Rheuma

Question 1

The primary mimics of rheumatic diseases are

Answer 1

infection and malignancy

Question 2

Common symptoms include

Answer 2

joint pain, fever, fatigue, and rash.

Question 3

Common in childhood and are a frequent reason for referral to pediatric rheumatologists.

Answer 3

Arthralgias

Question 4

A stronger predictor of the presence of rheumatic disease and a reason for referral to a pediatric rheumatologist

Answer 4

Arthritis

Question 5

Poor sleep, debilitating generalized joint pain that worsens with activity, school absences, and normal physical and laboratory findings in an adolescent suggests

Answer 5

Pain syndrome

Question 6

Children ages 3 to 10 yr who have a history of episodic pain that occurs at night after increased daytime physical activity that is relieved by rubbing, but who have no limp or complaints in the morning, likely have

Answer 6

Growing pains.

Question 7

Intermittent pain in a child, especially a girl age 3 to 10 yr, that is increased with activity and is associated with hyperextensible joints on exam is likely

Answer 7

Benign hypermobility syndrome.

Question 8

Fatigue and/or stiffness after physical inactivity

Answer 8

Gelling phenomenon

Question 9

Common presenting complaint in juvenile dermatomyositis (JDM)

Answer 9

Fatigue

Question 10

Large vessels: affected vessels are aorta and its proximal branches

Answer 10

Takayasu arteritis

Question 11

Medium vessels: affected vessels are renal arteries, mesenteric vasculature, coronary arteries

Answer 11

Polyarteritis nodosa & Kawasaki syndrome

Question 12

Small vessels: affected vessels are capillaries, arterioles, post capillary venules

Answer 12

HSP, Churg-Strauss syndrome

Question 13

Clinical criteria for Classical Kawasaki disease

Answer 13

fever for 5 days + at least 4 of the principal features: changes in extremities, polymorphous exanthema, bulbar conjunctival injection without exudates, changes in lips and oral cavity & unilateral cervical lymphadenopathy

Question 14

Other name for Kawasaki Disease

Answer 14

Mucocutaneous Lymph Node Syndrome

Question 15

Criteria of Kawasaki Disease

Answer 15

fever for 5 days + Conjunctivitis, Rash, Adenopathy, Strawberry tongue or other oropharyngeal changes, Hand changes

Question 16

What are the acute changes in extremities of Kawasaki disease

Answer 16

erythema of palms, soles or edema of hands or feet

Question 17

What are the acute changes in extremities of Kawasaki disease

Answer 17

periungal peeling of fingers, toes in weeks 2 and 3

Question 18

What are the changes in lips and oral cavity in Kawasaki disease

Answer 18

erythema and cracking of lips, strawberry tongue, diffuse injection of oral and pharyngeal mucosa

Question 19

What is the cut off size of the cervical lymphadenopathy in Kawasaki disease

Answer 19

=/> 1.5 cm

Question 20

Clinical phase of Kawasaki that is characterized by fever and other signs of illness

Answer 20

Acute febrile phase

Question 21

Acute febrile phase usually lasts up to

Answer 21

1-2 weeks

Question 22

Clinical phase of Kawasaki that is associated with desquamation, thrombocytosis, the development of coronary aneurysms

Answer 22

Subacute phase

Question 23

Clinical phase of Kawasaki where highest risk of sudden death in patients with aneurysm

Answer 23

Subacute phase

Question 24

Subacute phase of Kawasaki usually last for about

Answer 24

2 weeks

Question 25

Clinical phase of Kawasaki where it begins when all clinical signs of illness have disappeared and continues until the erythrocyte sedimentation rate (ESR) return to normal

Answer 25

Convalescent phase

Question 26

Convalescent phase of Kawasaki typically last for about

Answer 26

6-8weeks after the onset of illness

Question 27

Initial treatment for Kawasaki disease

Answer 27

Single dose of IVIG (2g/kg) and high dose aspirin (80 to 100mkday divided in 4 doses)

Question 28

What is the dose of IVIG in Kawasaki disease

Answer 28

2g/kg

Question 29

What is the dose of high dose of aspirin in Kawasaki disease

Answer 29

80 to 100mkday divided in 4 doses

Question 30

Why is high dose aspirin administered in Kawasaki

Answer 30

for the anti-inflammatory effect

Question 31

When should be treatment for Kawasaki initiated

Answer 31

within 10 days of fever onset

Question 32

Patients who fail to respond to the 1st dose of IVIG are given what

Answer 32

2nd dose of IVIG 2g/kg

Question 33

Low dose aspirin should be continued as treatment for Kawasaki until

Answer 33

6-8 weeks after disease onset if there are no coronary artery abnormalities or indefinitely if abnormalities are present.

Question 34

What is the dose of low dose aspirin for Kawasaki

Answer 34

3 to 5 mkday SD

Question 35

Why is low dose aspirin still administered in Kawasaki disease

Answer 35

for its antiplatelet effect

Question 36

Clinical criteria for Takayasu Arteritis

Answer 36

Angiographic abnormalities (conventional, CT, or MRA) of the aorta or its main branches and at least 1 of the ff: dec peripheral artery pulse and/or claudication of extremities, BP difference between arms or legs of >10mmHg, Bruits over the aorta and/or its major branches, HPN & elevated acute phase reactant (ESR or CRP)

Question 37

To diagnose myositis, you need at least 2 of the ff:

Answer 37

Proximal symmetric muscle weakness, Elevated Creatining Kinase, AST, LDG or aldolase, EMG findings characteristic of myopathy and denervation & Muscle biopsy documenting myositis/ MRI showing proximal muscle inflammation

Question 38

ANA in Juvenile Dermatomyositis is

Answer 38

Positive

Question 39

Is rarely needed to confirm cutaneous manifestation of Juvenile dermatomyositis

Answer 39

Skin biopsy

Question 40

MC pediatric inflammatory myopathies

Answer 40

Juvenile dermatomyositis

Question 41

Systemic vasculopathy with characteristic cutaneous findings and focal areas of myositis resulting in progressive proximal muscle weakness

Answer 41

Juvenile dermatomyositis

Question 42

Alligator skin like appearance seen in juvenile dermatomyositis

Answer 42

Gottron papules

Question 43

Described as skin over the metacarpal and proximal IPJ may be hypertrophic and reddish pink

Answer 43

Gottron papules

Question 44

Described as periorbital violaceous erythema that may cross over the bridge of the nose

Answer 44

Heliotrope eyelids / heliotrope rash