Pediatric Rheuma From www.rheumatology.com

Question 1

A four year-old white female is brought to your office because her mother says, for the last two months, she has limped every morning for about an hour, which eventually improves. She has had no fevers or rash. On your exam, she is happy and playful but walks with a limp. She has swelling of her left knee and left ankle. Her CBC, ESR, and CRP are normal. Her rheumatoid factor, anti-CCP antibodies, and Lyme studies are negative. She has an ANA that is positive at 1:160

Diagnosis?

Answer 1

JIA

Question 2

Differential Diagnosis of Patient with JIA:

Patient with significant joint swelling, especially of the knee

Answer 2

Lyme Arthritis

Question 3

Differential Diagnosis of Patient with JIA:

not associated with a morning preponderance of symptoms

Answer 3

Trauma

Question 4

Differential Diagnosis of patient with JIA:

With more acute in presentation and be associated with more systemic symptoms;

Answer 4

Septic Arthritis and Osteomyelitis

Question 5

Differential Diagnosis of patient with JIA:

________________ , such as pediatric lupus, juvenile dermatomyositis or systemic vasculitis, which would have more extraarticular features

Answer 5

Systemic Autoimmune Disease

Question 6

Differential Diagnosis of patient with JIA:

Non-_________ causes of joint pain, including slipped capital femoral epiphysis and idiopathic avascular necrosis, which would be associated with activity-related pain, rather than morning stiffness.

Answer 6

inflammatory

Question 7

Significance of :

• Negative RF
• Negative anti-CCp Ab
• Normal inflammatory markers

Answer 7

More aggressive course

JIA patients with just a few joints affected often have normal inflammatory markers

Question 8

Major commorbidity with JIA

Answer 8

Uveitis

Question 9

Significant Risk Factors for UVEITIS:

_____ gender
_____ age
RF ____
recent development of disease

Answer 9

Significant Risk Factors for UVEITIS:

Female gender
Young age
RF Positive
recent development of disease

Question 10

Patients with ENTHESITIS related arthritis get acute uveitis, characterized as:

Answer 10

Red, Painful, Photophobic eye

Question 11

Patient with Systemic JIA, with fever altered mental status, Transaminitis and Pancytopenia

Answer 11

Macrophage activation syndrome

most sever complication of sJIA

Question 12

Laboratory hallmarks of MAS:

Hyperferritinemia
Cytopenia
Transaminase \_\_\_\_\_\_\_\_
Paradoxically \_\_\_\_ or \_\_\_\_\_ ESR
\_\_\_\_\_\_  Triglycerides
\_\_\_\_\_\_\_\_\_\_\_ PT and PTT

Answer 12

Hyperferritinemia
Cytopenia
Transaminase elevation
Paradoxically low or normal ESR
Elevated Triglycerides
Prolonged PT and PTT

Question 13

You get called to a consult in the ED because there is a nine year-old Latino male who has a red raised rash on his legs and buttocks. When you see him, you also notice that his knee is swollen, but he seems to be walking without difficulty. Otherwise he looks happy and well. His CBC, ESR, and coagulation studies are normal.

Answer 13

HSP

IgA mediated vasculitis

Question 14

Histologic Features of HSP

Answer 14

Leukocytoclasis
-vascular damage caused by nuclear debris form infiltrating neutrophils

IgA deposition

Question 15

Major clinical manifestion of HSP:

Tetrad

Answer 15

1. Purpura
2. Arthritis
3. GI involvement
4. Renal involvement

Question 16

A five year-old boy has had seven days of fever up to 104F. Over the last two days, he has developed a large right-sided cervical lymph node, a maculopapular rash, and injected sclera. His lips are red and cracked.

Answer 16

Kawasaki Disease

Medium vessel vasculitis: esp coronary arteries

Question 17

Clinical criteria for Kawasaki disease:

Fever + at least 4 of

Answer 17

1) Bilateral conjunctival injection (occurs in 80-90% of patients; typically limbic-sparing); (2) Oropharyngeal mucous membrane changes (80-90%; injected and / or fissured lips, strawberry tongue, injected pharynx);
(3) Peripheral extremity changes (80%; Erythema and / or edema of the hands and feet or periungual desquamation);
(4) Polymorphous rash (>90%);
(5) Cervical lymphadenopathy with node >1.5 cm (50%)

Question 18

Major complication of Kawasaki Disease

Answer 18

Coronary aneurysm

Question 19

In clinic, a 14 year-old female is seen for erythematous plaques over her knuckles,elbows, and knees. She also has a purplish rash on her eyelids and some erythema on her cheeks. When you ask her to stand up so you can examine her back, she has trouble getting up out of the chair.

Answer 19

Juvenile Dermatomyositis

Question 20

Children with JDM or Adult DM?

Calcinosis, lipodystrophy, and gastrointestinal and cutaneous ulcerations.

Answer 20

Children with JDM

Question 21

Children with JDM or Adult DM?

(+) Anti-synthetase antibodies

Answer 21

Children 0-5%

Adult 20-25%

Question 22

A patient with JDM presents with acute onset of abdominal pain.

Diagnosis?

Answer 22

Visceral Vasculopathy which may lead to death.

Severe abdominal pain, with or without melena or hematemesis, may signify vasculopathy of the gastrointestinal tract and resulting tissue ischemia or mesenteric infarction.

Question 23

The presence of ___________ signifies a patient who is at higher risk for visceral ulceration and may warrant more aggressive therapy with cytotoxic agents.

Answer 23

Cutaneous ulceration

Question 24

Type of JIA:

Four or less joints affected in the first 6 months of disease.

Answer 24

Oligoarticular

Typically younger (2-5 year) females. 
Associated with the best prognosis but highest risk of uveitis.

Question 25

Type of JIA:

More than four joints in the first 6 months of disease.

Answer 25

RF-negative polyarticular

Bimodal (2-5 yo and 11-13 yo) age distribution.

Also high risk of uveitis.

Question 26

Type of JIA:

Very similar to adult RA in aggressiveness and prognosis.

Answer 26

RF-positive polyarticular

Also known as Childhood-onset Rheumatoid Arthritis.

Question 27

Type of JIA:

Associated with quotidian (once daily, typically evenings) fever
Evanescent (less than 24 hours) rash, Serositis,
Lymphadenopathy, or
Hepatosplenomegaly.

Answer 27

Systemic

Question 28

Type of JIA:

Similar to adult psoriatic arthritis.

Answer 28

Psoriatic

Question 29

Type of JIA:

Similar to spondyloarthritis seen in adults.

Answer 29

Enthesitis-related