Eur J Pediatr (1992) 151 : 339-,341

European Journal of

Pediatrics

9 Springer-Verlag1992

Disease patterns in early onset pauciarticular juvenile chronic arthritis R. Hertzberger-ten Cate 1, 2, 3, B. C. M. de Vries-van der Vlugt 1, L . W . A . van Suijlekom-Smit 3, and A . Cats 4 1Department of Paediatrics, University Hospital Leiden, Mailbox 9600, NL-2300 RC Leiden, The Netherlands 2juliana Children's Hospital, The Hague, The Netherlands 3Sophia Children's Hospital, Rotterdam, The Netherlands 4Department of Rheumatology, University Hospital, Leiden, The Netherlands Received May 28, 1991 / Accepted in revised form September 26, 1991

Abstract. 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. A n intermittent pattern of disease was found in 60 children (79%). O f the remaining 16 patients continuous persistent pauciarticular disease activity was present in 7 (9.2%) and extended pauciarticular in 9 children (11.8%). A n 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 seronegatire polyarticular J C A , underlining previous reports that the joint pattern during the course of disease may be m o r e important than joint pattern at onset of disease.

adults who had pauciarticular J C A in childhood does not differ from that found for young adults with other forms of J C A [10]. Different patterns of early onset pauciarticular J C A have been described [4] and there are indications that the pattern of disease may be m o r e important for the outcome than the joint pattern at onset [3]. Progression of pauciarthritis to polyarthritis, for example, is known to lead to significant functional disability and deformity [6]. A carefully defined group of children with early onset pauciarticular J C A was studied in order to find parameters that m a y help to identify children with a p o o r prognosis and to establish the frequencies with which the different patterns of disease occur. The frequencies of complicating factors as well as extra-articular manifestations within each pattern of disease were also investigated.

K e y words: Pauciarticular juvenile chronic arthritis Disease patterns

The study involved all children known to have early onset pauciarticular JCA who had been followed for a minimum of 3 years. Duration of disease varied from 3 to 16 years. Disease patterns were established retrospectively by chart review in December 1989. Of the patients 80% were seen within 6 months of onset by one of the authors. To avoid contamination with other subsets of JCA the following criteria at onset also had to be met:

Introduction Early onset or type 1 pauciarticular juvenile chronic arthritis (JCA) is the most c o m m o n subset of J C A , mainly affecting girls under 6 years of age. Circulating antinuclear antibodies ( A N A ) , which frequently occur, are associated with chronic anterior uveitis ( C A U ) [12]. The functional outcome for children with early onset pauciarticular J C A is believed to be good, although some patients develop a visual handicap as a result of C A U [6, 14]. In fact the prevalence of disability among young Offprint requests to: R. Hertzberger-ten Cate Abbreviations: ANA = antinuclear antibodies; CAU = chronic

anterior uveitis; JCA = juvenile chronic arthritis

Patients and methods

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age under 6 years, no hip involvement, no enthesiopathy, and no IgM rheumatoid factor.

Out of our total outpatient population of children with JCA who had been followed for at least 3 years in 1989, 76 children fulfilled all of the above-mentioned criteria and were included in the study. During follow up they were regularly seen by the same physician. The ANA test, using liver cells as a substrate, was positive in 81.5% of the children. Four children exhibited the HLA-B27 antigen (5%). By studying the medical charts different disease patterns could be recognized. The following parameters at onset were then analysed with respect to the different disease patterns: gender, age, joints involved, ESR and the presence of both ANA and the HLA-B27 antigen (Table 1). The frequencies of CAU, visual handicaps, psychosocial problems that required the help of a

340 Table 1. Comparison of clinical parameters at onset in 76 children

Table 2. Frequencies of complications and extra-articular manifes-

with three patterns of early onset pauciarticular JCA

tations according to disease pattern in children with early onset pauciarticular JCA

Intermittent Number Female sex Onset (years) Onset knee Onset ankle ESR (mm) ANA

60 49 (66%) 2.8 42 (70%) 14 (23%) 23 48 (80%)

Continuous Extended

Persistent

9 9 (100%) 2.8 5 (55%) 2 (22.2%) 36 9 (100%)

7 4 (57%) 3.2 6 (85.7%) 1 (14.2%) 14.5 5 (71.4%)

Intermittent Number CAU Visual handicap Psychological problems Local growth disturbances

Continuous Extended

Persistent

60 15 (25%) 1 (1.6%)*

9 1 (11.1%) 1 (11.1%)*

7 6 (85.7%) 4 (57.1%)*

13 (21.6%)*

6 (66.6%)*

5 (71.4%)*

23 (38%)*

9 (100%)*

7 (100%)*

* Statistical significance, for P-values see text psychologist or psychiatrist, local growth deformities seen at physical examination and adjusted schooling were also established for each pattern of disease.

Results

In 60 out of 76 children the episodes of arthritis alternated with episodes without arthritis in an intermittent pattern; only two of these children eventually developed arthritis in more than four joints (extended pauciarticular pattern). In the remaining 16 children continuous disease activity was noted: an extended pauciarticular pattern (more than four joints involved) in 9 cases and a persistent pauciarticular pattern (a maximum of four joints involved) in 7 cases. Children with intermittent disease were compared with those with continuous disease. In the group with a continuous pattern a further comparison was made between children with a persistent and those with an extended pauciarticular course. There was no significant difference in the clinical parameters at onset between the groups with the different disease patterns (Table 1). The frequencies of CAU, visual handicaps, psychosocial problems, local growth disturbances and adjusted schooling are shown in Table 2 for the different patterns of disease. The incidence of C A U did not differ significantly between children with continuous and intermittent disease. Extension to polyarthritis occurred after 1-6 years with a mean of 2.5 years. The pattern of disease was established within 3 years of onset of disease in the majority of patients. Five children, all with a continuous pattern, suffered from a visual handicap; this is significantly higher than the number of visually handicapped children with intermittent disease (P = 0.0003). Psychosocial problems were encountered more frequently in children with continuous disease compared to those with an intermittent pattern (P = 0.0006). Children with intermittent disease had fewer local growth disturbances than children with continuous disease. Children who needed adjusted schooling all had continuous disease: two children visited a school for visually handicapped children (persistent pauciarticular course), adjusted schooling was needed because of a

physical handicap in two cases (extended pauciarticular course) and one child lived in a remedial institute because of severe behavioural problems (extended pauciarticular course). Comparison of the patients with continuous disease activity and extended (n = 9) versus persistent (n = 7) joint involvement revealed that children with a persistent pauciarticular pattern exhibited a significantly higher incidence of C A U than children with an extended pauciarticular course (P = 0.0032). Of the 76 children with early onset pauciarticular JCA 9 had extended pauciarticular disease (12%) with gradual symmetrical involvement of small joints in the hands and feet; radiological impairment was demonstrated in 6 patients. The frequencies of visual handicap, psychosocial problems and/ or local growth disturbances did not differ significantly between children with persistent and extended disease.

Discussion

Three patterns of disease were clearly recognized: an intermittent pattern, continuous disease activity in an extended pauciarticular pattern and continuous joint involvement in a persistent pauciarticular pattern. The majority of children (80%) with early onset pauciarticular JCA have intermittent disease, a finding in accordance with other authors [2, 12]. The remaining 20% have continuous disease activity of either the extended pauciarticular or the persistent pauciarticular type. The frequency of complications or extra-articular manifestations is the highest in the group of children with continuous disease. They consist of CAU, psychosocial problems, local growth deformities and development of polyarthritis. In this study all children who needed adjusted schooling because of a visual or physical handicap had continuous disease. The clinical parameters investigated in this study as in others [12] were neither helpful in identifying at disease onset children who will ultimately have continuous disease, nor in distinguishing children with an extended pattern from children with a persistent course. The 11.8% progression to polyarthritis in our study is comparable to the findings of Schaller [12] but somewhat

341 lower than that described by Venning and Ansell [15]. The percentage of extended pauciarthritis varies between 10% and 40% [7, 12, 15]. Disease patterns are usually established within 3 years of onset [15]. Although the courses of arthritis and C A U are usually independent [11], it a p p e a r e d in this study that m o r e children with a continuous pattern of disease have a visual handicap than those with intermittent disease, even when age at onset and duration of C A U are comparable. In time there will be m o r e children who have developed C A U than there were at the time of the study, but the risk of developing C A U decreases with time and is small, 7 years or m o r e after the onset of arthritis [8]. The longt e r m visual outcome correlates with the severity of the inflammation at initial ocular examination [17] and the age at onset of C A U [11]. O u r findings suggest that an unfavourable outcome also correlates with a continuous pattern of joint disease. The total n u m b e r of families (31%) that needed help because of psychosocial problems was comparable to the findings of earlier studies of families with children who have pauciarticular J C A [5]. Families with a child with intermittent early onset pauciarticular J C A needed psychosocial help less frequently than families with a child with continuous disease activity. It is conceivable that each pattern of early onset pauciarticular J C A has its own consequences, but psychosocial problems form a major complicating factor in all. Disabled children are expected to have psychosocial problems, but it appears that psychosocial problems are encountered even m o r e often in juveniles with chronic arthritis without a handicap [9]. The extended pauciarticular pattern appears to resemble seronegative polyarticular J C A in joint pattern, radiological destruction of joints, the incidence of C A U and outcome. This underlines the statement m a d e by Cassidy that the pattern of disease m a y be m o r e important than the joint pattern at onset [3]. Children with an extended pauciarticular course could form a separate subgroup which until now can only be recognized during the course of their disease. In this study an overall favourable outcome was noted as 60 out of 76 children (79%) have remained with pauciarticular disease in an intermittent pattern, and 7 of the 16 with persistent disease also remained with limited joint disease. The need for criteria to diagnose early onset pauciarticular J C A remains, but even when strict criteria are

used it is still possible that these patients have a different disease [1]. Prognosis seems to be influenced by ophthalmological complications, psychological disturbances and extension to polyarthritis. Further investigation aimed at identification of children at risk for these prognostic factors in an early stage of their disease is necessary.

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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 a...
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