Chorioretinitis as a complication of pauciarticular juvenile rheumatoid arthritis

A girl with pauciarticular juvenile rheumatoid arthritis developed bilateral uveitis complicated by cataract and glaucoma. Sequential fundus photography documented development of extensive choroidal scarring and retinal pigment epithelial atrophy in the left macula. Vision was not impaired. This case suggests uveitis in juvenile rheumatoid arthritis can be associated with chorioretinitis.     

Aortic incompetence in pauciarticular juvenile chronic arthritis.

An 8 year old girl with seronegative pauciarticular onset juvenile chronic arthritis who developed progressive joint destruction and aortic incompetence is presented.     

The association of pauciarticular juvenile arthritis and myasthenia gravis

We encountered two children with fluorescent antinuclear antibody-positive pauciarticular juvenile arthritis who later developed myasthenia gravis. Acetylcholine receptor binding, blocking, and modulating antibodies, retrospectively tested on frozen serum, yielded negative results before the onset of myasthenic symptoms but all yielded strongly positive results coincident with the onset of weakness. In both children, myasthenia gravis responded to thymectomy, and one child had a beneficial response to plasmapheresis. Although, to our knowledge, only two patients with juvenile arthritis and myasthenia gravis have been described in the past, the presence of two additional children with both diseases in a single clinic population suggests that the association may be more prevalent than previously suspected.     

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.     

Human leukocyte antigen-DRB1*1104 in the chronic iridocyclitis of pauciarticular juvenile rheumatoid arthritis.

To determine whether genetic markers for chronic iridocyclitis could be identified, we used both serologic and oligonucleotide dot blot techniques to characterize immunogenetically 164 children with early-onset pauciarticular juvenile rheumatoid arthritis. Seventy-eight children (47.6%) had chronic iridocyclitis and 86 (52.4%) had not had evidence of eye disease during a mean follow-up period after the onset of arthritis of 15.8 years (minimum of 5.5 years). Control subjects were 218 healthy, unrelated individuals. The analysis was limited to alleles known to be associated with an increased or decreased risk of early-onset pauciarticular juvenile rheumatoid arthritis or of chronic iridocyclitis in this form of juvenile rheumatoid arthritis. Only one split of human leukocyte antigen (HLA)-DR5, HLA-DRB1* 1104, showed a statistically significant association with a risk of chronic iridocyclitis (chi-square value = 7.52; p = 0.036 adjusted; odds ratio 3.45); HLA-DQA1* 0501 and HLA-DQB1* 0301, both in linkage disequilibrium with HLA-DRB1* 1104, also were significantly associated with eye disease. Patients with both the DRB1* 1104 and DPB1* 0201 genes had a 7.7-fold increased risk for chronic iridocyclitis compared with that for other patients. The presence of HLA-DRB1* 1104 was about four times as specific, but only about one third as sensitive, as antinuclear antibodies in identifying patients at risk for eye disease. Although all children with early-onset pauciarticular juvenile rheumatoid arthritis should undergo periodic slit-lamp examinations, those with the HLA class II gene DRB1* 1104 are at particularly high risk for eye disease, and we recommend that they be monitored carefully for its evolution.     

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.     

Advanced drug therapy for juvenile rheumatoid arthritis

Childhood rheumatic diseases are frequently chronic, painful, and potentially debilitating. They may adversely affect growth and development, compromise future quality of life, and contribute added stress to the patient and family. Awareness of these consequences provides a stimulus to develop more effective therapeutic regimens. There is optimism that new therapeutic strategies will result in the more widespread and earlier use of drugs, including those discussed, that may substantially impede or arrest the underlying disease.     

Treatment of juvenile rheumatoid arthritis

Recently interest in pediatric rheumatology has increased. This review tries to summarize our knowledge about pharmacological mechanisms, as well as practical application and adverse of antirheumatic drugs. In addition, forms of treatment which sofar lack any scientific backing are mentioned and discussed. Various data about efficacy and side effects in children are poor or lacking, and current treatment in children is based on analogy conclusions derived from experiences with adult RA. The therapeutic concept presented in this article offers a good chance to improve the functional status of the rheumatic child. However, one should be aware that several controlled trials with antirheumatic drugs in children still have to be performed. Uncertainties have to be replaced by knowledge. This is true also for the surgical treatment mentioned.