JIA Flashcards
JIA
Most common form of chronic arthritis in childhood
S/S: joint swelling, pain, and limited mobility = restricts child’s participation in activities
JIA classifies all forms of arthritis that begin before age 16, persist longer than 6 weeks, and are of unknown cause
JIA represents and exclusion dx
Role of PT and Goals of PT
Complete a comprehensive exam: identify impairments and determine relationship to observed or reported participation/activity restrictions
Goal of PT: reduce functional impairment, prevent secondary problems, improve activity and participation levels
Interventions must be balanced in rest and exercise
Provide guidelines for choosing activities
Role of PT varies on stage and progression of disease and care setting
Diagnosis and Classification
3 systems used: ACR (american), EULAR (european), and ILAR (international)
No definitive lab tests: DX is made based on clinical presentation
Systemic Arthritis
4-17% of cases
Onset age: throughout childhood, no preferential onset
F = M
Dx marker = spiking a fever of 102.2 or higher that occurs 1-2x daily (morning or evening) for at least 2 weeks with a rapid return to normal or below between spikes
Fever is accompanied by rash found on trunk or limbs, may be seen on face, palms, and soles of feet
Other systemic signs: pleuritis, pericarditis, myocarditis, hepatosplomegaly, and lymphadenopathy
Systemic disease may precede arthritis by several months or years
Systemic signs often subside after initial disease period may recur during exacerbation periods
Oligoarthritis: age of onset, sex ratio, presentation
AKA pauciarticular
27-56% of cases
Onset age: early childhood, peak at 2-4 y/o
F»_space;> M
low grade inflammation in 4 or fewer joints, joint is swollen and warm, not always painful
knee > ankle > elbows
Does not affect hips and small joint of hands
Limited systemic signs, 30% of children develop iridocyclitis (eye inflammation that can lean to functional blindness)
Rx: systemic or topical corticosteroids for inflammation control
Polyarthritis: age of onset, sex ratio, presentation
Rh +
Rh-
Rh +: 2-7%, later childhood or adolescence, F»_space; M, follows disease course similar to RA in adults, may have rheumatoid nodules
Rh-: 11-28%, biphasic distribution, early peak at 2-4 years, later peak at 6-12 years, F»_space; M, nodules less common, fewer joints affected
Arthritis in 5 or more joints
Insidious onset with arthritis progressively noted in more joints
Symmetrical and affects both large and small joints
Can include the cervical spine and TMJ
Joints are swollen and warm
Systemic signs: low grade fever, mild to moderate hepatosplenomegaly and lymphadenopathy
some develop iridocyclitis
persistent arthritis may cause: juxta-articular osteopenia, mm atrophy, weakness, contractures and growth disturbances
Enthesitis- related Arthritis: age of onset, sex ratio, presentation
3-11%
late childhood or adolescence
M»_space; F
Psoriatic Arthritis: age of onset, sex ratio, presentation
2-11%
biphasic distribution, early peak at 2-4 years, later peak at 9-11 years
F > M
Primarily involves distal joints oh hands with psoriasis, usually mild but may lead to general joint destruction
Educate on joint protection
Secondary clinical manifestions
limited joint motion, soft tissue contracture
fatigue
decreased aerobic capacity, reduced excercise tolerance
growth abnormalities (local and general)
osteopenia, osteoporosis and result of long term use of oral steroids
difficulty with ADL’s
possible activity/participation restrictions
gait deviations
Undifferentiated arthritis: age of on set, sex ratio, presentation
11-21% of cases
No specific age of onset or sex ratio
Undifferentiated arthritis: age of on set, sex ratio, presentation
11-21% of cases
No specific age of onset or sex ratio
Incidence and Prevalence
Affects girls more than boys
Between 2-20 per 100,000 at risk in US
Origin and Pathogenesis
Poorly understood, but includes both genetic and environmental components
Autoimmune inflammatory disorder activated by an external trigger in a genetically predisposed host
A viral or bacterial infection precedes disease onset
Suspected genetic predispositions found in MHC region of chromosome 6
Medical Mgmt: Pharmacologic intervention NSAIDS Methotrexate Sulfasalazine Steroids
Want to control arthritis to prevent joint erosion and manage extra articular manifestations
NSAIDS: first line therapy, naproxen, tolmetin, IBU, do not alter disease course treat symptoms
Adverse effects: GI irritation
Methotrexate: m/c disease modifying antirheumatic drug (DMARD) used of poly and systemic JIA
Oral med 1x per week
Monitor liver enzymes due to risk for liver toxicity
Pt with poor prognosis and failure to respond to methotrexate are treated with biological meds that target tumor necrosis factor (cytokine responsible for inflammation)
Entanercept, infliximab, and adalimumab
Sulfasalazine: oligo, DMARD, high risk for toxicity
Systemic glucocorticoid used in systemic who do not respond to other therapies
Steroids do not alter disease course but have potent anti inflammatory effect
Adverse effects: cushings, DM, osteoporosis, myopathy, growth disturbance, obesity, increased susceptibility to infection
Prognosis
Poor articular and functional outcomes in systemic and poly are linked to hip involvement in 1st year of disease
Oligo = best prognosis for joint preservation and function but may develop contractures later on, high risk for eye involvement
Better prognosis associated with early and effective rx to prevent or minimize adverse outcomes
PT Exam
Consider stage, age, motor development before dx, cognitive and emotional development, activities/participation
- Acute Stage: increased impairments resulting in compensatory motor behavior that changes or disappears with appropriate meds–important to communicate with rheumatologist due to importance of meds
** PT intervention will be hindered when a child is not appropriately medicated
Monitor joint motion and integrity- loss of ROM may be first sign to joint damage and increased risk for functional decline
Participation and Activity Restrictions
Depends on extent and duration of disease, developmental stage, resiliency, desire to be independent, and expectations placed on child
S/S of wrist involvement in toddler: incomplete or compensated palmar support (does not bear full weight on palm, bears weight only all palm surface of fingers) in quadruped = limited creeping
S/S of knee involvement in toddler: change in ambulation, shift from walking to bottom scooting
Participation can be impacted by parents discouraging children due to fear of injury, etc.
Restrictions also impacted by services and resources available to family
Children may have difficulty coping due to daily fluctuations in disease
Standardized instruments for examination of activities CHAQ JASI and JAFAR QOL Function Assessment
Childhood Health Assessment Questionnaire (CHAQ): measure of physical function for children 1-19, 30 activities in 8 categories, scores are based on difficulty performing the task in the last week. Disability index is calculated
JA Functional Assessment Index (JASI) and JA Functional Assessment Report (JAFAR): measure physical function
School Function Assessment
QOL: JAQOL questionnaire, PedsQL
JA Functional Assessment Scale (JAFAS): only instrument that measures child’s actual performance, child observed and timed completing 10 tasks
Joint Structure and Function
Cardinal signs of swelling: swelling, end range stress pain, stiffness, and loss of full joint ROM
Reasons for joint enlargement or swelling: intra-articular effusion, synovial hypertrophy, soft tissue edema, or periarticular tenosynovitis, bony overgrowth
Swelling and mm spasm contribute to pain
Contraction deformity= mm spasm
Conception contracture = morphologic change in periarticular soft tissue
Stiffness often due to inactivity in AM or after prolonged sitting = common indicator or disease activity
Chronic inflammation results in abnormalities of joint structure and function. = increased production of synovial fluid stretches and weakness joint capsule and surrounding structures = lig laxity/ joint instability
Pannus = massive overgrowth of synovium that results in invasion of articular cartilage and results in increased inflammation
Joint erosion compromises joint alignment/congruency/stability
Arthritis can occur in any joint, large joints most affected
Joint Exam
Consider stage
- Acute: joint inflammation, joint effusion, lig laxity, joint instability
- Subacute and Chronic: inflammation has become prolonged (>3m), joint swelling due to synovial hypertrophy (doughy feel), loss of joint integrity (joint erosion, loss of alignment)
JC-S- joint counts for swelling
JC-LOM- joint counts for limitation of motion
—Articular Severity Scale: scores global ROM for each joint, average left and right sides
—Global ROM Scores (GROMS): provides single score for global joint function
—Pediatric Escola Paulista de Medicina ROM Scale (pEPM-ROM)- excellent test and retest and interrater reliability, looks at 10 essential joint movements (may not be appropriate for extensive arthritis or patient without 10 joints involved)
Joint effusion- look for bulge sign
AROM can be estimated by observing child perform movements and motor activities. Should also take goni measurements
Muscular Structure and Function
Consider stage:
- Acute: mm spasm or hypertonus (contraction deformity)
- Subacute and chronic: mm atrophy, weakness
Physical Fitness: Health Related (Peak O2 uptake)
Peak O2 uptake in kids with JIA is lower
Lower peak work rate (watts generated on cycle ergometer), peak exercise hear rate, exercise time–may be due to pain/fatigue not necessarily due to cardiopulm limitation
HR and VO2 values were higher during submax exercise- worked a higher percentage of their aerobic capacity
Impaired aerobic capacity not due to severity of arthritis but due to secondary disease symptoms (anemia, mm atrophy, general weakness and stiffness)= poor mechanical efficiency limiting performance
Physical Fitness: Performance Related (mm strength, anaerobic capacity)
should examine mm strength, bulk and endurance at onset and throughout disease course
Bulk= circumferential measurements
Functional strength = observation of ability to perform age appropriate cross motor tasks or ADL’s
Strength testing: can be difficulty bc children break due to pain
Dynamic mm testing: completed when no sign of joint inflammation or joint damage
MM Endurance = have child perform as many reps as possible at 60-80% of 1 Rep Max
6MWT: performance related test also used
weakness in hip ext/abd, knee ext, PT, shoulder abd/flex, elbow flex/ext, wrist ext, grip
Assessment of Pain
Older children, esp newly dx, reported more plain than young children or those with long standing disease—pain more closely related to age rather than disease severity
Pain assessment should be ongoing
- pain hx
- self report if older than 4 y/o
- parent report
- behavioral observation
Validated pain behaviors: bracing, guarding, rubbing. rigidity, and flexing
Self reporting = FACES scale, body map
Over age 7 use numeric pain scale or VAS
Varni/thompson Pediatric Pain Questionnaire (PPQ) = comprehensive assessment of both parent and child report
Growth disturbances and postural abnormalities
Retardation of linear growth associated with: extended periods of active disease and is exacerbated by longer term use of systemic steroids
Accelerated growth may occur during remission if the epiphyses are still open. Accelerated growth of ossification centers may result in bony overgrowth due to increased blood supply
Puberty may be delayed
Osteopenia in appendicular skeleton due to inadequate bone formation, low bone turn over, depressed bone formation—increased risk of bone fracture
LLD as result of unilateral knee arthritis– may result in functional scoliosis (want to confirm or rule out by trying to level out pelvis with lifts)
Permanent closure of growth plates- can be widespread and symmetrical
Microagnathia at TMJ (undersized jaw)
Observe postural alignment in sitting and standing, look for contractures
Torticollis may occur in children with asymmetrical C-spine arthritis
Intervention
Acute: maintain and preserve joint fxn
Subacute/chronic: restoration and compensation of function and activities
GOALS OF PT: maintain/improve fxn, education/support to patient and family
Intervention is individualized to each child
PA and graded exercise important for mgmt of disease and optimal health
Aquatics
Mild to moderate JIA can do land based PT
Adherence to program is key, HEP- incorporate activities into daily routine
provide older children with an opportunity to collaborate
Goals of Intervention
Perform physical tasks for ADL’s and community activities
Increase/improve: aerobic capacity, gait, joint integrity and mobility, postural control, strength/power/endurance
Decrease: pain, swelling/inflammation/restriction
Specific interventions
Aerobic conditioning- pool or low impact wb activities Gait training Body mechanics postural training Strength/power/endurance: AAROM, AROM. resistive exercise, task specific training Flexibility: stretching Balance, coordination, agility training Encourage active healthy living
Mgmt of joint health
RICE- decrease acute swelling and inflammation
NO HEAT—causes increased inflammation
NO US or Diathermy—not used for children
Must balance rest and exercise
Encourage group activities- social, emotional and physical benefits
SLEEP
- restful sleep = decreased morning stiffness
- resting splints for support, function, and pain relief
Daily AROM
–no PROM- may increase inflammation
Static progressive stretching- serial casts, orthoses, dynamic splints—contracture prevention
Want continuous low load stretch
Orthopedic Surgery and Role of PT
Aimed at preserving joint health, ambulation, ability to perform ADL’s
Synovectomy- want to prevent post op contracture, ROM started soon after surgery
Joint arthroplasty- m/c at hip and knee, completed when there is irreversible joint damage and child has significant pain and functional limitations. Follows same precautions as adults
Fitness Training: Aerobic Capcity
important for endurance
should train 2x/week with mod to vigorous intensity (60-85% of HR max) for 45-60 minutes per session for 6-12 weeks
WB activities necessary to maintain bone growth and density
low impact activities improve proprioception, balance, coordination
Fitness Training: Anaerobic Capcity
15 high intensity cycling sprints with each sprint followed by 1-2 min of active rest
Fitness Training: MM Strength
Target mm around and supporting joints with arthritis and adjacent areas
Acute: isometric recommended but avoid prolonged maximal isometric cxn bc can increase intra- articular pressure resulting in decreased blood flow to mm, want submax cxn
- hold submax cxn for 6 seconds while exhaling and inhale during relaxation phase—5-10 reps per day
Dynamic exercise incorporated when inflammation is decreased. Includes concentric and eccentric exercise. Light weights or resistance bands 8-10 reps agains gravity without pain
Self Care
Goal: independence in self care at home, community, and school
Modifications at home or school if needed
Functional mobility
Wb and ambulation critical for bone growth and bone mineral density, joint health, and mm development
Encourage standing,cruising and walking– avoid use of baby walkers bc can promote abnormal gait pattern
Supportive footwear
Few children with JIA required AD
Accomodate LLD
May need walker or crutches if bilateral involvement
May need w/c for long distances
Issues related to school
Modification of schedule or desk, adaptations (writing tools etc.)
May need IEP
Regular participation in PE is encouraged
Prohibited activities: headstands, somersaults, cartwheels, push ups, high impact running or jumping
Recreational Activities
physical and psychosocial benefits
participation may need to be modified based on disease symptoms that day
Should encourage choosing low impact activities
Discourage contact sports
Encourage warm up and cool down
may need adaptive equipment or orthosis to improve joint alignment
