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  • Poster presentation
  • 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

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