rheumatology peds

Question 1

definition of henoch schonlein purpura

Answer 1

IgA mediated vasculitis that involves the skin, joints, GI tract, and kidneys

Question 2

What are the clinical features of HSP?

Answer 2

• preceding viral or URI
• skin issues: petechiae/palpable purpura on butt and lower extremities
• edema
• GI (colicky abd pain, bleeds, intussusception) and joint symptoms (arthritis/arthralgia)
• renal symptoms are variable; may not be apparent until 3 mo after initial presentation

Question 3

Diagnosis of HSP

Answer 3

history, physical. elevated serum IgA in 50%. Normal platelet counts in spite of petechiae and purpura

Question 4

Kawasaki disease. Mean age.

Answer 4

acute febrile vasculitis. involves heart, skin, mucous membranes, GI tract, CNS, joints, vascular bed. most common cause of acquired heart disease in US kids
mean age 18-24 months.

Question 5

kawasaki dx

Answer 5

fever >102 for at least 5 days AND 4/5:
-bilateral conjunctivitis without exudate
-oropharyngeal changes: red swollen lips
-cervical adenopathy (node >1.5 cm)
-rash
-distal extremity changes:
first 7-10 days- edema; later peeling around nail beds

Question 6

Important complications of kawasaki disease

Answer 6

• coronary artery aneurysms in 20% usually around day 7-14
• sterile pyuria
• hydrops of the gallbladder

Question 7

Time course of kawasaki

Answer 7

acute phase: wks 1-2
subacute phase- weeks to months
convalescent phase: weeks to years

Question 8

management of kawasaki disease

Answer 8

• IVIG. Initiated within 10 days of fever decreases coronary artery dilation
• ASA (high dose in acute phase, low dose in subacute phase for antiplatelet effects)
• Serial echos
• possibly steroids? if unresponsive to IVIG, but not comon practice

Question 9

Juvenile inflammatory arthritis

Answer 9

chonic joint inflammation in kids, with or without extra-articular involvement. most common peds rheumatic disease.

Question 10

epidemiology of JIA

Answer 10

onset 1-3 years. very rare before 6 mo.

Question 11

Pauciarticular JIA definition

Answer 11

less than or equal to 4 joints invovled.
may be early or late onset
articular involvemnt need not be symmetric

Question 12

early onset pauciarticular JIA

Answer 12

female predominant
usually between 1-5 yrs
75% with positive ANA
High risk of chronic uveitis—> can lead to blindness

Question 13

late onset pauciarticular JIA

Answer 13

male predominant (only JIA that is male predominant)
presentation >8 yrs
HLA-B27 positive
hips and sacroiliac joints commonly involved
uveitis rarer

Question 14

polyarticular JIA: definition. how are these diseases categorized? epidemiology?

Answer 14

over 4 joints involved. systemic features are rare.
may be categorized as fheumatoid factor positive vs. negative (RF is IgM against IgG).
female predominant
articular involvement usually symmetric

Question 15

RF negative vs. positive polyarticular JIA

Answer 15

negative: early and late in childhood
positive: kids >8, usually more severe than RF negative, higher risk of arthritis and rheumatoid nodules

Question 16

systemic JIA

Answer 16

aka still’s disease

• high spiking fevers (over 39)
• salmon colored rash. evanescent (occurs with fevers) and nonpruritic
• hepatosplenomegaly
• LAD
• fatigue, anorexia, failure to thrive, serositis
• other manifestations possible- CNS, myositis, tenosynovitis

Question 17

management of JIA

Answer 17

NSAIDS, immunomodulatory meds (steroids, MTX, sulfasalazine, hydroxychloroquine)