Lecture 6 - Rheum/MSK Flashcards
What are the 5 pediatric rheumatology disorders?
juvenile arthritis SLE (lupus) Juvenile Dermatomyositis Scleroderma Vasculitis -Henoch-Schonlein Purpura -Kawasaki Disease
JIA
Juvenile Idiopathic Arthritis (JIA)
fromerly known as juvenile rheumatoid arthritis
MC childhood arthritis 1:1000 children (pretty common) two peaks: 1-3 years old 8-12 years old
When do you see JIA?
two peaks
1-3 years old
8-12 years old
How do you dx JIA?
must be chronic (>6 weeks)
age of onset <16 years
evidence of joint inflammation - redness, swelling, limited ROM, pain
What signs show chronicity of JIA?
synovial thickening
bony proliferation
contracture
limb length discrepancy - d/t affect on growth plates
Oligoarticular JIA vs polyarticular JIA
oligoarticular <5 joints
polyarticular >5 joints
Classification of JIA
oligoarticular <5 joints
polyarticular >5 joints
systemic JIA
Systemic JIA
aka Still’s Disease
think of it as a systemic inflammation that PRECEDES the onset of arthritis
typical fever curve
daily and diurnal temperature spike over 39C
returns quickly to below baseline
child feels well between temperature spikes
Characteristic Rash
-dematographia
-papulomacular rash
migratory and quickly fades
What labs will you expect to see with JIA?
evidence of systemic inflammation
- CBC (WBC, RBC, platelets)
- erythrocyte sedimentation rate
- C reaction protein
- serum protein
Antinuclear antibodies
negative rheumatoid factors
How can you tell the difference between JIA and rheumatoid arthritis?
JIA has negative rheumatoid factors
RA has positive rheumatoid factors
When do you expect to see ANA?
ANA - antinuclear antibodies
highest in <7 years and female pts
MC in oligoarticular JIA
For pts with suspected JIA, what do you need to test for r/o other diseases?
joint fluid analysis CBC serologic testing for Lyme radiography -osteopenia -osteomyelitis -malignancy
What is the treatment in JIA?
NSAIDs first line treatment
disease modifying agents
- hydroxychloroquine
- methotrexate
- biologic agents
What is the number one treatable cause of blindness in children?
uveitis
a complication of JIA
Uveitis
number one treatable cause of blindness in children
a complication of JIA
inflammation of the iris, ciliary body and choroid
tends to be asymptomatic in
get screening in pts with chronic arthritis
What is the prognosis of JIA?
remission 70-85% within 2-5 years
oligoarticular >90%
JDM
juvenile dermatomyositits
0.1:100,000 (rare)
peak of onset 4-10 years
F>M
Heliotrope Rash (face)
Gottron’s Papules (hand)
Periungual erythema
hip and shoulder gridles
abdominal and neck muscle weakness
gour sign - muscular dystrophy in their thigh and hips
What is the clinical manifestation of JDM?
Heliotrope Rash (face)
Gottron’s Papules (hands)
Periungual erythema
hip and shoulder gridles
abdominal and neck muscle weakness
gour sign - muscular dystrophy in their thigh and hips
inflammatory myositis
What is the treatment of JDM?
immunosuppressive therpay
- prednisone
- steroid-sparing agents
stretching to maintain ROM
What is the prognosis of JDM?
70% well and functional
10% die
Henoch Schonlein Purpura
most common systemic vasculitis of childhood MC in 3-15 years M > F 13:100,000 peaks in winter
100% get palpable purpura seen on the legs and buttocks (dependent areas) edema Arthralgias/arthritis (80%) -usually in the lower extremity joints -acute and very painful
GI involvement (50%) Renal involvement (35%)
What age group is more likely to get Henoch Schonlein Purpura?
3-15 years
What is the most common systemic vascultitis in children?
Henoch Schonlein Purpura
HSP lab testing
elevated inflammatory markers (ESR, CRP, WBC)
MUST NOT HAVE THROMBOCYTOPENIA or else you should be thinking leukemia
normocytic, normochronic anmeia (anemia of chronic illnes)
Pathology of HSP
leukocytoclastic vasculitis with IgA deposition
EULAR consensus criteria for HSP
classical palpable non-thrombotyopenic purpruic rash and any one of the following:
- arthritis or arthralgia
- abdominal pain and/or GI blood loss
- any biopsy with predominant IgA deposition
What is the treatment for HSP?
supportive care
NSAIDS for arthritis
corticosteroids
-for severe GI disease and active renal disease
What is the prognosis of HSP?
66% resolve within one month
at least 50% have recurrence -usually with 6 weeks but can be up to 2 years
Kawasaki Disease
2nd most common vascultitis of childhood
MC in East Asia
<5 years old MC - uncommon before 6 months
february to may MC
fever lasting 5 days red eyes -bilateral conjunctivitis body rash swollen red tongue lymphadenopathy
What age group is MC to get Kawasaki Disease?
6 months - 5 years
Clinical Manifestations of Kawasaki disease
fever lasting 5 days red eyes - bilateral conjunctivitis body rash swollen red tongue lymphadenopathy
FEBRILE
dx criteria of Kawasaki disease
F- fever E -enanthem B- bulbar conjunctiviits R - rash I - internal organ involvement L - lymphadenopathy E - extremity changes
if a pt presents with 4 or more –suggestion of Kawasaki even if they haven’t had fever for the 5 days
What is the treatment for Kawasaki disease?
goal: prevent CAD
IVIG within 7 days of fever to better prevent CV complications
aspirin to help reduce pain and fever and decrease risk of blood clots (only time kids every get ASA)
Growing pains
occur in 10-20% of all children
peak age of onset 3-7
worse after activity and late in the day
check CBC to r/o leukemia
tx: stretching and reassurance
What are Red Flags for potential malignancy?
non-articular bone pain back pain bone tenderness severe constitutional sxs night sweats ecchymosis/bruising
DDH
developmental dysplasia of the hip
newborns have ligamentous laxity that can lead to spontaneous dislocation of the hip
1-2:1000
L > R (33% bilateral)
What are the risk factors of DDH?
female
breech presentation
family hx
What are the clinical manifestations of DDH?
asymmetrical creases on the back of thighs and buttocks
Galeazzi Sign - when both feet are on the table and one knee is lower than the other
Galeazzi Sign
when both feet are on the table and one knee is lower than the other
seen with DDH
Barlow vs Ortolani Tests
Barlow - dislocation
Ortolani - reduction
What is the treatment for DDH?
based on age at dx
goal is stable reduction that results in normal development of hip
Pavlik harness - up to 6 months –hips should be reduced 2 weeks after the start of harness
Avascular Necrosis of Femoral Head
aka legg calve perthes disease
caused by interruption of blood flow to the capital femoral epiphysis
typically presents in pts 2-12 years old
M > F
atraumatic, painless limp
may have some milk or intermittent hip/groin pain, anterior thigh or knee pain
common complication of sickle cell disease
What age group is most likely to get Avascular Necrosis of Femoral Head?
3-12 years old
Flattening of femoral head
seen in late disease of avascular necorsis of femoral head
What is the treatment of avascular necrosis of femoral head?
should be followed by orthopedics
pain control and restoration of hip range of motion
braces, surgery for containment of the femoral head in the acetabulum (usually for kids >6)
Slipped capital femoral epiphysis
orthopedic EMERGENCY
common adolescent hip disorder
M > F
average age 10-16 years
risk factors
- obesity
- trisomy 21
- endocrine disorders
Risk factors of slipped capital femoral epiphysis
- obesity
- trisomy 21
- endocrine disorders
How do pts with slipped capital femoral epiphysis present?
hip or knee pain
limp
decreased ROM
may hold leg in external rotation position
What is the treatment for slipped capital femoral epiphysis?
go non-weight bearing with URGENT referral to orthopedics
surgery - internal fixation with cannulated screw
goal: to prevent further slippage, enhance physeal closure, minimize complications
Osgood-Schlatter disease
common among athletes
common cause of knee pain at insertion of patellar tendon on the tibial tubercle
microfracture or partial avulsion in the ossification center on developing tibial tubercle
usually occurs after growth spurt
M > F
11 years for girls
13-14 for boys
What is the clinical manifestation of osgood-schlatter disease?
pain during and after activity
may have tenderness and local swelling over tibial tubercle
can develop bony enlargement of the tibial tubercle
What is the treatment for osgood-schlatter disease?
rest and activity modification NSAIDS, ice lower extremity flexibility and strengthening exercises course typically benign may last 1-2 years
In Toeing vs Out Toeing?
In toeing more common in girls
worse around 4-6 years of age
internal femoral torsion >2 years
internal tibial torsion <2 years
out toeing
external tibial torsion
most of these go away on their own
tx is reassurance
Genu Varum vs Genu Valgum
Varum - bowleg
Valgum - knock knees
knock knees MC in 3-4 year olds and typically resolves by 5-8 years of age
surgery only if they’re not able to walk normally
Torticollis
shortening of sternocleidomastoid muscle
may result in plagiocephally
usually first identified in infant because of head tilt
Treatment is aimed at increasing ROM
Polydactyly
2nd most common hand anomaly behind syndactaly (fusion of the fingers)
AA more common and typically involve little finger
Caucasians typically involve thumb
What are the 3 different types of polydactyly?
Type 1 - soft tissue only –tx: ligation or electrocautery
Type 2 - duplicate finger, including bones/joints -surgery
Type 3 - duplication of finger AND metacarpal –tx: surgery
Scoliosis
lateral curvature of the spine
most often idiopathic
80% >11 years of age
What are non idiopathic causes of scoliosis?
cerebral palsy
muscular dystrophy
MSK disorders
When does the AAP recommend scoliosis screening?
Females at age 10 and 12
Males once at age 13-14
Cobb angle
on Xray
derived from positions of most tilted vertebrae above and below apex of curve
scoliosis
What are the major risk factors of scoliosis?
F > M
potential for future growth (SMR - if they’re presenting early in SMR its more concerning of progression in curve)
Magnitude of curve at time of dx
What is the treatment for scoliosis?
bracing for 20-40 degrees
spinal fusion for >40 degrees
Nursemaid’s Elbow
axial traction on a pronated forearm with elbow in extension
annular ligament slips over head of radius and becomes trapped in radiohumeral joint
by the age of 5 this annular ligament has strengthened enough to not move over the radius
When is Nursemaid’s Elbow most common?
1-4 years
How to pts with Nursemaid’s elbow present?
not using arm
child holds arm close to body with elbow fully extended or slight flexed and forearm pronated
pain with active supination
little distress unless attempting to use the arm
What is the treatment for Nursemaid’s elbow?
place thumb on prominence of radial head
apply gentle longitudinal traction
supinate forearm fully
flex elbow
