pediatric rheumatology Flashcards
7 rheumatic diseases that present differently when in kids
Juvenile Idiopathic Arthritis Juvenile-onset Spondyloarthritis Connective tissue diseases Hypermobility syndrome Growing pains Kawasaki disease Henoch-Schonlein Purpura (HSP)
who gets juvenile idiopathic arthritis most?
girls age 1-6yo (but can occur at any age)
after 16yo its considered adult arthritis
pathophys of JIA?
Probably similar immune phenomenon as adult RA but different HLA gene associations
Juvenile idiopathic arthritis can present what 3 different ways?
Systemic onset
Polyarticular onset
Pauciarticular onset
JIA: systemic onset (6 parts)
- spike high fever
- salmon-pink macular rash (koebner’s rash: develops where there is trauma)
- pain in muscles/joints
- enlarged spleen/liver
- high WBCs
- Polyserositis
what is polyserositis?
serous membranes inflamed pericarditis, pleuritis
occurs in systemic onset JIA
JIA: polyarticular onset (what is it + time period)
> 4 joints involved in first 6 months
- Chronic pain and swelling in many symmetrical joints, large and small
- Classically mandible involved, inflammation causes premature closure of growth plate and SHORTENED JAW
JIA: pauciarticular onset (what is it + time period)
- Chronic arthritis of a few joints: <5 for 6 weeks
- Large asymmetrical joint involvement
- few if any systemic features ( except iridocyclitis in girls)
what is iridocyclitis?
- inflammation in different parts of the eyes (iris + ciliary bodies)
- asymptomatic but can cause blindness (if untreated)
- iris isn’t round/symmetric
(in pauciarticular JIA)
what % of kids with pauciarticular JIA get iridocyclitis? does it correlate with severity of arthritis?
30%
does NOT correlate with severity of arthritis
if someone has iridocyclitis, what do they need?
opthamology consult! slit lamp exam every 3-6 months
3 goals of txt for JIA
Restore function
Relieve pain
Maintain joint motion
JIA txt: nonpharm
PT/OT/ orthopedic
JIA txt: pharmacological
NSAIDS
DMARDs (less likely to use)
what are the liquid NSAIDs available ( for young kids) ?
Naprosyn, ibuprofen, diclofenac
DMARDs for JIA (3)
Reluctant to use unless have to because of unknowns about long term effects
- Hydroxychloroquine
- Methotrexate- also helps the eye (the only one)
- TNF inhibitors
prognosis of JIA? __ % get better with age
85%
juvenile onset spondyloarthropathies: how are they like adult? how are they different:
Like adult: a group of HLA-B27 associated disorders
Unlike the adult form: in which inflammatory low back pain is the predominant clinical symptom, the juvenile form has more PERIPHERAL ENTHESITIS and ARTHRITIS (predominantly of the lower extremities) as its main clinical features
connective tissue diseases in children: SLE, dermatomyositis, scleroderma
SLE – just like the adult counterpart
Dermatomyositis – similar to adults with an occasional case of calcinosis; polymyositis is very rare
Scleroderma – typically the “Localized” subtype; linear scleroderma (most commonly affects the legs in children)
hypermobility syndrome: what is it? boys or girls more?
joint able to move beyond normal limits.
mostly in girls
(Consider when presented with joint pain, no inflammation, but increased range of motion)
txt for hypermobility syndrome
Build up muscles around joints with physical therapy
NSAIDS
what are Benign episodic bilateral limb pains?
growing pains (4-12 yo kids)
classic presentation of growing pains
long bones (anterior thigh, knee, calf)
bilateral, symmetric
comes and goes (usually later evening/night)
what is kawasaki dz?
Acute self-limited necrotizing vasculitis of small to medium vessels
Immune system fails to recognize self and develop inflammation of vessel walls
Leads to aneurysms that can rupture or inflammation can fill lumen and cause blockage with no blood flow to all distal tissues
Acute self-limited necrotizing vasculitis of small to medium vessels
kawasaki dz
who gets kawasaki Dz?
80% of cases are kids <4yo
what triggers kawasaki Dz?
possibly virus (b/c it occurs in seasons)
presentation of kawasaki Dz?
kinda non-specific: high fever, enlarged cervical lymph nodes, skin rash, arthralgia
what is the skin rash like with kawasaki Dz?
Polymorphous exanthem, redness and swelling of hands and feet with desquamation (probably the most specific of the presenting symptoms)
PE of kawasaki (4)
Conjunctivitis
Skin rash
Strawberry tongue, erythema of lips ( Inflammation of oral mucosa)
S3, S4, CHF, pericarditis
while the Dx of kawasaki is clinical, what test should be done?
evaluate heart for cardiac complications with echo and angiography
txt for kawasaki ? (why is early txt important?)
IVIG-intravenous immunoglobulin
Aspirin high doses
*early txt to prevent cardiac complications!
what % of untreated kawasaki Dz get heart complications?
20-30%
can lead as far as myocarditis, pericarditis, MI, death
what is Henoch-Schonlein Purpura?
small vessel Vasculitis affecting skin, GI, and kidney
Self-limiting
what is the most common vasculitis to occur in children?
henoch-schonlein purpura
who usually gets Henoch-schonlein purpura?
boys, usually after viral infection
pathophys of henoch-schonlein purpura
IgA deposition in small blood vessels of skin (leukocytoclastic vasculitis), & sometimes GI tract & kidney
4 clinical manifestations of henoch-schonlein purpura
- Palpable purpura- purple raised rash
- Arthritis/arthralgias (knee & ankle)
- Abdominal pain (GI bleed in 25%)
- hematuria, proteinuria (renal disease)
Dx of henoch-schonlein purpura ?
Nonspecific tests of inflammation are present: Elevated ESR, WBC, mild anemia
- usually made clinically
- if VERY sick, you can bx the kidney for Dx confirmation
what are the supportive and symptomatic txts for henoch-schonlein purpura?
Supportive – rest, hydrate, and pain control
Symptomatic – NSAIDs, acetaminophen for abdominal pain and joint pain
prognosis of henoch-schonlein purpura
GREAT!
Get better in 1-6 weeks. Complications are uncommon
