JIA and Other Rheumatic Diseases in Children. Norma Liburd , RN-BC , MN. Objectives. Define Juvenile Idiopathic Arthritis (JIA) and discuss the diagnostic criteria. Identify the subtypes of JIA and discuss characteristics of each.
JIA and Other Rheumatic Diseases in Children
1/100,000 in Japan
20/100,000 in Norway
300,000 children in the US have chronic arthritis.
Disease associated death rate is
< 1% in Europe
< 0.3% in North America
These numbers represent a
4 Fold to 14 fold Increase in Mortality Rate
Compared with General Population
Causes are cardiac, infection & macrophage activation syndrome
Age at onset <16 years
Duration of Arthritis: 6 weeks
Arthritis in one or more joints defined as swelling or effusion, or presence of two or more of the following signs: (in 1 or more joints)
Limitation of ROM
Tenderness or pain on motion
Exclusion of other diseases
The H&P should determine the labs, not the reverse
Soft tissue swelling
Periosteal new bone formation
Narrowing of cartilaginous
Immune mediated disease
Abnormal cytokine production in the inflammatory pathway (TNF, IL-6, IL-2R, IL-1alpha)
Complex genetic predispositions
Synovial lining is a thin membrane enclosing the joint space. The joint space contains fluid that bathes the joint and reduces friction on motion.
With onset of inflammation, the synovial lining thickens and secretes more fluid, which may remain in the joint and cause swelling. The inflamed lining produces warmth, swelling, and pain.
As inflammation progresses, the synovial lining grows over the cartilage and starts to erode it. As inflammation continues, changes include marked erosion of cartilage, cystic changes and thinning of the bone.
a. Persistentb. Extended
3.Polyarthritis (rheumatoid factor negative)
4.Polyarthritis (rheumatoid factor positive)
5. Psoriatic arthritis
7. Undifferentiated arthritis
a. Fits no other categoryb. Fits more than one categoryFrom Petty RE, Southwood TR, Baum J et al: Revision of the proposed classification criteria for juvenile idiopathic arthritis: Durban, 1997, J Rheumatol 25:199-1994, 1998.
Intermittent fever of systemic JIA in a 3-year-old girl. The fever spikes usually occurred daily in the late evening to early morning (quotidian pattern), returned to normal or below normal, and were accompanied by severe malaise, tachycardia, and rash.
JIA: Oligo – persistent
JIA: Oligo - extended
Complications: cataracts 20%, glaucoma 20%, band keratopathy 16% (end stage scarring)
OligoarticularANA+<7 years at Dx
Oligoarticular ANA+ 7 or older at Dx
Oligoarticular ANA - <7 years at Dx
Oligoarticular ANA –
<7 years at Dx
Q 3-4 months for 7 years, then yearly.
Q 4-6 months for 7 yrs, then yearly.
Q 4-6 months for 4 yrs, then yearly.
Guidelines for ophthalmological screening of children with JIA
High risk – screen every 3 months
Moderate risk – screen every 4-6 months
Low risk: screen every 12 months
All patients considered to be at low risk 7 yr after onset of arthritis; should have yearly ophthalmological exams indefinitely.
All patients are considered to be at low risk 4 years after onset of arthritis, should have yearly ophthalmological exams indefinitely.
All high risk patients are considered to be at medium risk 4 years after onset of arthritis.
Modified from Yancy C, et.al, The Guidelines of the Rheumatology and ophthalmology sections of the AAP. Pediatrics 92:295-296, 2003.
Naproxen 10-20 1000
--Given every 2 weeks by IV, over one hour.
--Dosing interval can be shortened to every week if condition warrants.
IV over 30 minutes: at 0, 2 4 weeks, then every 4 weeks
Approved for children 6 and older as monotherapy or with methotrexate
<75 Kg: 10 mg/Kg
If over 75 Kg: follow adult dosing
Approved for adults: weekly SQ self injection
Thalidomide: 2 mg/kg/day
Mechanism of action probably by effects on TNF and other inflammatory cytokines
Very rigorous patient monitoring
Bone Marrow Transplant
Experimental for severe autoimmune disease unresponsive to conventional therapy
Autologous stem cell transplant being evaluated in small number of children
Infections ~ very risky – high death rate