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.

Design and validation of a new scale to assess the functional ability in children with juvenile idiopathic arthritis (JIA).

OBJECTIVE: The assessment of the functional ability is one of the items of the core set to define improvements in patients with JIA, CHAQ being the most used scale already validated in 32 countries. The aim of this study was to design and validate a new scale named CAPFUN (capacidad funcional = functional ability) to assess functional ability in children with JIA. METHODS: This scale includes 20 items, 8 of upper limbs, 8 of lower limbs, 3 combined, and 1 of cervical spine, developed in two steps according with OMERACT. Each item is scored: 0 when it is impossible to be performed, 1 when it is performed incompletely or with difficulties, and 2 when it is well performed. Seventy three patients with JIA according to ILAR criteria were assessed: 25 boys (34.2%) and 48 girls (65.8%) whose aver-age age was 12.8 years (95% CI 11.8 - 13.8) and the time from disease onset was 5.02 years (95% CI 3.9 - 6.1). For validation purposes, it was applied to 91 healthy children and adolescents. In every patient, correlation with active joints count and functional class according to Steinbrocker was assessed and with CHAQ in 31 patients in this series. RESULTS: The CAPFUN index obtained in all healthy children was 2. Patients' media CAPFUN index was 1.54 (95%CI 1.38 - 1.68). The CAPFUN index for Steinbrocker's class I was 1.84 +/- 1.8; for class II 1.60 +/- 1.5 and for class III 0.91 +/- 1 (F 24.1 p < 0.001). CAPFUN showed significant correlation with CHAQ (Spearman coefficient -0.79 p < 0.001), with active joints count (Spearman coefficient -0.72 p < 0.001) and with Steinbrocker functional classes (Spearman coefficient -0.69 p < 0.001). This scale showed a good internal reliability (alpha coefficient equal to 0.94), its construct validity is demonstrated by its good correlation with Steinbrocker's scale and with CHAQ. CONCLUSION: CAPFUN is a new instrument in order to assess functional ability in children with JIA. This scale showed a good internal reliability. Construct validity is demonstrated by its high correlation with Steinbrocker's scale and with CHAQ. This study demonstrates the usefulness of CAPFUN for the assessment of functional ability in children with JIA.

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

We report herein the results of the cross-cultural adaptation and validation into the Argentinian language 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 independently from the underlying disease. The Argentinian CHAQ was already published and therefore it was revalidated while the Argentinian CHQ was derived from the European Spanish version by changing few words which use is different in the 2 countries. A total of 124 subjects were enrolled: 61 patients with JIA (29% systemic onset, 38% polyarticular onset, 7% extended oligoarticular subtype, and 26% persistent oligoarticular subtype) and 63 healthy children. The CHAQ clinically discriminated between healthy subjects and JIA patients, with the systemic onset, and polyarticular having a higher degree of disability, pain, and a lower overall well-being when compared to their healthy peers. Also the CHQ clinically discriminated between healthy subjects and JIA patients, with the systemic onset, polyarticular onset and extended oligoarticular subtypes having a lower physical and psychosocial well-being when compared to their healthy peers. In conclusion the Argentinian version of the CHAQ-CHQ is a reliable, and valid tool for the functional, physical and psychosocial assessment of children with JIA.

Favourable outcome in 135 children with juvenile systemic sclerosis: results of a multi-national survey.

OBJECTIVE: To increase the current knowledge of the outcome of juvenile systemic sclerosis (jSSc), which is currently limited. METHODS: In order to investigate the patient outcome and prognostic factors, starting October 1994, we distributed questionnaires to 324 paediatric rheumatology centres. RESULTS: Until 15 May 1998 responses from 46 centres were received, 34 of which returned completed questionnaires on a total of 135 patients. One hundred and twenty-two of the 135 patients were Caucasian, 100 were female. The mean age at disease onset was 8.8 yr (S.D. +/- 3.3 yr). The mean disease duration at the last follow-up was 5 yr(S.D. +/- 3.3 yr). At the last follow-up the disease was still active and required medication in 82 patients, 36 had inactive disease on medication, and 16 were in remission. Ninety per cent of the living patients were fully active in daily life at the last follow-up. Eight of the 135 patients had died. These patients had a median age at onset of the disease of 10.5 yr (range 6.7-15.8 yr). The median disease duration until death was 2 yr (range 1-8 yr). The causes of death were heart failure (five), renal failure (one), sepsis (one) and in one case the cause was not defined. The 1 yr survival rate was 99%, the 2 yr was 97% and the 4 yr was 95%. CONCLUSIONS: At a mean follow-up of 5 yr, the current results show a favourable outcome in most patients with childhood onset jSSc and a significantly better survival than in the adult SSc patients.

Chondrosarcoma of the proximal phalanx.

An 87-year-old male presented with a painless, large mass on the dorsum of the left foot. He reported that the mass had first appeared 10 years ago and now had become so large that he could no longer tie his shoe. The mass originated from the proximal aspect of the second digit, encompassing the second web space and distal one third of the second and third metatarsals. Surgical excision of the mass was performed and pathologic diagnosis of the specimen confirmed a grade 1 chondrosarcoma. As expected with a lower grade chondrosarcoma, the patient did not have metastasis and fully recovered. While the occurrence of chondrosarcoma is not uncommon, it rarely affects the foot. This appears to be the third case of chondrosarcoma appearing in the proximal phalanx second digit of the foot.

Soluble intercellular adhesion molecule-1 in paediatric connective tissue diseases.

The intercellular adhesion molecule-1 (ICAM-1) is a cytokine-induced glycoprotein involved in the recruitment of cells into tissues undergoing inflammatory responses. The aim of this study was to compare the levels of soluble ICAM-1 (s-ICAM-1) in children with juvenile chronic arthritis (JCA) and systemic lupus erythematosus (SLE) and to evaluate the usefulness of this molecule as marker of disease activity. Levels of s-ICAM-1 were measured in sera using a monoclonal antibody sandwich enzyme-linked immunoassay. Serum levels (mean+/-SD) of s-ICAM-1 in 37 children with JCA, 18 patients suffering from SLE and 25 healthy controls were 609+/-184, 513+/-139 and 210+/-95 ng/ml, respectively. A significant difference could be demonstrated between the levels of s-ICAM-1 in sera from each disease, as a group, and those of healthy controls. Higher levels of s-ICAM-1 were recorded in JCA patients with systemic features and patients who had polyarthritis than in children who were pauciarticular. A positive correlation was observed between s-ICAM-1 levels and disease activity score in SLE patients. Moreover, s-ICAM-1 levels closely followed clinical conditions in five children with SLE during follow-up. The data show that s-ICAM-1 levels are increased in children suffering from connective tissue diseases and reflect disease status or activity, suggesting the usefulness of this molecule in the follow-up of these diseases.

Vasculitis, Kawasaki disease, and pseudovasculitis.

Vasculitis is the inflammation and necrosis of vessel wall and may be a primary disease or secondary to another condition. At present, there is not an accurate classification; rather, classification depends on the changing nomenclature of these conditions and it is difficult to categorize some syndromes because they involve several vessel sizes. The most interesting type of vasculitis for the pediatrician is Kawasaki disease. New developments in this field are showing a relationship between this disease and superantigens. Some patients are resistant to high doses of intravenous gammaglobulin; in such cases, when some risk factors are present, some authors suggest the use of steroid pulse therapy. Inflammation of vessel walls results in occlusion of the lumen with necrosis; many other diseases have similar findings, such as infective endocarditis, Sneddon's syndrome, and others discussed here that should be included in the differential diagnosis.

Juvenile onset Reiter's syndrome. A retrospective study of 26 patients.

Clinical, laboratory and radiological findings were evaluated in 26 children with Reiter's syndrome, all of whom met the 1982 diagnostic criteria of A. Calin. Twenty-two of the patients (85%) were male and 4 were female (15%); the mean age at onset was 10.5 years (range 4-15.5 yrs). Mean follow-up time was 28.6 months. Diarrhea prior to onset was observed in 18 cases (69%), but there was no report of venereal disease. The full classic triad was initially observed in only 9 patients (35%), urethritis alone in 6 (23%) and conjunctivitis alone in 4 (15%). Arthritis was present in all patients, with the lower limb joints involved in 25 cases (96%); the pattern was pauciarticular in 18 (69%), polyarticular in 7 (27%) and monoarticular in one (4%). There was complete remission in 15 out of the 26 patients (58%), while a sustained and fluctuating course was seen in 7 (27%) and 3 (11.5%) patients, respectively; a single patient showed a remitting course. Balanitis was present in 11 out of the 22 male (50%) cases. Twelve out of 18 patients tested (67%) proved to be HLA B27 positive and there was radiological evidence of sacroiliitis in 5 out of 24 patients (21%). Reiter's syndrome should be included in the differential diagnosis of the arthritic child. As a rule, the course of joint involvement is remittent and sequelae affecting functional capacity are indeed exceptional.

Triamcinolone into the knee joint in juvenile chronic arthritis.

Sixty children aged less than 10 years with pauci-articular onset juvenile chronic arthritis and knee joint involvement received local injections with triamcinolone hexacetonide into the knee (total 83 knees). One year after injection, 77% of knees were excellent with no soft tissue swelling, no deformity and full range of movement, or good with slight swelling or deformity, but functioning well. Eleven knees which had relapsed within one year improved after a second injection and 2 after a third. Complications were limited to small areas of subcutaneous tissue atrophy in 2 patients. Intra-articular injection of long-acting steroids is a useful means of controlling disease activity in the knee joints of young children with pauci-articular juvenile chronic arthritis.

Piroxicam in juvenile rheumatoid arthritis.

There is little consistency in drug treatment of children with juvenile rheumatoid arthritis, in part because there are few controlled studies of the use of nonsteroidal anti-inflammatory drugs (NSAIDs) in this disease. To determine the safety and efficacy of piroxicam in children with juvenile rheumatoid arthritis, 26 patients ranging in age from three to 25 years were randomly assigned to treatment with piroxicam or naproxen. The number of painful and swollen joints decreased significantly (p less than 0.05) in the piroxicam group. The overall assessment of the investigators was that 67% of the patients in the piroxicam group showed clinical improvement, in contrast to 38% of the naproxen-treated patients. Side effects in the piroxicam group were mild and transient, and no patient was removed from the study because of side effects. The recurrence of a cutaneous rash necessitated the removal of one naproxen-treated patient from the study. Although the results should be interpreted with caution because of the small sample size, piroxicam appears to be more effective and better tolerated than naproxen in this study.

Auranofin in juvenile rheumatoid arthritis. An open label, non-controlled study.

An open label, non-controlled trial of six-month duration was designed to determine the safety and efficacy of auranofin in the treatment of 13 children with polyarticular JRA. Adverse reactions were observed in 5 of the 13 patients (38%) but only in one was it serious enough to discontinue treatment. None of the patients developed diarrhea or hematologic abnormalities. Therapeutic response was evaluated in the 11 patients who completed the six-month treatment. According to the final overall assessment 9 of the 11 children had improved, one remained unchanged and one worsened. After four months of treatment serum gold levels in 11 patients ranged between 28 and 59 micrograms/dl, with a mean value of 34 micrograms/dl. There was no correlation between serum gold levels and the frequency and severity of side effects.

Enfermedad de Kawasaki. Comunicacion de un caso. / Kawasaki's disease. Report of a case

Se presenta un caso de enfermedad de Kawasaki en una nina de 13 meses de edad. Clinicamente presentaba exantema, adenopatia unilateral cervical, hipertemia que no remitia con antipireticos, lesiones en labios y mucosas, inyeccion conjuntival, artritis y artralgias, eritema de palmas y plantas, descamacion periungueal, estrias longitudinales en las unas, trombocitos y eritrosedimentacion elevada.En nuestro conocimiento este seria el cuarto caso publicado en nuestro medio y el primero en la ciudad de Buenos Aires

Enfermedad de Kawasaki. Comunicacion de un caso. / Kawasakis disease. Report of a case

Se presenta un caso de enfermedad de Kawasaki en una nina de 13 meses de edad. Clinicamente presentaba exantema, adenopatia unilateral cervical, hipertemia que no remitia con antipireticos, lesiones en labios y mucosas, inyeccion conjuntival, artritis y artralgias, eritema de palmas y plantas, descamacion periungueal, estrias longitudinales en las unas, trombocitos y eritrosedimentacion elevada.En nuestro conocimiento este seria el cuarto caso publicado en nuestro medio y el primero en la ciudad de Buenos Aires

Pronostico funcional en artritis reumatoidea. / Functional prognosis in rheumatoid arthritis

El prognostico de la capacidad funcional en artritis reumatoidea juvenil (ARJ) no ha podido ser establecido con seguridad y distintos son los factores que se han involucrado en el. En este trabajo se evaluo la capacidad funcional en 100 pacientes con ARJ, luego de un tiempo promedio de evolucion de 10 anos, que fueron seguidos en este Servicio. La capacidad funcional fue vinculada con el sexo, la edad de comienzo, la forma de comienzo, la forma de evolucion de la enfermedad y la presencia de factores reumatoideos. Luego de 10 anos de evolucion, 4 de cada 10 pacientes se encontraban en clases funcionales III y IV No se observo relacion entre el grado de capacidad funcional y el sexo, la edad de comienzo y la forma de comienzo de la enfermedad. Los pacientes con evolucion poliarticular y seropositivos para factores reumatoideos tuvieron peor pronostico funcional

Manifestaciones osteoarticulares en leucemias agudas de la infancia. / Osteoarticular manifestations in acute leukemia in childhood

En el periodo comprendido entre 1974 y 1981, de un total de 1050 ninos que consultaron por distintas manifestacions osteoarticulares en la Seccion de Estudio y Prevencion de Fiebre Reumatica del Hospital de Pediatria Pedro de Elizalde, 7 tuvieron un diagnostico final de leucosis.Cinco fueron leucemias linfociticas agudas (LLA) y 2, leucemias mieloides agudas (LMA). En todos los casos el diagnostico fue realizado por puncion aspirativa de medula osea. Artritis fue la primera manifestacion de enfermedad en 6 de 7 pacientes; en 5 asociada a fiebre y en 1 a adenomegalia. El compromiso fue poliarticular en 4 pacientes, oligoarticular en 2 y monoarticular en 1. El curso de estas manifesticones fue persistente en 5 pacientes y remitente en 2. Las grandes articulaciones fueron las mas afectadas frecuentemente. Estos casos demuestran que la leucemia aguda debe incluirse en el diagnostico difencial de las enfermedades reumaticas de la infancia. El mielograma es un estudio que debe ser considerado en todo nino afectado de patologia osea o articular cuyo diagnostico sea dudoso

Pronostico funcional en artritis reumatoidea. / Functional prognosis in rheumatoid arthritis

El prognostico de la capacidad funcional en artritis reumatoidea juvenil (ARJ) no ha podido ser establecido con seguridad y distintos son los factores que se han involucrado en el. En este trabajo se evaluo la capacidad funcional en 100 pacientes con ARJ, luego de un tiempo promedio de evolucion de 10 anos, que fueron seguidos en este Servicio. La capacidad funcional fue vinculada con el sexo, la edad de comienzo, la forma de comienzo, la forma de evolucion de la enfermedad y la presencia de factores reumatoideos. Luego de 10 anos de evolucion, 4 de cada 10 pacientes se encontraban en clases funcionales III y IV No se observo relacion entre el grado de capacidad funcional y el sexo, la edad de comienzo y la forma de comienzo de la enfermedad. Los pacientes con evolucion poliarticular y seropositivos para factores reumatoideos tuvieron peor pronostico funcional

Manifestaciones osteoarticulares en leucemias agudas de la infancia. / Osteoarticular manifestations in acute leukemia in childhood

En el periodo comprendido entre 1974 y 1981, de un total de 1050 ninos que consultaron por distintas manifestacions osteoarticulares en la Seccion de Estudio y Prevencion de Fiebre Reumatica del Hospital de Pediatria Pedro de Elizalde, 7 tuvieron un diagnostico final de leucosis.Cinco fueron leucemias linfociticas agudas (LLA) y 2, leucemias mieloides agudas (LMA). En todos los casos el diagnostico fue realizado por puncion aspirativa de medula osea. Artritis fue la primera manifestacion de enfermedad en 6 de 7 pacientes; en 5 asociada a fiebre y en 1 a adenomegalia. El compromiso fue poliarticular en 4 pacientes, oligoarticular en 2 y monoarticular en 1. El curso de estas manifesticones fue persistente en 5 pacientes y remitente en 2. Las grandes articulaciones fueron las mas afectadas frecuentemente. Estos casos demuestran que la leucemia aguda debe incluirse en el diagnostico difencial de las enfermedades reumaticas de la infancia. El mielograma es un estudio que debe ser considerado en todo nino afectado de patologia osea o articular cuyo diagnostico sea dudoso