Idiopathic limb edema in children with chronic arthritis: a multicenter report of 12 cases.

Lymphedema, a well known extraarticular manifestation of rheumatoid arthritis, has been rarely described in children with idiopathic chronic arthritis. We describe 12 cases of lymphedema and idiopathic arthritis of childhood seen at 4 different pediatric rheumatology centers. Eight patients were girls, 4 boys; the age at appearance of lymphedema ranged from 2.3 to 17 years. In all patients except one, lymphedema was localized to the lower limbs. The outcome of lymphedema was variable, but not always related to the arthritis course, and was mostly independent of any specific therapy. Lymphography was performed in only one patient, and revealed lack of lymphatic drainage in the affected leg. We conclude that the association of lymphedema and idiopathic arthritis of childhood is not rare; this association is unlikely to be coincidental, even though the pathogenetic mechanisms are currently not well understood.

Juvenile chronic arthritis and coeliac disease in The Netherlands.

OBJECTIVE: It has been suggested that juvenile chronic arthritis (JCA) is associated with coeliac disease in a frequency of 0.4-2%. In order to investigate the frequency of coeliac disease in cases of JCA and the possibility of underdiagnosis in our area, we screened 62 children with JCA (mean age 9.8 +/- 3.5 year) for coeliac disease. METHODS: All children were screened for coeliac disease by measuring the IgA-class of antigliadin, antireticulin and antiendomysium antibodies in serum and by measuring intestinal permeability by a sugar absorption test using lactulose and mannitol. In cases of at least one positive test, a small-bowel biopsy for diagnosis of coeliac disease was offered. RESULTS: Of the 62 children with JCA, 8 had an abnormal screening result and were suspected of having coeliac disease. In four of the five children in whom a small-bowel biopsy was performed, the intestinal mucosa was normal and in one child villous atrophy characteristic of coeliac disease was found. Therefore, the prevalence of coeliac disease in our study group was 1.5%, which is in agreement with the literature. CONCLUSION: These findings indicate no underdiagnosis of coeliac disease in JCA in our area.

Screening for coeliac disease in Dutch children with associated diseases.

Our objective was to assess the frequency of coeliac disease in children with associated disorders in the province of "Zuid-Holland". The Netherlands. We therefore screened 115 children with Down's syndrome, 62 children with juvenile rheumatoid arthritis (JRA) and 46 children with diabetes mellitus for CD using the IgA-class of antigliadin, antiendomysium and antireticulin antibodies in serum, and a functional sugar absorption test. The antiendomysium antibody test was the screening test that performed the best. Every patient who has at least one positive test underwent a jejunal biopsy for the diagnosis of CD. No association could be demonstrated between CD and diabetes mellitus. The frequency of CD in Down's syndrome was 7.0%, which is much higher than that found from screening the general population. CD was found in one child with JRA (1.5%), who also had Down's syndrome. We recommend screening for CD in all persons with Down's syndrome using at least the antiendomysium antibody test.

Teenager with an irritable hip, anaemia and malaise.

We have described a boy with a juvenile spondyloarthropathy who also showed inflammatory changes in the gut. Gastrointestinal symptoms initially indicated the presence of an inflammatory bowel disease, but this was excluded histopathologically.

Increased prevalence of spondylarthropathies in parents of children with pauciarticular juvenile chronic arthritis, type 1.

Using a standard questionnaire, parents of 70 children with type 1 pauciarticular juvenile chronic arthritis were asked about inflammatory back-pain. When the answer was positive, the parents were seen by a rheumatologist who used the preliminary criteria of the European Spondylarthropathy Study Group to decide whether the parent had spondylarthropathy or not. A diagnosis of spondylarthropathy was established in 6 out of 138 parents (4%). This is considerably higher than the expected prevalence of spondylarthropathy, which should not exceed 0.25%. This finding sheds a new light on pauciarticular JCA, type 1, since no adult counterpart has as yet been described.

Disease patterns in early onset pauciarticular juvenile chronic arthritis.

A group of 76 children with early onset pauciarticular juvenile chronic arthritis (JCA) was studied in order to establish different disease patterns and to try and identify parameters associated with an unfavourable outcome. An intermittent pattern of disease was found in 60 children (79%). Of the remaining 16 patients continuous persistent pauciarticular disease activity was present in 7 (9.2%) and extended pauciarticular in 9 children (11.8%). An extended pauciarticular pattern was seen predominantly in children with continuous disease activity. It appeared to be impossible to predict the course of the disease on the basis of clinical parameters. The frequency of complications, such as local growth disturbances or psychosocial problems and of chronic anterior uveitis resulting in visual handicap correlated with continuous disease activity. The extended pauciarticular pattern, resulting in polyarthritis resembled seronegative polyarticular JCA, underlining previous reports that the joint pattern during the course of disease may be more important than joint pattern at onset of disease.

Antibodies to a 15 kD nuclear antigen in patients with juvenile chronic arthritis and uveitis.

Young girls with a pauciarticular onset of juvenile chronic arthritis and circulating antinuclear antibodies are at risk for chronic uveitis. The actual nuclear antigen for these antinuclear antibodies has not been defined. Conventional laboratory techniques, such as counter immunoelectrophoresis, have shown that antibodies to well defined "extractable nuclear antigens" (eg, RNP, Sm, SS-A, and SS-B) are not present in patients with juvenile chronic arthritis. Therefore, other, previously unknown nuclear antigens may be involved. Sera of 64 patients with juvenile chronic arthritis, including 22 patients with chronic anterior uveitis, were studied using the immunoblotting technique to characterize the nuclear antigens. Antinuclear antibodies were present in 12 (55%) of the 22 patients with uveitis, and only in six (14%) of the 42 patients without chronic anterior uveitis. With the immunoblotting technique, antibodies to a 15 kD nuclear antigen were found in 10 (45%) of the 22 patients with chronic anterior uveitis, whereas only two (5%) of the 42 patients without chronic anterior uveitis showed these antibodies (P less than 0.001). Only clearly visible and reproducible lines in the immunoblotting patterns were studied. This may provide a diagnostic tool for the early detection of uveitis and means for further pathogenetic studies.

[Pauciarticular juvenile chronic arthritis]. / Pauciarticulaire juveniele chronische arthritis.

On basis of clinical and immunogenetic factors most children with pauciarticular juvenile chronic arthritis can be included in one of the subtypes: type 1 and type 2 pauciarticular JCA. Type 1 occurs in young children, mainly girls, with involvement of knees, ankles or elbows. In the majority of children antinuclear antibodies can be detected. The presence of these autoantibodies is associated with chronic anterior uveitis. Type 2 or the juvenile spondylarthropathies include morbus Bechterew, the reactive arthritides and arthritis associated with psoriasis and inflammatory bowel diseases. Large joints of the lower extremities are involved, back pain is unusual at onset, but enthesitis is frequently present. There is a strong association with HLA-B27. Treatment of both subsets consists of non-steroidal anti-inflammatory drugs, application of intra-articular steroids, physio- and hydrotherapy and splinting. In children with a polyarticular course of type 1, or a prolonged course of type 2 disease modifying drugs are often needed.

[Drug treatment of juvenile chronic arthritis]. / Medicamenteuze behandeling van juveniele chronische arthritis.

Medical therapy is an important component of the management of children with juvenile chronic arthritis, which should involve a multidisciplinary approach. NSAID's are the most important drugs in the treatment of JCA. Apart from salicylates a wide variety of NSAID's is now available, which have proven to be safe and effective for children with JCA. Medical treatment with NSAID's alone is satisfactory in most cases, especially the mono- and oligoarticular subtypes. Intra-articular corticosteroid injections in mono- and pauciarticular JCA have show to be a safe and highly efficacious form of treatment. Disease modifying antirheumatic drugs (DMARD's) are indicated for progressive polyarthritis unresponsive to NSAID's. More controlled clinical trials are needed to clarify the place of these drugs in the treatment of JCA. For the seropositive erosive polyarthritis institution of DMARD's early in the disease is generally accepted. The first experiences with methotrexate are promising for treatment of the more severe cases.

Intra-articular steroids in pauciarticular juvenile chronic arthritis, type 1.

The use of intra-articular steroids in one or both knees was evaluated in 21 children with type 1 pauciarticular juvenile chronic arthritis (JCA). The beneficial effect of the injection was noted within 3 days with no significant adverse reactions. Remission exceeding 6 months was seen in 70% of the knees and the arthritis remained inactive during the follow up period in 37%. The beneficial effect of the injection did not correlate with sex, age of onset or the presence of antinuclear antibodies or HLA-B27 antigen and there was no relationship with the size of involved joints at onset, the ESR at onset, or the presence of uveitis. Intra-articular corticosteroids in this type of JCA may provide prompt relief of swelling and pain and reduce the need for other forms of therapy. Remission was long lasting in the majority of the children.

Toxicity of sulfasalazine in systemic juvenile chronic arthritis.

The use of sulfasalazine as a long acting anti-rheumatic drug in patients with systemic JCA was studied in an open trial. Severe toxic side-effects in three of the four patients entered required discontinuation of the trial. These adverse effects included high fevers and a generalised exanthem. The symptoms were considered to represent an allergic reaction, most likely to the sulfa component of sulfasalazine. After resolution of these episodes the joint disease was suppressed for several weeks in these three children.