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We present the results of antineutrophil cytoplasmic antibody (ANCA) staining in patients with juvenile chronic arthritis (JCA). Thirty-one patients with an age range of 1-16 years were included in the study: 13, 15 and three patients, respectively, were classified having oligoarticular, polyarticular and systemic-onset disease. Indirect immunofluorescence analysis revealed ANCA staining in 45% of the patients. All, except one, revealed atypical pANCA staining. ELISA studies for anti-myeloperoxidase were positive in only one patient with typical pANCA staining. PR-3 ANCA tested negative in all patients. There were no significant correlations between ANCA staining and the clinical parameters of the patients. We conclude that, although the specificity of ANCA in JCA remains to be elucidated, it may be effective in the pathogenesis of the disease.

Objective: To investigate the prevalence of juvenile chronic arthritis (JCA), familial Mediterranean fever (FMF), and Behçet's disease in Turkish children through a field survey., Methods: The field survey was based on cluster centering with 2 level strata. A total of 46,813 children were screened. For the diagnosis of chronic arthritis and Behçet's previously suggested criteria were used. We have developed criteria for the diagnosis of probable FMF. Children previously diagnosed to have these diseases were also defined and included., Results: JCA was found in 6.4/10,000. 2.8/10,000 children were previously diagnosed as FMF (minimum phenotype frequency). Together with the probable diagnosis of FMF, the prevalence increased to 9.3/10,000. The findings were also compared with those of our center. None of the 46,813 children had Behçet's disease., Conclusion: The prevalence of chronic arthritis is similar to the other childhood populations reported. However, FMF has a very high prevalence.

The immunoinflammatory pathogenesis of juvenile chronic arthritis (JCA) involves the activation of many pathways including various cytokines. We have evaluated the levels of interleukin(IL)-1, IL-6 and IL-8 in 29 JCA patients. The age range was 1-16 with a mean of 10.1. A disease activity score was developed on the basis of: 1. constitutional symptoms and/or morning stiffness, 2. presence of joint swelling, 3.warmth, 4.limited range of motion, and 5.joint pain. This score correlated very significantly with laboratory disease activity markers such as erythrocyte sedimentation rate (ESR) and CRP (both p = 0.006) and also correlated with IL-1 and IL-6 levels. The levels of IL-1 decreased in four of the five patients with improved disease activity. IL-6 but not IL-1 correlated significantly with the number of inflamed joints (p = 0.013); IL-6 also strongly correlated with rheumatoid factor supporting this cytokine's role in B cell induction (p = 0). Haemoglobin values correlated negatively with the activity index, ESR, CRP, IL-1 and IL-6. IL-8 did not correlate with disease activity markers. In the systemic patients all cytokines tended to be higher. Our data suggest that interleukins 1 and 6 are effective in the pathogenesis of JCA. Whether cytokines may be used for monitoring therapy may be clarified with further studies.

We have attempted to investigate the dysplastic changes in the hematopoietic system associated with juvenile rheumatoid arthritis (JRA) and its relation to disease activity. The peripheral blood smear and bone marrow aspiration samples of 17 JRA patients were investigated and correlations with laboratory parameters of disease activity sought. The age range was 6-16 years and the duration of disease 1.5-108 months. Abnormal finding of the peripheral smear and bone marrow were scored separately. The score of pathological peripheral blood findings correlated significantly with CRP and ferritin (both P <0.05). In the bone marrow specimens marked changes were noted in the myeloid, erythropoietic, and megakaryopoietic series; however, the score of pathological findings did not correlate with laboratory parameters of disease activity (P > 0.05). We suggest that JRA is associated with marked myelodysplastic changes, also manifested in the peripheral blood smear; these changes may well be the consequence of the inflammatory milieu, including cytokines, during active disease.

Anaemia is a common manifestation of juvenile rheumatoid arthritis (JCA). We have evaluated 26 JCA patients with anaemia and compared their laboratory parameters to those without anaemia. In the patients with anaemia, activation criteria such as erythrocyte sedimentation rate (ESR) and CRP were significantly higher than in those without anaemia. Anaemia was present in all systemic JCA patients and was present in 42% and 78% of the oligoarticular and polyarticular types, respectively. Serum iron levels and transferrin saturations were low in all, whereas serum iron-binding capacities of the patients were normal. Mean ferritin level was 249pg/l (range 8.46-1000pg/l). There was a significant correlation between ferritin levels and CRP and ESR (r = 0.48 and r = 0.55 respectively) (both p < 0.05). Epo levels were normal. Twelve (60%) of the bone marrow aspiration specimens stained positive for iron whereas 40% stained negative; there were also changes suggestive of myelodysplasia. Sideroblasts were also decreased in number. Thus, in these patients iron is not sufficiently transferred to the erythroid series and/or cannot be used by erythroblasts, accompanied by a possible absolute iron deficiency. Thus we suggest that the iron in JCA tends to be stored in the form of ferritin, not in an accessible form and impaired metabolism along with other factors are effective in the anaemia of JCA.