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  • Open Access

Determing an “autoimmune” phenotype in systemic JIA

Pediatric Rheumatology20119 (Suppl 1) :P150

https://doi.org/10.1186/1546-0096-9-S1-P150

  • Published:

Keywords

  • Public Health
  • Arthritis
  • Retrospective Analysis
  • Juvenile Idiopathic Arthritis
  • Phenotypic Characteristic

Background

Data from research, clinics and response to treatment point out to an autoinflammatory pathogenesis in Systemic onset Juvenile Idiopathic Arthritis (SoJIA). However a subgroup of patients present with c hronic a ctive course of disease, destructive p olyarthritis and production of a utoantibodies (CAPA-subtype) not responsive to anti IL-1 treatment.

Aim

Determine phenotypic characteristics of the CAPA-subtype of SoJIA.

Methods

Retrospective analysis in a cohort of 35 SoJIA patients well characterized according clinical course and ANA, Rheumatic factor and ANCA.

Results

Autoantibodies have been found in 25,7% of the patients, with ANA being the most frequent auto-AK (table 1). There was a significant difference according the age of onset. Moreover females and carditis have been observed more frequently in the AK-pos. group.

SoJIA-Patients

35

Observation (month)

mean 69 (range 1-262)

 

n

%

n

%

Autoantibodies

pos

neg

 

9

25,7

26

74,3

ANA

6

17,1

  

RF

2

5,7

  

ANCA

3

8,6

  
 

percentage for each group

male

3

33,3

11

42,3

female

6

66,6

15

57,7

onset age (years)

6,03

 

3,54

 

relapses

4

 

3,6

 

joints at onset

2,1

 

3,1

 

joints when AK pos

7

   

MAS

1

 

1

 

Carditis

6

66,7

13

50

SAA

2 of 4

 

8 of 12

 

Amyloidosis

1

 

1

 

Conclusion

There is evidence for an autoimmune course of disease in SoJIA in a subset of patients with chronic polyarthritis but without relapses of autoinflammation. These patients probably will need a switch in treatment to prevent further joint destruction.

Declarations

Acknowledgement

supported by the BMBF (AID-Net 01GM0907)

Authors’ Affiliations

(1)
Deutsches Zentrum für Kinder- und Jugendrheumatologie, Garmisch-Partenkirchen, Germany

Copyright

© Fischer et al; licensee BioMed Central Ltd. 2011

This article is published under license to BioMed Central Ltd. This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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