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