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Volume 22, Number 10—October 2016
CME ACTIVITY - Synopsis

Infection-Related Death among Persons with Refractory Juvenile Idiopathic Arthritis

Mario AbinunComments to Author , Jonathan P. Lane, Mark Wood1, Mark Friswell, Terence J. Flood, and Helen E. Foster
Author affiliations: Great North Children's Hospital, Newcastle upon Tyne, UK (M. Abinun, J.P. Lane, M. Friswell, T.J. Flood, H.E. Foster); Newcastle University, Newcastle upon Tyne (M. Abinun, H.E. Foster); Leeds General Infirmary, Leeds, UK (M. Wood)

Main Article

Table 1

Disease characteristics and treatment at time of death for 2 children with JIA, Newcastle upon Tyne, United Kingdom*

Characteristic Patient 1 Patient 2
Year of disease onset/year of death 1996/2004 2000/2004
Age at So-JIA diagnosis/at death, y
 5/13
 2/6
JIA symptom or sign, ever
Fever Yes Yes
Rash Yes Yes
Arthritis Yes Yes
Lymphadenopathy No No
Hepato/splenomegaly Yes Yes
Serositis No No
Macrophage activation syndrome
 No
 Yes
Disease remission, ever No No
JADAS-10†
 20–35
 12–32
Treatment
Corticosteroids‡ Yes§ Yes§
Methotrexate¶ Yes§ Yes§
Cyclosporin A No Yes#
Intravenous immunoglobulins** No Yes††
Etanercept Yes‡‡ No
Infliximab
 Yes§,§§
 Yes§¶¶
Side effects of treatment
Cushingoid Yes Yes
Cataracts Yes No
Osteoporosis Yes Yes
Osteoarticular tuberculosis Yes No
Stunted growth Yes Yes

*JADAS, Juvenile Arthritis Disease Activity Score; JIA, juvenile idiopathic arthritis; So-JIA, systemic JIA.
†Linear sum of the scores of the 4 JADAS components (0–40): physician global assessment of disease activity (measured on a 10-cm visual analog scale; 0 = no activity and 10 = maximum activity); parent/patient global assessment of well-being (measured on a 10-cm visual analog scale; 0 = very well and 10 = very poor); count of joints with active disease (0–10); erythrocyte sedimentation rate (actual value/10; range 0–10) (13).
‡Methylprednisolone pulses (intravenous 30 mg/kg/day; maximum. 1 g) given as a 3-day course or a single dose (weekly or otherwise), when indicated based on clinical decision (for treating a disease flare). Prednisolone maintenance dose (orally 0.5–1 mg/kg/d) with adjusting and aiming for alternate day regimen whenever possible depending on the clinical course or disease activity.
§Treatment at time of death.
¶Subcutaneously 15 mg/m2/wk.
#March–December 2002.
**2 g/kg/mo.
††December 2003–January 2004.
‡‡0.4 mg/kg/wk during March–May 2000 (12) and again during May 2003–January 2004.
§§5 mg/kg single dose, February 2004.
¶¶6 mg/kg/mo during December 2002–December 2003.

Main Article

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1Current affiliation: Leeds General Infirmary, Leeds, UK.

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Page updated: September 15, 2016
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