Treatment with oral antibiotics (from 31% to 14%) and antiseptics (from 11% to 3%) was prescribed less often. CONCLUSIONS: We have shown an increased incidence of impetigo in the past decade, which may be the result of an increased tendency to seek help, or increased antibiotic resistance and virulence of Staphylococcus aureus. Further microbiological research on the marked regional difference in incidence may contribute to understanding the factors that determine the spread of impetigo. Trends in prescribing for impetigo generally follow evidence-based knowledge on the effectiveness of different therapies, rather than the national practice guideline.

Growth hormone treatment in children with rheumatic disease, corticosteroid induced growth retardation, and osteopenia.

BACKGROUND: In children with severe rheumatic disease (RD), treatment with corticosteroids (CS) is frequently needed and growth retardation and osteopenia may develop. A beneficial effect of human growth hormone (hGH) has been reported but mostly in trials without a control group. AIMS: To study the effect of hGH on growth, bone mineral density (BMD), and body composition, taking the disease activity and CS use into account. METHODS: Randomised controlled trial on 17 prepubertal RD patients with growth retardation and/or decreased BMD. The hGH group (n = 10) received treatment with hGH 4 IU/m2/day (approximately 0.045 mg/kg/day) during two years. The controls (n = 7) received no GH treatment. RESULTS: During the two year study period the disease activity, and use of CS and methotrexate (MTX) did not differ between the groups. There was a significant mean increase in height standard deviation score (HSDS) in the hGH group (0.42+/-0.16 SDS) and a non-significant decrease in the controls (-0.18+/-0.11 SDS). Change in BMD did not differ significantly between the groups, although the increase in BMD for lumbar spine within the hGH group was significant. Lean body mass improved significantly in the hGH group compared to controls (0.64+/-0.19 SDS versus -0.20+/-0.17 SDS), while the decrease in percentage fat was not significant. CONCLUSIONS: There was a significant effect of hGH on growth and lean body mass, but a longer duration of treatment might be necessary to evaluate the effect of hGH on BMD.

Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study.

OBJECTIVE: Juvenile localized scleroderma (JLS) includes a number of conditions often grouped together. With the long-term goal of developing uniform classification criteria, we studied the epidemiological, clinical and immunological features of children with JLS followed by paediatric rheumatology and dermatology centres. METHODS: A large, multicentre, multinational study was conducted by collecting information on the demographics, family history, triggering environmental factors, clinical and laboratory features, and treatment of patients with JLS. RESULTS: Seven hundred and fifty patients with JLS from 70 centres were enrolled into the study. The disease duration at diagnosis was 18 months. Linear scleroderma (LS) was the most frequent subtype (65%), followed by plaque morphea (PM) (26%), generalized morphea (GM) (7%) and deep morphea (DM) (2%). As many as 15% of patients had a mixed subtype. Ninety-one patients (12%) had a positive family history for rheumatic or autoimmune diseases; 100 (13.3%) reported environmental events as possible trigger. ANA was positive in 42.3% of the patients, with a higher prevalence in the LS-DM subtype than in the PM-GM subtype. Scl70 was detected in the sera of 3% of the patients, anticentromere antibody in 2%, anti-double-stranded DNA in 4%, anti-cardiolipin antibody in 13% and rheumatoid factor in 16%. Methotrexate was the drug most frequently used, especially during the last 5 yr. CONCLUSION: This study represents the largest collection of patients with JLS ever reported. The insidious onset of the disease, the delay in diagnosis, the recognition of mixed subtype and the better definition of the other subtypes should influence our efforts in educating trainees and practitioners and help in developing a comprehensive classification system for this syndrome.