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Development of positive antinuclear antibodies and rheumatoid factor in systemic juvenile idiopathic arthritis points toward an autoimmune phenotype later in the disease course.

Hügle B, Hinze C, Lainka E, Fischer N, Haas JP - Pediatr Rheumatol Online J (2014)

Bottom Line: A retrospective chart survey was used to extract demographic and clinical data as well as presence and titers of ANA and RF at diagnosis and during follow-up. 32 patients were included in the study, with a median age of 4.2 years and median follow-up of 6.0 years. 8/32 patients had ANA titers ≥ 1:80 at diagnosis, with 22/32 patients showing rising ANA titers with titers ≥ 1:80 at last follow-up (p =0.001). 10/32 patients had a positive RF at least once during follow-up, compared to 0/32 at diagnosis (p = 0.001).In 5/10 patients, positive RF was documented at least twice, more than twelve weeks apart.Patients treated with TNF antagonists were not significantly more likely to develop positive ANA titers (p = 0.425) or positive RF (p = 0.703).

View Article: PubMed Central - HTML - PubMed

Affiliation: German Center for Pediatric and Adolescent Rheumatology, Gehfeldstrasse 24, 82467 Garmisch-Partenkirchen, Germany.

ABSTRACT

Background: Systemic juvenile idiopathic arthritis (sJIA) is commonly considered an autoinflammatory disease. However, sJIA patients may develop aggressive arthritis without systemic inflammation later in the disease, resembling an autoimmune phenotype similar to other subtypes of JIA. The objective of this study was to determine whether antinuclear antibodies (ANA) and rheumatoid factor (RF) will develop in patients with sJIA over the course of the disease.

Findings: A single center sample of sJIA patients with follow-up of more than one year was obtained. A retrospective chart survey was used to extract demographic and clinical data as well as presence and titers of ANA and RF at diagnosis and during follow-up. 32 patients were included in the study, with a median age of 4.2 years and median follow-up of 6.0 years. 8/32 patients had ANA titers ≥ 1:80 at diagnosis, with 22/32 patients showing rising ANA titers with titers ≥ 1:80 at last follow-up (p =0.001). 10/32 patients had a positive RF at least once during follow-up, compared to 0/32 at diagnosis (p = 0.001). In 5/10 patients, positive RF was documented at least twice, more than twelve weeks apart. Patients treated with TNF antagonists were not significantly more likely to develop positive ANA titers (p = 0.425) or positive RF (p = 0.703).

Conclusions: Patients with sJIA developed increased ANA titers and positive RF over the course of the disease, independent of treatment with TNF antagonists. This might point towards an autoimmune, rather than an autoinflammatory phenotype later in the course of sJIA.

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Dot dispersion plot of antinuclear antibodies titers at diagnosis and at last follow-up in patients with systemic juvenile idiopathic arthritis (n = 32).
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Figure 1: Dot dispersion plot of antinuclear antibodies titers at diagnosis and at last follow-up in patients with systemic juvenile idiopathic arthritis (n = 32).

Mentions: 32 patients were included in the study (20 of these female), with a median age at diagnosis of 4.2 years (range 0.5 – 11.4 years). The median follow-up was 6.0 years (range 1.1 – 17.3 years). During the course of disease, 96.8% were treated with disease-modifying antirheumatic drugs (of those: methotrexate 100%, azathioprine 52% and cyclosporine A 48%), 65.6% with any TNF antagonist (of those: etanercept 100%, infliximab 14% and adalimumab 29%), 65.6% with anti-interleukin(IL)-1 antagonists and 15.6% with anti-IL-6 antagonists. 8/32 patients had ANA titers ≥ 1:80 at diagnosis, with 22/32 patients showing a titer of ≥ 1:80 at last follow-up (p =0.001) (Figure 1). There was no difference according to age at diagnosis (p = 0.949), length of follow-up (p = 0.197), maximum joint count at any time (p = 0.348) or total joint count at last follow-up (p = 0.314) among persistently ANA-negative, persistently ANA-positive and ANA-seroconverted patients. Using measures at 463 time points, there was no correlation between ANA titers and total active joint count (r = 0.180, p = 0.703). During follow-up, 10/32 patients had a positive RF at least once, compared to 0/32 at diagnosis (p = 0.001). In five of these patients, positive RF were documented at least twice, more than twelve weeks apart. Patients treated with TNF antagonists were not significantly more likely to develop a positive ANA (p = 0.425) or positive RF (p = 0.703).


Development of positive antinuclear antibodies and rheumatoid factor in systemic juvenile idiopathic arthritis points toward an autoimmune phenotype later in the disease course.

Hügle B, Hinze C, Lainka E, Fischer N, Haas JP - Pediatr Rheumatol Online J (2014)

Dot dispersion plot of antinuclear antibodies titers at diagnosis and at last follow-up in patients with systemic juvenile idiopathic arthritis (n = 32).
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4127434&req=5

Figure 1: Dot dispersion plot of antinuclear antibodies titers at diagnosis and at last follow-up in patients with systemic juvenile idiopathic arthritis (n = 32).
Mentions: 32 patients were included in the study (20 of these female), with a median age at diagnosis of 4.2 years (range 0.5 – 11.4 years). The median follow-up was 6.0 years (range 1.1 – 17.3 years). During the course of disease, 96.8% were treated with disease-modifying antirheumatic drugs (of those: methotrexate 100%, azathioprine 52% and cyclosporine A 48%), 65.6% with any TNF antagonist (of those: etanercept 100%, infliximab 14% and adalimumab 29%), 65.6% with anti-interleukin(IL)-1 antagonists and 15.6% with anti-IL-6 antagonists. 8/32 patients had ANA titers ≥ 1:80 at diagnosis, with 22/32 patients showing a titer of ≥ 1:80 at last follow-up (p =0.001) (Figure 1). There was no difference according to age at diagnosis (p = 0.949), length of follow-up (p = 0.197), maximum joint count at any time (p = 0.348) or total joint count at last follow-up (p = 0.314) among persistently ANA-negative, persistently ANA-positive and ANA-seroconverted patients. Using measures at 463 time points, there was no correlation between ANA titers and total active joint count (r = 0.180, p = 0.703). During follow-up, 10/32 patients had a positive RF at least once, compared to 0/32 at diagnosis (p = 0.001). In five of these patients, positive RF were documented at least twice, more than twelve weeks apart. Patients treated with TNF antagonists were not significantly more likely to develop a positive ANA (p = 0.425) or positive RF (p = 0.703).

Bottom Line: A retrospective chart survey was used to extract demographic and clinical data as well as presence and titers of ANA and RF at diagnosis and during follow-up. 32 patients were included in the study, with a median age of 4.2 years and median follow-up of 6.0 years. 8/32 patients had ANA titers ≥ 1:80 at diagnosis, with 22/32 patients showing rising ANA titers with titers ≥ 1:80 at last follow-up (p =0.001). 10/32 patients had a positive RF at least once during follow-up, compared to 0/32 at diagnosis (p = 0.001).In 5/10 patients, positive RF was documented at least twice, more than twelve weeks apart.Patients treated with TNF antagonists were not significantly more likely to develop positive ANA titers (p = 0.425) or positive RF (p = 0.703).

View Article: PubMed Central - HTML - PubMed

Affiliation: German Center for Pediatric and Adolescent Rheumatology, Gehfeldstrasse 24, 82467 Garmisch-Partenkirchen, Germany.

ABSTRACT

Background: Systemic juvenile idiopathic arthritis (sJIA) is commonly considered an autoinflammatory disease. However, sJIA patients may develop aggressive arthritis without systemic inflammation later in the disease, resembling an autoimmune phenotype similar to other subtypes of JIA. The objective of this study was to determine whether antinuclear antibodies (ANA) and rheumatoid factor (RF) will develop in patients with sJIA over the course of the disease.

Findings: A single center sample of sJIA patients with follow-up of more than one year was obtained. A retrospective chart survey was used to extract demographic and clinical data as well as presence and titers of ANA and RF at diagnosis and during follow-up. 32 patients were included in the study, with a median age of 4.2 years and median follow-up of 6.0 years. 8/32 patients had ANA titers ≥ 1:80 at diagnosis, with 22/32 patients showing rising ANA titers with titers ≥ 1:80 at last follow-up (p =0.001). 10/32 patients had a positive RF at least once during follow-up, compared to 0/32 at diagnosis (p = 0.001). In 5/10 patients, positive RF was documented at least twice, more than twelve weeks apart. Patients treated with TNF antagonists were not significantly more likely to develop positive ANA titers (p = 0.425) or positive RF (p = 0.703).

Conclusions: Patients with sJIA developed increased ANA titers and positive RF over the course of the disease, independent of treatment with TNF antagonists. This might point towards an autoimmune, rather than an autoinflammatory phenotype later in the course of sJIA.

Show MeSH
Related in: MedlinePlus