Rituximab resistant evans syndrome and autoimmunity in Schimke immuno-osseous dysplasia

Table 2 Evolution of the immune and autoimmune status of the proposita

	Test results at the indicated ages
Age (years)	3^§	4	5^¶
Test (normal values)
IgG (g/l) (5.53-10.20)	7.72	12.1	1.63
IgA (g/l) (0.33-0.91)	0.83	1.66	0.43
IgM (g/l) (0.47-1.67)	0.97	2.19	3.49
C3 (g/l) (0.83-2.25)	1.06	1.47	1.41
C4 (g/l) (0.14-0.35)	0.18	0.10	0.11
ANA	negative	negative	negative
ANCA	negative	negative	negative
ACLA (GPL/ml) (0.8-11)	7.0	6.3	4.3
ENA screening	negative	negative	NM
ATA IgA (U/ml) (0-10)	2.09	2.11	NM
CD4 absolute count x10³/ml (35-51%)	130 (12%)	200 (13%)	70 (50%)
CD8 absolute count x10³/ml (18-40%)	20 (1.8%)	170 (11%)	60 (44%)
CD4/CD8 (1-3%)	6.7%	1.2%	1.1%
CD19 absolute count x10³/ml (9-35%)	0.440 (45%)	530 (35%)	0 (0%)*

^§While receiving prednisone (60 mg/m²/day)
^¶Nephrotic syndrome worsened and proteinuria increased during the 5th year of life.
*After rituximab treatment.

ISSN: 1546-0096