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478 Chapter 8: Autoimmune Disease<br />

Table 1. Clinical characteristics of the JCA patients before autoHSCT<br />

Patient 1 Patient 2 Patient 3 Patient 4<br />

Sex Female Female Male Female<br />

Age at disease onset 1 year 3 years 3.5 years 5.25 years<br />

Onset form of JCA Systemic Polyarticular Systemic Systemic<br />

ANA - - — +<br />

RF - - — _<br />

Treatment NSAIDs, NSAIDs, NSAIDs, NSAIDs,<br />

corticosteroids, corticosteroids, corticosteroids, oral gold,<br />

IVIG, methotrexate, methotrexate, ATG,<br />

methotrexate, CsA CsA azathioprine<br />

CsA methotrexate,<br />

CsA,<br />

pulses methyl-<br />

prednisolone<br />

Clinical Erosions, Erosions, Erosions, Erosions,<br />

characteristics growth growth growth growth<br />

disturbance disturbance disturbance disturbance<br />

age at AHSCT 6 years, 7 years, 11 years, 11 years<br />

7 months 9 months 2 months<br />

DISCUSSION<br />

The effect of autoHSCT on signs <strong>and</strong> symptoms of chronic joint inflammation<br />

in four patients was very favorable. The initial improvement could be explained by<br />

the immune suppression of the conditioning regimen, but the duration of the<br />

remission, even after recovery of the T cells <strong>and</strong> T cell function, is indicative of an<br />

immune modulatory effect of autoHSCT. However, the follow-up period is too<br />

short for definite conclusions. An interesting phenomenon is the occurrence of a<br />

benign, oligoarticular synovitis in two of the four patients. This occurred simulta-<br />

Table 2. Complications after autoHSCT in children with refractory arthritis<br />

Patient 1<br />

Varicella Zoster at 3 months, transient oligoarticular synovitis at 4 months follow-up<br />

Patient 2<br />

Varicella Zoster at 6 months follow-up<br />

Patient 3<br />

Transient oligoarticular synovitis at 6 months follow-up<br />

Patient 4<br />

Throat infection with hemolytic streptococci B at 4 months follow-up

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