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pediatric rheumatology_shaw_20151210

Terms in this set (20)

JIA systemic
age
gender
joint distribution
other:
labs:
treatment:
Age: 5 yr
Sex: M=F
Joint Distribution:
Varied, often polyarticular
Other:
Daily fevers x 2 weeks (>102 F)
Rash
Hepatosplenomegaly
Ill-appearing
Labs:
Very high inflammatory markers
Must rule out leukemia
Treatment:
Prednisone
Methotrexate
Biologic (anti- IL-1, anti IL-6, others)
JIA Enthesitis-Related Arthritis
age
gender
joint distribution
other:
labs:
treatment:
Age: School-aged
Sex: M>F
Joint distribution:
Entheses
Large lower extremity joints
Sacro-iliac / hip
Other
Acute anterior uveitis
Inflammatory Bowel disease
Sacroiliitis
Labs
Normal/elevated ESR, HLA B27
Treatment:
NSAID
Methotrexate
Biologic (TNF alpha inhibitor)
JIA Psoriatic

age
gender
joint distribution
other:
labs:
treatment:
Preschool age- school age-teens (F>M)
Arthritis may precede the development of the rash
Joint distribution: Dactylics (circumferential swelling of finger - also happens in sickle cell), oligoarticular or polyarticular
Sacroiliitis, uveitis

-Labs: inflammatory markers may be elevated or normal
-Treatment:
NSAIDs
Methotrexate
Biologic drugs (TNF-alpha inhibitor)
JIA Oligoarticular (4 or fewer joints involved)

age
gender
joint distribution
other:
labs:
treatment:
Age: 2 years
Sex: F>>>>M
Joint Distribution:
Typically lower extremity, large joint
Other:
Afebrile, well, limping with minimal pain, big swelling
Morning stiffness
Labs:
No significant inflammation
ANA - risk of uveitis
RF - neg (don't need)
Treatments:
NSAID, Joint Injection
JIA Polyarticular (5 joints or more)


age
gender
joint distribution
other:
labs:
treatment:
Age: 3 yr or 12 yr
Sex: F>>M
Joint Distribution:
Symmetric swelling, small + large joints
Labs:
Inflammatory markers elevated
(increased ESR, CRP, platelets, mild anemia)
Treatment:
NSAIDs
Methotrexate
Biologics (anti-TNF alpha inhibitor)
what is ANA female w oligoarticular JIA a high risk of?

what can it lead ti?
uveitis
blindness
JIA Polyarticular:
IgM rheumatoid factor negative or positive
-- whats the prognosis?
negative: variable disease course, early onset in 2-5 years of age

positive: increase risk of erosive disease, teenage, female, usually 10% are positive w RF
15 yo African-American female presents with fatigue, weight loss, symmetric polyarthritis of wrists/MCP/PIP. ANA is strongly positive (titer >1:5120). She recently had a bad sunburn on her face which is scarring.
Vital Signs: BP 140/90 T 101 F tachycardic, normal RR and SpO2
Labs: WBC 2.9 (LOW), Hb 8 g/dl (LOW), platelets 80,000 (LOW)
Urinalysis: elevated protein and large blood
BUN: 35 (elevated), Serum Creatinine: 1.5 (elevated)

What diseases should you be worried about?
SLE
how many of the following do you need to diagnose, which disease?

Malar rash
Discoid-lupus rash, Photosensitivity
Oral or nasal mucocutaneous ulceration
Positive ANA
Nonerosive arthritis
Nephritis
Encephalopathy (psychosis/seizures)
Pleuritis or pericarditis
Positive immunoserology (anti-dsDNA, anti-SM antibodies, antiphospholipid antibodies)
Hemotologic disorder (leukopenia, thrombocytopenia, Coombs-positive hemolytic anemia)
4 of 11
for systemic lupus erthematosus
is SLE more common in girls or boys?

which of the ethnicities? african americans, asians, hispanics, caucasians

what's the treatment? prognosis
girls: 8:1

less frequent in caucasians

steroids then DMARDS
depends on major organ system involved
5yo female, w gower sign, sunburn over eyelids and progressively weaker. has eczema. what's the diagnosis?
juvenile dermatomyositis

eczema is actually Gottron's papules

proximal muscle weakness

has vasculitis

has malar rash/
heliotrope rash on eyelids
heliotrope rash
gottron papules
nail fold capillary changes
6 yo male presents with non-pruritic rash on lower extremities and buttocks, ankle and knee swelling with stiffness, poor PO intake for days due to abdominal pain
Also with strange migrating patches of swelling (ear, then trunk, then arm)- now gone

Recent history of acute otitis media

labs
Urinalysis: trace blood and protein
Hemoccult stool: negative
ESR 21
CBC: WBC 15.2, normal Hct and platelets
PT/PTT/INR: normal
Lytes normal


Diagnosis?
Hence Schonlein purpura

rule out thrombocytopenia and DIC
how many of the 4 are needed for diagnosis of what?

Diffuse abdominal pain (colicky)
Any biopsy showing predominant IgA deposition (leukocytoclastic vasculitis, proliferative GN)
Arthritis or arthralgia
Renal involvement (hematuria and/or proteinuria >0.3g/24h)
1 of 4 for HSP
19 month old male if Japanese descent with irritability, fever to 103 F for 5 days
Red rash on trunk
Cracked, fissured lips and tongue is red, with prominent papillae
Reddened conjunctivae without exudate
Swollen appearance to hands and feet
No other focus of infection
Large cervical lymph node


diagnosis?
kawasaki disease
Fever persisting at least 5 days AND
Presence of at least 4 principal features:
1. Changes in extremities
Acute: Erythema of palms, soles; edema of hands, feet
Subacute: Periungual peeling of fingers, toes in weeks 2 and 3
2. Polymorphous exanthem
3. Bilateral bulbar conjunctival injection without exudate
4. Changes in lips and oral cavity: Erythema, lips cracking, Strawberry tongue, diffuse injection of oral and pharyngeal mucosae
5. Cervical lymphadenopathy (1.5-cm diameter), usually unilateral

what disorder?
kawasaki disease

85% of affected children are <5 years of age

Slightly more common in boys than in girls
prognosis of kawasaki disease?
15-25% develop coronary artery abnormalities

small lesions (<5mm) have greatest chance of regression, and larger can progress.
3 year old female presents with left knee swelling
parents don't know when it started- insidious onset
Limping in the mornings, cranky
Will try to run and play by the afternoon
No fevers, no weight loss, no other joints bothering her, no GI complaints, no recent infections, no rashes, no tick bites
No family history of psoriasis or rheumatic diseases
Labs: normal complete blood count, ESR mildly elevated at 26, ANA positive
Xray of left knee suggests left knee effusion, no bony abnormality
Eye exam: bilateral anterior uveitis
juvenile arthritis
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