Table 1 (abstract P2088). See text for description

Disease

SAVI (lung and skin involvement, systemic inflammation)

Aicardi Goutieres (neurological involvement, rash, fever)

Undefined (epilepsy, arthritis,skin involvement)

Undefined

(recurrent fever, hypertransaminasemia, hemophagocytic lymphohistiocytosis)

Undefined

(hemiplegia)

Undefined

(epilepsy, arthritis,skin involvement)

Undefined (recurrent fever, lymphadenopathy, hypogammaglobulinemia

Clinical improvment after MMF

Resolution of lung involvement

Resolution of fever and rash

Resolution of rash, arthritis, improvement of seizures

Resolution of fever, inflammation and hypertransaminasemia

Improvement of motility

Resolution of arthritis and skin involvement

Resolution of fever and limphadenopathy

Gender

F

F

M

M

M

F

M

Age at onset (yrs)

3 (days)

2

4

7

4

6

16

WBC/ mmc³*

16 /7,4

8,0/8,4

9,8/8,5

1,7/8,4

4,4/8,2

4,4/5,2

6,6/5,9

Hb g/dl *

7,7/8,9

8,5/12,3

12,9/12,8

9,7/13

12,1/10,7

12/12,9

11,5/15,1

PLT/mmc³ *

950 /766

680/320

194/181

56/161

358/421

381/390

218/323

ESR (mm) *

44/40

49/9

38/10

7/6

2/5

24/6

3/3

CRP (mg/dl) *

1,22/0,73

3,13/0,06

5,55/0,32

<0,05/<0,05

<0,05/<0,05

1,15/<0,05

8,34/0,98

SAA (mg/dl)*

95,8/3,62

5,37/2,82

188/8,11

-/1,89

<0,84/-

4,19-0,78

/

C3 (90-180mg/dl)*

154/134

122/108

81/92

83/118

203/170

127/108

53/133

C4 (10-40 mg/dl)*

22/22

10/24

6/8

16/27

38/28

24/14

15/41

Antinuclear antibody*

Absent

1:2560/1:160

1:10.240/1:1280

Absent

Absent

1: 2560 /1:320

Absent

ENA*

pANCA:40/1:20

Absent

Anti dsDNA 1:1280/1: 160

Absent

Absent

Anti dsDNA 1:320/1:40

Absent

IFN signature*

43,2/18,3

42,6/79,8

54,73/64,6

8,66/18,19

40,6/5,95

171,5/13,12

14,34/2,24