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.

Decreased capillary density in juvenile dermatomyositis and in mixed connective tissue disease.

OBJECTIVE: To assess whether quantitative capillary microscopy is a useful tool to evaluate capillary abnormalities in children with connective tissue diseases. METHODS: Eight children with juvenile dermatomyositis (JDM), 6 with mixed connective tissue disease (MCTD) and 23 healthy children were investigated with computer based quantitative capillary microscopy. Median disease duration was 1 year among JDM (1-4) and 3 years (1-7) among MCTD. RESULTS: Capillary density was decreased in JDM [median 2.5 (1.4-4.3) loops/mm (p < 0.001)] and in MCTD [median 5.0 (4.1-7.0) loops/mm (p < 0.05)] compared to healthy controls [median 6.8 (5.3-8.0) loops/mm]. Median capillary loop area was increased in JDM [median 8.5 (3.0-15.8) 10(-3) mm2 (p < 0.001)] and in MCTD [median 4.5 (3.0-6.0) 10(-3) mm2 (p < 0.02)] compared to controls [median 2.5 (1.0-4.0) 10(-3) mm2]. CONCLUSION: Quantitative nailfold capillary microscopy is a sensitive indicator of JDM. In MCTD this technique is less discriminative.

Wrist synovectomy in juvenile chronic arthritis (JCA).

Synovectomy of 20 wrist joints in 15 patients with juvenile chronic arthritis (JCA) was performed and results were evaluated 3 (0.5-5) years after surgery. The surgical procedure consisted of synovectomy of the radiocarpal joint, around the ulnar head and in all intercarpal joints. Resection of the ulnar head was not performed. Results at follow-up showed that four wrist joints had been re-operated by arthrodesis because of persistent pain. In one case a spontaneous ankylosis had developed. These cases were not included in the re-examination. Of the remaining 15 cases, 12 were improved and 7 of these were totally pain-free. At follow-up, clinical examination revealed a mean improved grip strength (0.2 kp/cm2) and a mean decreased range of motion (10 degrees of flexion and extension, 16 degrees of pronation, supination unchanged). All wrist joints showed some radiographic changes preoperatively and at follow-up about half showed some further deterioration. This study indicates that the results of wrist synovectomy in patients with juvenile chronic arthritis do not differ significantly from results of wrist synovectomy in adult arthritic patients.

Synovectomy of the knee in juvenile chronic arthritis. A retrospective, consecutive follow-up study.

We report 60 synovectomies of the knee in 51 children with juvenile chronic arthritis. Synovitis had been present for an average of 5 years and the average age at operation was 13 years. Results were evaluated in terms of pain, knee movement, relapse of synovitis and radiological change during a follow-up averaging 7.5 years. The relief of pain was rewarding and there was a slight postoperative gain in range of knee movement in most cases. The older the patient at onset of disease, the greater the risk of pain during follow-up. Progressive joint destruction was more common in younger patients, those with systemic or polyarticular disease, and those with highly active disease at the time of operation. Recurrence of synovitis was more frequent in patients who had their operation in a phase of high disease activity; this occurred most often in patients with polyarticular disease.

Cardiac involvement in juvenile rheumatoid arthritis. A follow-up study.

Cardiac involvement was diagnosed in 15 of 320 cases of JRA (4.7%) and was most frequent in children with active systemic disease. Ten children had pericarditis, 2 had myocarditis, 2 had peri-myocarditis and 1 had aortic valvulitis. The highest risk of heart involvement was found during the first three years but it could occur at any time. Recurrent episodes were seen in 60% of cases. The prognosis in pericarditis seems to be good since no patient developed cardiac tamponade or constrictive pericarditis and cardiac function evaluated by echocardiography was normal in all patients at follow-up. In patients with myocarditis and peri-myocarditis, a dilated left ventricle was found in 2 of 4 patients and 1 patient who died, had severe cardiac changes at autopsy. The prognosis in myocarditis thus seems to be worse. In valvulitis the prognosis depends on which valve is involved. The benefit of early treatment with corticosteroids is discussed.

Prognosis in juvenile rheumatoid arthritis with systemic onset. A follow-up study.

Thirty-three patients with systemic onset JRA were followed up for 4 to 24 years (median 10 years). None had positive RF or ANA. Most patients developed polyarthritis. Cardiac involvement occurred in 14 patients (42%). Cardiac prognosis was good for pericarditis but seemed to be worse for myocarditis or perimyocarditis. Three patients contracted renal amyloidosis (9%). Severe growth retardation was observed in 39 per cent. Half of the patients had low activity or were in remission after in average 5.9 years' duration of the disease. Seventy per cent of the patients were in a good functional state. Patients with less than average radiological progression during the first 5 years of disease and those with onset of disease after the age of 5, seemed to have a better functional prognosis.