Pedia: Rheuma, Allergy and Immunology

Question 0

Adolescent boy presents with difficulty flexing the spine. PE revealed loss of normal spinal curvature. X-ray revealed bamboo spine. What is the HLA type found to be increased in these patients?

Answer 0

HLA-B27

This is classic ankylosing spondylitis.

Question 1

HLA type found in increased frequency in polyarticular JRA

Answer 1

HLA-DR4

Question 2

HLA type found to be increased on pauciarticular JRA

Answer 2

HLA-DR5 and HLA-DR8

Question 3

To make a diagnosis of JRA, onset of symptoms must be at what age?

Answer 3

Onset <16 yrs. Duration of illness must be at least 6 weeks

Question 4

AS is associated with which eye condition in 25% of patients

Answer 4

Iridocyclitis

Question 5

This antibody is more specific for SLE and reflects disease activity.

Answer 5

Anti-dsDNA

Question 6

Most common of the pediatric inflammatory myopathies

Answer 6

Dermatomyositis

Question 7

2 possible infectious triggers for dermatomyositis:

Answer 7

Coxsackie B

GABHS

Question 8

HLA type in dermatomyositis

Answer 8

HLA AG DQA1*0501

Question 9

Heliotropic rash, rash on upper torso and extensor of arms and legs. (-) muscle weakness, dysphagia, abdominal pain.

Work up revealed ANA with speckled pattern.

What would be treatment of choice?

Answer 9

Dx: dermatomyositis
Treatment for skin findings only:
Hydroxychloroquine with low dose oral steroids

Question 10

Indicators of inflammation in dermatomyositis

Answer 10

Neopterin
vWf:AG
CD19+ B cells

Question 11

Mortality rate of dermatomyositis

Answer 11

3%

Question 12

Usually the earliest manifestation of scleroderma is:

Answer 12

Raynaud phenomenon

Question 13

Minor criteria for systemic scleroderma. One has to meet how many to make the diagnosis?

Answer 13

1. Sclerodactyly
2. Bibasilar pulmonary fibrosis
3. Digital pitting scars

Question 14

Kawasaki disease is a vasculitic disease with predilection for which blood vessels.

Answer 14

Medium sized arteries, eg coronary

Question 15

The following are diagnostic criteria for Kawasaki disease except:

A. Changes in mucosa of the oropharynx
B. changes in the peripheral extremities
C. Truncal polymorphous rash
D. CLAD >1.5 cm, unilateral
E. unilateral bulbar conjunctival injection

Answer 15

E. it should be bilateral

Question 16

Skin biopsy of lesions in HSP would reveal ___.

Answer 16

Leukocytoclastic angiitis

Question 17

HLA type found in higher frequency in HSP:

Answer 17

HLA-DRB1*07

Question 18

Normal CD4:CD8 ratio

Answer 18

2:1

Question 19

First line of defense against neonatal herpes virus infection

Answer 19

NK Cells

Question 20

How long does it take for a PMN to mature from a myeloblast? What is its half life in bloodstream? Half life in tissues?

Answer 20

14 days.
Half life:
6h in circulation;
1-2 days in tissues

Question 21

Which interleukin activates and influences the production of eosinophils?

Answer 21

IL-5