Previous synovectomy or epiphyseal stapling and the influence on knee replacement in juvenile chronic arthritis.

Seventy-seven anatomically graduated components (AGC) total knee arthroplasties (TKA) were performed on 52 patients with juvenile chronic arthritis. According to the nature of previous surgery on the knee, the patients were subdivided into three groups. The mean age at onset of disease in 23 patients with previous synovectomy of the knee was 11 (1.5-16) years, the mean age at the time of synovectomy was 20 (4-42) and the mean age when arthroplasty was performed was 31 (18-45) years. In nine patients with previous epiphyseal stapling, the mean age at disease onset was 4 (1.5-8) years, at stapling 8 (4-16) years, and at arthroplasty 23 (18-30) years. In patients with no previous surgery, the mean age at disease onset in this group was 7 (1.5-16) years and the mean age at arthroplasty 34 (16-64) years. Patients with need for epiphyseal arrest had an early disease onset and knee replacement in early adulthood. The mean age at knee replacements was highest in the group with no prior surgery.

Long-term results of leg length discrepancy treated with temporary epiphyseal stapling in children with juvenile chronic arthritis.

OBJECTIVE: The aim of the present study was to evaluate retrospectively the long-term efficacy of temporary stapling of the knee epiphyses over four decades of use in children with JCA. METHODS: Medical data of the patients with temporary epiphyseodesis due to leg length discrepancy (LLD) were studied. Seventeen knees in 17 patients were found with sufficient follow-up data for evaluation. Patient documents and radiographs of these patients were evaluated. RESULTS: The mean age at the time of the operation was 11 years (range: 6-15) in 3 males and 14 females. The preoperative mean LLD was 21 mm (SD 8) and at staple removal 4 mm (SD 10). The difference was -17 mm (95% CI: -10 to -23). Statistically the result remained the same during the follow-up. Two reversible complications were documented among the 17 stapled knees. In five (29%) cases the correction was affected by re-occurrence of LLD quickly after removal of the staples. CONCLUSION: In this study with 17 patients and a wide range of follow-up times we found that the good correction of LLD achieved by stapling is usually permanent.

Juvenile idiopathic arthritis in multicase families.

OBJECTIVE: To characterize juvenile idiopathic arthritis (JIA) patients from multicase families. METHODS: The study series comprised 80 affected siblings belonging to 37 families. Comparisons were made with a population-based series of JIA patients from Finland and with a sibling series from the United States. RESULTS: The distribution of cases according to onset type was similar in the sibling and population-based series. The age at diagnosis was significantly lower in the sibling series (4.8 years vs 7.4 years; p < 0.001). There was more intra-pair similarity in onset and course types in the United States series compared to the Finnish series and the proportion of girls was higher in the former. CONCLUSION: The only significant difference between familial and sporadic cases with JIA is an earlier onset of disease in familial cases. There is no essential difference in clinical features of the disease between patients in the multicase and sporadic groups. Differences between the Finnish and US series may be due to selection bias in the latter.

Eosinophilia as a side-effect of methotrexate in patients with chronic arthritis.

This report describes isolated reversible eosinophilia without additional subjective symptoms or signs in three patients on methotrexate therapy, two of them with juvenile idiopathic arthritis and one with rheumatoid arthritis.

Development rate of mutilans fingers in patients with rheumatic disease.

OBJECTIVE: To describe arthritis mutilans (AM) deformity during the progression of rheumatic disease. METHODS: The development of mutilans-like hand deformities in 2 patients with juvenile chronic arthritis (JCA) and in 2 patients with adult onset rheumatoid arthritis (RA) are presented. The hands of these patients were evaluated at least at two time points during the course of disease using two different scoring methods based on differently summed Larsen grades of the hand joints. RESULTS: Two patients (one with JCA and one with RA) showed AM changes after a disease period of less than 10 years and 2 not until after 30 years. The patients with adult onset disease were young at the onset of joint disease. Early wrist fusions were performed on both patients showing a slow development rate. CONCLUSIONS: The development rate of AM is very variable, even in patients with the same diagnoses. Wrist fusion prevents shortening of the carpus and may decrease the development rate of AM.

Survivorship of AGC knee replacement in juvenile chronic arthritis: 13-year follow-up of 77 knees.

This study analyzed the survivorship and results of 77 knee replacements in 52 patients with juvenile chronic arthritis using the nonconstrained Anatomically Graduated Components (AGC; Biomet, Warsaw, IN) prosthesis design. Patients were operated on between the years 1985 and 1995. The mean duration of the general disease was 24 years (range, 10-56 years), and the mean age of the patients at the time of surgery was 33 years (range, 16-64 years). Bone-grafts were installed into 15 knees, custom-made components were used in 5 knees, and cemented fixation in 4 knees. The patella was resurfaced in 23 knees. Clinical follow-up examinations were conducted 3 months, 1 year, 4 years, and 8 years postoperatively. An interview was arranged at the end of 1998, 3 to 13 years after surgery; 2 patients were not reached, and 2 died during the follow-up. Fifty-five of 73 (75%) knees were subjectively excellent, 18 (25%) were fair, and none was poor. Radiolucent lines of 1.0 to 1.5 mm were found under 14 tibial trays but not adjacent to femoral components. No deep infections were detected. One knee was revised 4 years after the implantation. The overall survival was 99% (95% confidence interval, 92-100) at 5 years. We consider these results excellent in this demanding patient material. The nonconstrained AGC prosthesis with cementless fixation proved to be feasible in knee replacement in patients with juvenile chronic arthritis.

Assessment of mutilans-like hand deformities in chronic inflammatory joint diseases. A radiographic study of 52 patients.

OBJECTIVES: To evaluate patients with mutilans-like hand deformities in chronic inflammatory joint diseases and to determine radiographic scoring systems for arthritis mutilans (AM). METHODS: A total of 52 patients with severe hand deformities were collected during 1997. A Larsen hand score of 0-110 was formed to describe destruction of the hand joints. Secondly, each ray of the hand was assessed individually by summing the Larsen grade of the wrist and the grades of the MCP and PIP joints. When the sum of these grades was > or = 13, the finger was considered to be mutilated. A mutilans hand score of 0-10 was formed according to the number of mutilans fingers. Surgical treatment and spontaneous fusions were recorded. RESULTS: The study consisted of 22 patients with juvenile rheumatoid arthritis (JRA), nine with rheumatoid factor (RF) positive and 13 with RF negative arthritis, 27 patients with RF positive RA, and three adult patients with other diagnoses. The mean age of patients with adult rheumatic diseases was 27 years at the onset of arthritis. The mean disease duration in all patients was 30 years. The mean Larsen hand score was 93. Four patients had no mutilans fingers and in 15 patients all 10 fingers were mutilated. The Larsen hand score of 0-110 and the mutilans hand score of 0-10 correlated well (rs = 0.90). Fourteen patients showed spontaneous fusions in the peripheral joints. A total of 457 operations were performed on 48 patients. CONCLUSION: Both the Larsen hand score of 0-110 and the mutilans hand score of 0-10 improve accuracy in evaluating mutilans-like hand deformities, but in unevenly distributed hand deformities the mutilans hand score is better in describing deformation of individual fingers.

Chlorambucil in severe juvenile chronic arthritis: longterm followup with special reference to amyloidosis.

OBJECTIVE: To assess the effectiveness and side effects of chlorambucil therapy and patient outcome in juvenile chronic arthritis (JCA) treated with chlorambucil. METHODS: Followup of 79 consecutive patients with JCA refractory to previous therapy, in whom chlorambucil treatment was initiated from 1982 to 1995. Mean treatment duration was 7 months in 72 patients and more than 20 months in 6 patients with amyloidosis and 1 with severe iridocyclitis. RESULTS: Within 6 months, remission was attained in 41 patients (52%): 29 of these relapsed after a mean period of 2.5 years (range 0.3-8.3). Twenty-one (27%) patients did not respond. Seven out of 11 patients with secondary amyloidosis had proteinuria, which cleared completely in 4 and almost completely in 1. In 16 patients (20%) chlorambucil was stopped because of side effects, and in 1 because of infection. After a mean followup of 8.5 years, 14 patients (18%) were in complete remission without drugs, 34 (43%) had minor symptoms only, but 26 (33%) had limitations in daily activities. Five (6%) had died (2 of leukemia; 2 of infection, 0.5 and 3.3 years after withdrawal of chlorambucil; 1 of amyloidosis). CONCLUSION: Chlorambucil was found to be a very potent drug for JCA with acceptable short term, but serious longterm side effects. It may still be useful in JCA complicated by amyloidosis.

HLA B27: a prognostic factor in juvenile chronic arthritis.

This study was performed to assess the frequency of HLA B27 in patients with juvenile chronic arthritis (JCA) of varying severity and outcome by studying three patient categories: those in whom cytostatic treatment with azathioprine had been started, those with secondary amyloidosis, and those with arthroplasty of the knee or hip joints. In the first category the frequency of the HLA B27 allele was compared between those who had attained remission and those who had not. In the second and third categories the rate at which amyloidosis developed and the timing for the need of arthroplasty, were compared for HLA B27-positive and -negative patients. A control group consisted of 37 patients with uncomplicated seronegative polyarthritis. Ten of the 37 patients in the control group (27%) were HLA B27 positive as opposed to 84 out of 190 (44%) in the three study groups. Of the 101 patients treated with azathioprine, two out of 15 in remission were HLA B27 positive, whereas as many as 41 out of 86 with still active disease were HLA B27 positive (p = 0.013). Of the secondary amyloidosis patients, 29 out of 51 carried HLA B27. The HLA B27-positive patients contracted amyloidosis on average 5.9 (median 6.7) years earlier than the HLA B27-negative patients (p = 0.038). Of the arthroplasty patients, 39 out of 91 carried HLA B27. The HLA B27-positive patients underwent arthroplasty on average 2.9 (median 3.5) years earlier than the HLA B27-negative patients (p = 0.050). We conclude that HLA B27-positive cases are accumulated among the most severe cases of JCA.

Survivorship of the Charnley total hip arthroplasty in juvenile chronic arthritis. A follow-up of 186 cases for 22 years.

Between 1971 and 1991 we performed Charnley low-friction arthroplasty (LFA) on 116 patients (186 hips) with juvenile chronic arthritis (JCA). We have now carried out a survival study, taking endpoints as revision, death or the end of the year 1993. Overall survival was 91.9% at ten years and 83.0% at 15 years. That of the femoral component was 95.6% at ten years and 91.9% at 15 years and of the acetabulum 95.0% and 87.8%, respectively. Only the use of steroids significantly impaired the survival. We therefore recommend the use of Charnley LFA for young patients with JCA requiring total hip replacement.

Azathioprine in patients with juvenile chronic arthritis: a longterm followup study.

OBJECTIVE: To evaluate drug survival, efficacy, side effects, and longterm toxicity of azathioprine treatment in patients with juvenile chronic arthritis (JCA). METHODS: In an uncontrolled, prospective study we evaluated 129 consecutive patients with JCA refractory to therapy in whom azathioprine treatment was begun during 1980-1989. In the first 29 patients, a 2 year trial was planned, while for the remaining 100 patients the protocol was to continue until remission or dropout. The median treatment period was 13 months (range 3 days-8.5 yrs). Patients were assessed every 2 months for 2 years for efficacy, side effects, growth and need for glucocorticoids, and outcome evaluated in late 1996. RESULTS: Remission without drugs was attained in 19 patients (15%); in addition, temporary remission in patients continuing treatment was attained in 18 cases (14%). Treatment was discontinued due to side effects in 18 cases (14%); in two-thirds of these cases side effects occurred during the first 2 months. Of the total number of patients, 49 (38%) completed 2 years of treatment, with significant improvement in both clinical and laboratory indices of disease activity. Treatment had no noticeable effect on iridocyclitis. One patient died of cytomegalovirus infection during azathioprine treatment. CONCLUSION: Azathioprine is a useful drug in severe JCA, with a sustained effect and acceptable side effects. Even in cases of incomplete remission, its glucocorticoid sparing effect was noteworthy.

Increased risk of leukaemia in patients with juvenile chronic arthritis treated with chlorambucil.

Two cases of acute leukaemia have developed in a group of 77 patients treated with chlorambucil (Chl) because of severe juvenile chronic arthritis. The total follow-up from the beginning of Chl treatment in these patients was 560 years, indicating a highly increased risk of leukaemia. Despite favourable results, especially in patients with secondary amyloidosis, Chl should only be used in selected cases.

Incidence of systemic connective tissue diseases in children: a nationwide prospective study in Finland.

OBJECTIVE: To study the incidence of systemic connective tissue diseases (CTD) and of systemic onset juvenile arthritis (S-JA) in children aged 0 to 15 years. METHODS: A nationwide, prospective, hospital based series of new patients from Finland collected during a 4-year period and supplemented with data from the National Hospital Discharge Register. The population at risk was 1.02 million. Classification criteria developed for adult CTD and the American Rheumatism Association criteria for JA were used. RESULTS: The annual incidence rates found were as follows: systemic lupus erythematosus 0.37, polymyositis/dermatomyositis (PM/DM) 0.30, mixed connective tissue disease 0.10, scleroderma 0.05, and S-JA 0.47/100,000. Girls outnumbered boys in the whole series of CTD (24:9) and also in S-JA (11:8), but in PM/DM the sex distribution was even. CONCLUSION: CTD and S-JA are rare before the age of 16 years. From the present incidence figures and those recently published for JA the incidence of CTD is estimated to be between 4 and 7% of that of JA, and S-JA accounts for only 2 to 4% of all JA.

Decrease in the number of deaths from secondary amyloidosis in patients with juvenile rheumatoid arthritis.

OBJECTIVE: The analysis of the mortality rate and causes of death of all Finnish patients with juvenile rheumatoid arthritis (JRA) in 1969-1979 and 1980-1990. METHODS: Cases with JRA and deaths at the age of 24 years or younger were identified by using the nationwide register of persons entitled to free medication because of rheumatoid arthritis and allied diseases. Causes of death are based on autopsy in all but 4 of 47 cases. RESULTS: There were 24 deaths during 1969-1979 and 23 during 1980-1990. The expected numbers of deaths were 8.9 and 9.5, giving a standardized mortality rate of 2.7 and 2.4, respectively. Secondary amyloidosis was the direct cause of death in 10 patients during the earlier period and in 4 patients during the later period. CONCLUSIONS: The decrease in amyloidosis as a cause of death may be explained by an increased use of cytotoxic drug treatment for severe JRA in the early 1980s. Deaths from violence have increased.

Bacterial infection following intra-articular injection. A brief review.

The literature on 443 cases of postinjection bacterial arthritis is reviewed. The utter rarity of the condition may have been overemphasized in the past. Haematogenous infection of the puncture track may be more important than the transfer of skin bacteria into the joint.