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Table 1 Juvenile eosinophilic fasciitis cohort patient characteristics. Bolded laboratory values are elevated above reference ranges

From: Juvenile eosinophilic fasciitis: a single center case series

 

Patient 1

Patient 2

Patient 3

Patient 4

Patient 5

Patient 6

Sex

M

F

F

F

F

F

Race

White

White

White

White

White

Black

Ethnicity

Non-Hispanic

Hispanic

Hispanic

Non-Hispanic

Non-Hispanic

Non-Hispanic

Age at diagnosis (years)

14

4

10

12

16

16

Current age (years)

28

14

15

15

18

18

Symptom onset to diagnosis (months)

12

12

6

12

 < 1

1

Follow up duration (months)

58

123

52

28

23

14

Other medical conditions

Prior to EF diagnosis

At or after EF diagnosis

Localized scleroderma

Localized scleroderma,

JIA

Localized scleroderma,

JIA

22q11.23 deletion,

Epilepsy

JIA

Hashimoto thyroiditis,

environmental and food allergies

genetic factor XI deficiency

None

Acquired hemophilia A

Diagnosis labs

AEC (cells/uL, ref: 20–320)

IgG (mg/dL, ref: 641–1353)

CK (U/L, ref: > 295)

Aldolase (U/L, ref: 3.3 – 9.7)

ESR (mm/hr, ref: < 1–20)

700

1990

209

7.1

14

8206

1888

21

12

25

1390

2100

33

11.4

75

865

2221

20

17.3

6

2130

1330

28

15.6

21

390

1720

9

34

Immunomodulation medications

CS

MTX

HCQ

CS

MTX

HCQ

IVIG

TOCI

CS

MTX

HCQ

IVIG

RTX

TOCI

CS

MTX

MMF

CS

MTX

HCQ

IVIG

CS

MTX

RTX

Current rheumatology medications

None

TOCI

HCQ

MTX, MMF

HCQ, MTX

MTX

History of recurrence of EF

No

Yes

Yes

No

No

Yes

  1. Abbreviations: AEC absolute eosinophil count, CS corticosteroids, EF eosinophilic fasciitis, CK creatine kinase, ESR erythrocyte sedimentation rate, HCQ hydroxychloroquine, IgG immunoglobulin G, IVIG intravenous immunoglobulin, JIA juvenile idiopathic arthritis, MMF mycophenolate mofetil, MTX methotrexate, RTX rituximab, TOCI tocilizumab