Lecture 6 - Rheum/MSK Flashcards

1
Q

What are the 5 pediatric rheumatology disorders?

A
juvenile arthritis 
SLE (lupus) 
Juvenile Dermatomyositis
Scleroderma 
Vasculitis 
-Henoch-Schonlein Purpura 
-Kawasaki Disease
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2
Q

JIA

A

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
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3
Q

When do you see JIA?

A

two peaks
1-3 years old
8-12 years old

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4
Q

How do you dx JIA?

A

must be chronic (>6 weeks)
age of onset <16 years
evidence of joint inflammation - redness, swelling, limited ROM, pain

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5
Q

What signs show chronicity of JIA?

A

synovial thickening
bony proliferation
contracture
limb length discrepancy - d/t affect on growth plates

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6
Q

Oligoarticular JIA vs polyarticular JIA

A

oligoarticular <5 joints

polyarticular >5 joints

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7
Q

Classification of JIA

A

oligoarticular <5 joints

polyarticular >5 joints

systemic JIA

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8
Q

Systemic JIA

A

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

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9
Q

What labs will you expect to see with JIA?

A

evidence of systemic inflammation

  • CBC (WBC, RBC, platelets)
  • erythrocyte sedimentation rate
  • C reaction protein
  • serum protein

Antinuclear antibodies
negative rheumatoid factors

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10
Q

How can you tell the difference between JIA and rheumatoid arthritis?

A

JIA has negative rheumatoid factors

RA has positive rheumatoid factors

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11
Q

When do you expect to see ANA?

A

ANA - antinuclear antibodies

highest in <7 years and female pts

MC in oligoarticular JIA

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12
Q

For pts with suspected JIA, what do you need to test for r/o other diseases?

A
joint fluid analysis 
CBC
serologic testing for Lyme 
radiography 
-osteopenia
-osteomyelitis
-malignancy
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13
Q

What is the treatment in JIA?

A

NSAIDs first line treatment

disease modifying agents

  • hydroxychloroquine
  • methotrexate
  • biologic agents
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14
Q

What is the number one treatable cause of blindness in children?

A

uveitis

a complication of JIA

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15
Q

Uveitis

A

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

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16
Q

What is the prognosis of JIA?

A

remission 70-85% within 2-5 years

oligoarticular >90%

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17
Q

JDM

A

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

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18
Q

What is the clinical manifestation of JDM?

A

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

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19
Q

What is the treatment of JDM?

A

immunosuppressive therpay

  • prednisone
  • steroid-sparing agents

stretching to maintain ROM

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20
Q

What is the prognosis of JDM?

A

70% well and functional

10% die

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21
Q

Henoch Schonlein Purpura

A
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%)
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22
Q

What age group is more likely to get Henoch Schonlein Purpura?

A

3-15 years

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23
Q

What is the most common systemic vascultitis in children?

A

Henoch Schonlein Purpura

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24
Q

HSP lab testing

A

elevated inflammatory markers (ESR, CRP, WBC)
MUST NOT HAVE THROMBOCYTOPENIA or else you should be thinking leukemia
normocytic, normochronic anmeia (anemia of chronic illnes)

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25
Pathology of HSP
leukocytoclastic vasculitis with IgA deposition
26
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
27
What is the treatment for HSP?
supportive care NSAIDS for arthritis corticosteroids -for severe GI disease and active renal disease
28
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
29
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 ```
30
What age group is MC to get Kawasaki Disease?
6 months - 5 years
31
Clinical Manifestations of Kawasaki disease
``` fever lasting 5 days red eyes - bilateral conjunctivitis body rash swollen red tongue lymphadenopathy ```
32
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
33
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)
34
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
35
What are Red Flags for potential malignancy?
``` non-articular bone pain back pain bone tenderness severe constitutional sxs night sweats ecchymosis/bruising ```
36
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)
37
What are the risk factors of DDH?
female breech presentation family hx
38
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
39
Galeazzi Sign
when both feet are on the table and one knee is lower than the other seen with DDH
40
Barlow vs Ortolani Tests
Barlow - dislocation Ortolani - reduction
41
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
42
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
43
What age group is most likely to get Avascular Necrosis of Femoral Head?
3-12 years old
44
Flattening of femoral head
seen in late disease of avascular necorsis of femoral head
45
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)
46
Slipped capital femoral epiphysis
orthopedic EMERGENCY common adolescent hip disorder M > F average age 10-16 years risk factors - obesity - trisomy 21 - endocrine disorders
47
Risk factors of slipped capital femoral epiphysis
- obesity - trisomy 21 - endocrine disorders
48
How do pts with slipped capital femoral epiphysis present?
hip or knee pain limp decreased ROM may hold leg in external rotation position
49
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
50
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
51
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
52
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 ```
53
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
54
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
55
Torticollis
shortening of sternocleidomastoid muscle may result in plagiocephally usually first identified in infant because of head tilt Treatment is aimed at increasing ROM
56
Polydactyly
2nd most common hand anomaly behind syndactaly (fusion of the fingers) AA more common and typically involve little finger Caucasians typically involve thumb
57
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
58
Scoliosis
lateral curvature of the spine most often idiopathic 80% >11 years of age
59
What are non idiopathic causes of scoliosis?
cerebral palsy muscular dystrophy MSK disorders
60
When does the AAP recommend scoliosis screening?
Females at age 10 and 12 | Males once at age 13-14
61
Cobb angle
on Xray derived from positions of most tilted vertebrae above and below apex of curve scoliosis
62
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
63
What is the treatment for scoliosis?
bracing for 20-40 degrees | spinal fusion for >40 degrees
64
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
65
When is Nursemaid's Elbow most common?
1-4 years
66
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
67
What is the treatment for Nursemaid's elbow?
place thumb on prominence of radial head apply gentle longitudinal traction supinate forearm fully flex elbow