Pediatric Board Review - Rheumatology

Front 1

Which of the following can cause arthritis in kids?
Gonnhea
Genetic syndromes
Lyme
Osteomyelitis
Viral (Toxid synovitis)
Evasive infection (ie septic arthritis)

Back 1

All of them

Front 2

Which of the following are normal and which are positive in Ankylosing Spondylitis?
HLA B27
ANA
RF
ESR

Back 2

HLA B27 is positive
ANA normal
RF normal
ESR elevated

Front 3

A 16 yo boy has night pain in his knee which is releived with exercise. On x-ray you see a bamboo spine. What is the diagnosis?

Back 3

Ankylosing spondylitis

Front 4

A 15yo boy has back and leg pain along with weight loss and low grade fever. His x-ray looks like this. What is his diagnosis and treatment?

Back 4

Bamboo spine. Ankylosing spondylitis, ossification fo annulus fibrosis. HLA-B27.

Rx: exercise, NSAIDS, Methotrexate, etanercept, infliximab

Front 5

A child has apthous ulcers, genital, GI ulcers, uveitis and arthritis. His ANA and RH are normal but ESR and CRP are high. What is the diagnosis and treatment?

Back 5

Behcet's syndrome

Think oral, genital and stomach ulcers while eating a baguette

Front 6

A child younger than ten has this rash as well as shiny skin on extensor surfaces of the extremities (Grotton Papules). She is clumsy and has been having difficulty climbing steps and getting dressed. What is the diagnosis and first step in evaluation?
A) EMG
B) CT
C) Skin biopsy
D) CK levels
E) MRI

Back 6

Dermatomyositis and D) CK level.

Front 7

A 4yo child presents with poor wound healing and multiple bruising. She has hyper-elastic skin and joints. What is her diagnosis?

Back 7

Ehlers-Danlos Syndrome

Front 8

A child has hyper elastic joints, blindness, mental retardation, and hypotonia. (ie. low tone, low cognition, low vision) What is the diagnosis?

Back 8

Lowe's syndrome

Front 9

A 5yo boy has had palpable blanching purpura on lower exptremities and buttocks. He also has colicky abdominal pain with hematuria and proteinuria. His labs show an elevated BuN, creatinine and heme positive stools. He is diagnosed with HSP. What is the physiologic mechanism of this disease?

Back 9

Vasculitis of skin, GI tract, joints, kidneys.

Front 10

True or false.
Intussusception is a potential complication of HSP and it can be easily detected by barium or air enema.

Back 10

False. Intussusception is a complication of HSP, and it occurs in an ileoileal location making it difficult to detect by air or barium enema.

Front 11

True or False.
HSP is associated with joint involvement.

Back 11

False. It is associated with periarticular involvement typically around the soft tissues around the knees and ankles.

Front 12

True or False
HSP typically has normal platelet levels.

Back 12

True.

Front 13

What is the treatment for HSP?

Back 13

Supportive care

Front 14

How long must there be symptoms to diagnose JRA?

Back 14

At least 6 weeks

Front 15

True or False.
Large joint involvement is more common than small joint involvement in JRA.

Back 15

True

Front 16

True are False.
ANA and RF are usually positive in JRA.

Back 16

False. ANA is positive but RF is not.

Front 17

Which of the following is not typical of JRA?
A) Morning stiffness
B) Gradual loss of motion
C) Rash
D) RF positive
E) ANA positive

Back 17

D.

Front 18

How many joints are affected in polyarthritis?

Back 18

5 or more

Front 19

A teenage girl has JRA. Which type is most consistent with the following characteristics. 1=Oligoarthritis, 2=Polyarthritis and 3= Systemic JRA
A) Males and Females equal
B) Extraarticular involvement
C) HLA-B27
D) Chronic uveitis
E) ANA positive in young females
F) 4 or fewer joints involved
G) 5 or more joints involved

Back 19

A) Males and Females equal -- 3
B) Extraarticular involvement -- 1
C) HLA-B27 -- 2
D) Chronic uveitis -- 2
E) ANA positive in young females -- 1 and 2
F) 4 or fewer joints involved -- 2
G) 5 or more joints involved -- 1

Front 20

Which of the following is a harbinger of systemic JRA?
A) High fever
B) WBC 30000-50000
C) Rash
D) Hepatosplenomegaly
E) Lymphadenopathy
F) Pleuritis/Pericarditis
G) All of the above

Back 20

G.

Front 21

Are ANA and RF positive or negative in systemic JRA?

Back 21

negative

Front 22

How do you distinguish JRA and acute leukemia?

Back 22

JRA has morning stiffness. Acute leukemia is likely to awaken the child at night and bone pain does not involve a joint.

Front 23

True or False
LAD and HSM are present in JRA and in acute leukemia.

Back 23

True

Front 24

What are the 1st and 2nd line treatments for JRA?

Back 24

NSAIDS then steroids and immunosuppressants.

Front 25

What is the 1st line treatment for JRA with cardiac involvement?

Back 25

Steroids

Front 26

A 2yo presents with 5 days of fever. She also has conjunctivitis, desquamation of hands and feet, cervical LAD, a polymorphous exanthem and these are her lips. What is this and what is the treatment?

What is the diagnosis and treatment.

Back 26

Kawasaki syndrome - also called mucocutaneous lymph node syndrome. This reaction involves inflammation of mucous membranes, erythematous skin rashes followed by desquamation (particularly marked around the nails folds), high fever and lymphadenopathy. The trigger is unknown. Treatment involves high dose aspirin and immunoglobulin..

Front 27

What is the treatment for Kawasaki?

Back 27

High dose aspirin and IVIG.

Front 28

What do you expect on CBC of a child with Kawasaki on the 5th day AND 12th day?

Back 28

elevated platelets on 5th day, leukocytosis on 12th day
Normocytic anemia is present throughout

Front 29

How long are CRP, ESR, Alpha-1-antitrypsin elevated?

Back 29

4-6 weeks.

Front 30

True or False.
ANA and RF and circulating anticoagulant will not be elevated in Kawasaki disease.

Back 30

True.

Front 31

What is the major worrisome complication of Kawasaki? How do you monitor for this complication?

Back 31

A serious complication is coronary arteritis and aneurysm formation.
Do a 2D ECHO at time of diagnosis, then in 2-3 weeks then 6-8 weeks from initial diagnosis.

Front 32

What is the treatment regimen for KD?

Back 32

IVIG 2g/kg and ASA 80mg/kg/day for 2 days THEN ASA 5mg/kg/day for 2 months.

Front 33

Of the following findings. Indicate 1 for Kawasaki, 2 for JRA/Systemic type or 3 for both.
1. Acute onset fever
2. Adenopathy
3. Evanescent Reticular Rash
4. Conjunctivitis
5. Polymorphous exanthem
6. Desquamation of hands and feet.
7. Hepatomegaly
8. Gradual onset.
9. Cardiomegaly
10. Pleural effusions

Back 33

1 for Kawasaki, 2 for JRA/Systemic type or 3 for both.
1. Acute onset fever = 1
2. Adenopathy = 3
3. Evanescent Reticular Rash = 2
4. Conjunctivitis = 1
5. Polymorphous exanthem = 1
6. Desquamation of hands and feet. = 1
7. Hepatomegaly = 2
8. Gradual onset = 2
9. Cardiomegaly = 2
10. Pleural effusions = 2

Front 34

How do you distinguish measles and kawasaki?

Back 34

Measles rash goes head to toe and the conjunctivitis is exudative.

Front 35

How do you distinguish rash of scarlet fever and that of kawasaki?

Back 35

Scarlet fever rash is primarily in flexural areas

Front 36

True or False.
Lyme disease may present with joint pain and history of fatigue and cold like symptoms.

Back 36

True

Front 37

A patient comes to ED and you suspect Lyme. What is the first test you order?

Back 37

Lyme antibody titer then Western blot for confirmation.

Front 38

What is the treatment for lyme disease?

Back 38

30 days of doxy or erythromycin.

Front 39

A child is being treated for lyme disease with appropriate antibiotics. She comes into ED hypotensive, febrile and septic. What is the reason?

Back 39

The medications are lysing the bacteria which is causing toxin release.

Front 40

Which of these is not within the diagnostic criteria of Marfan's syndrome?
A) Dilitation and dissection of the ascending aorta
B) Lumbosacral dural ectasia
C) Mitral valve prolapse
D) Ecotopia lentis
E) Four skeletal anomalies

Back 40

C.

Front 41

What are the two preventive measures that are recommended for patient's with Marfan?

Back 41

Regular echo and regular slit lamp exam

Front 42

A 15yo comes in complaining of vague joint aches. She recently received an MMR booster but is otherwise healthy. What would explain the joint aches?

Back 42

Post infectious arthritis from the rubella in the MMR vaccine especially in a post-pubertal girl.

Front 43

Is the ANA and RF expected to be positive or negative in reiter's syndrome?

Back 43

Negative

Front 44

What are the three problems in Reiter's syndrome?

Back 44

Can't see, pee, or climb a tree.
Conjunctivitis, Urethritis, Arthritis

Front 45

True or False
Urethritis and Conjunctivitis are typically associated with chlamydia or gonorrhea.

Back 45

False.

Front 46

True or False
Reactive arthritis (or Reiter's syndrome) typically occurs after a bacterial enteral or venereal disease.

Back 46

True

Front 47

What is the treatment for Reactive arthritis?

Back 47

NSAIDs and Antibiotics

Front 48

A young boy comes in with R knee pain and left ankle pain. He also has conjunctivitis and dysuria. What is the cause of his dysuria?

Back 48

Urethritis from reactive arthritis.

Front 49

A 5yo child comes in after recent pharyngitis. Today she is complaining of knee pain. Her mom comments that normally her child is very calm and relaxed, but lately she has been emotional and has these fits of flailing her arms unnecessarily. On exam you not a new murmur that does not change with respiration. What is her diagnosis?

Back 49

Rheumatic fever.

Front 50

A child with all the symptoms of rheumatic fever has mild CHF. What is the likely problem?

Back 50

Aortic regurgitation.

Front 51

A child has a holosystolic murmur heard best at the apex. What is the most likely murmur?

Back 51

Mitral regurgitation.

Front 52

What are the requirements of Rheumatic Fever diagnosis?

Back 52

Recent Group A Strep infection.
AND
2 Major Criteria
AND
1 Major or 2 Minor Criteria

Front 53

What are the Major Criteria for Rheumatic Fever?

Back 53

Joints = Polyarthritis
❤ = Carditis (CHF, new murmur, cardiomegaly)
N = nodules - firm and painless on extensor surfaces of wrists, elbows and knees
E = erythema marginatum
S = sydenham chorea - rapid purposeless movements of face and arms

Front 54

What are the Minor Criteria for Rheumatic Fever?

Back 54

H = hot - fever
E = elevated acute phase reactants
L = arthaLgia
P = prolonged PR interval

Front 55

True or False.
The arthritis of rheumatic fever is migratory and the rash is erythema marginatum.

Back 55

True

Front 56

What "SHAPeD" your treatment regimen for rheumatic fever?

Back 56

S = steroids
H = Haldol
A = aspirin
P = penicillin
extra support
D = digoxin for CHF

Front 57

Which of the following are acceptable as proof of strep infection? (Pick 2)
A) Throat culture
B) Rapid strep test
C) ASO titer
D) Positive streptozyme.

Back 57

C and D

Front 58

A 12yo African American boy is brought to your office for a chronic cough and constantly being tired. He has no TB contact and a negative PPD. You order labs and note hypercalcemia and renal insufficiency. This is the CXR. What is the diagnosis?

Back 58

Sarcoidosis.

Front 59

A 14yo girl has a linear hyper-pigmented patch with a brown border that is painful and tender. What is the diagnosis and treatment?

Back 59

This is scleroderma. If limited to fingers treat with topical lubricants. If widespread (Type 3), treat with steroids, immunosuppressives, methotrexate and peicillamine.

Front 60

True or False.
ANA is positive in systemic scleroderma.

Back 60

True.

Front 61

What is the most common pathogen in septic arthritis for kids over age 2yo-teen. What about for teens?

Back 61

For 2yo-teen is S. aureus
For teens it is N. gonorrhea

Front 62

How do you diagnose septic arthritis?

Back 62

Aspirate the joint

Front 63

A 16yo girl has FEVER, URTICARIA and ARTHRALGIA. She can not recall any recent allergic reactions and has been healthy other than a recent UTI which was treated with bactrim.
What is her diagnosis?

Back 63

Serum sickness. Type 3 hypersensitivity.

Front 64

What is the treatment for septic arthritis?

Back 64

Long term IV Antibiotics. Do not wait.

Front 65

What are the three classic features of Sjogren's syndrome?

Back 65

Dry mouth (xerostomia), Dry eyes (xeropthalmia), Painless parotid swelling.

Front 66

What is the Shirmer test?

Back 66

It measures tear production

Front 67

What is the risk of untreated Sjogren's?

Back 67

Lymphoma.

Front 68

What is Sjogren-Larsson Syndrome?

Back 68

An autosomal recessive inborn error in lipid metabolism.

Front 69

True or False.
SLE is more common in caucasians than blacks.

Back 69

False. It is 4 times more common in blacks.

Front 70

True or False.
SLE is more common in girls than boys.

Back 70

False. This is true in adults, but it is gender equal in boys.

Front 71

What is the diagnostic serology in SLE?

Back 71

Anti-smith antibodies

Front 72

What is the most specific serology for SLE?

Back 72

Anti-smith antibody

Front 73

What is the screening test for SLE?

Back 73

ANA

Front 74

True or False.
Lupus anticoagulant can result in a false positive serology for syphillis.

Back 74

True

Front 75

What is Raynaud's Triad?

Back 75

White ischemic fingers
Red cold fingers
Blue cyanotic fingers

Front 76

Which drugs cause Drug Induced Lupus (DIL)?

Back 76

D = Drugs
S = Sulfonamides
L = Lithium
E = Epilepsy

Queen (Quinadine) Hydalazine Procainamide

Front 77

What is the most sensitive test for Drug induced Lupus?

Back 77

ANA

Front 78

What are the signs of active reanl disease in lupus?

Back 78

Increased anti-DNA titers, Decreased C3, C4 and CH50.

Front 79

True or False
Microischemia and vascular disease result in seizures in SLE

Back 79

True

Front 80

What would be the most likely explanation for death of an infant born to a mother with SLE?

Back 80

Heart Block.

Front 81

A neonate presents with a scaly erythematous rash, bradycardia and thrombocytopenia. What are the confirmatory tests for neonatal lupus?

Back 81

Anti-Ro and Anti-La

Front 82

What are the best indicators of good response to treatment in SLE?

Back 82

Normalization of C3 and C4.

Front 83

What are the two major side effects of hydroxychloroquine?

Back 83

Ototoxicity and Retinal damage

Front 84

What is the treatment for mild SLE?

Back 84

NSAIDS
then hydroxycholorquine (plaquenil)
then methotrexate

Front 85

What is the treatment of severe SLE?

Back 85

High dose steroids and cyclophosphamide.

Front 86

True or False.
Lupus patients undergoing treatment with immunosuppressive agents are at risk for varicella.

Back 86

True

Front 87

What are the three organ systems involved in Wegener Granulomatosis?

Back 87

Sinus
Lungs
Kidney.

Front 88

What determines the severity of Wegener Granulomatosis?

Back 88

Extent of kidney involvement.

Front 89

What are the expected lab findings in Wegener?

Back 89

C-ANCA with joint pain, fevers and sinusitis.

Front 90

What is the treatment for Wegener?

Back 90

Cyclophosphamide

Front 91

A child has bilateral joint pains without any swelling. It is worse at night. You note that he walks with a limp. What is the diagnosis?

Back 91

Growing Pains

Front 92

An athelete has loose joints. What is your advice to this anymore?

Back 92

The must be mindful of stretching other than that.

Front 93

Synovial Fluid is yellow or clear, has increased viscosity and WBC < 200. What is it?

Back 93

Normal synovial fluid

Front 94

Synovial Fluid is bloody or clear, has increased viscosity and WBC < 2000. What is it?

Back 94

Arthritis secondary to trauma

Front 95

Synovial Fluid is yellow or clear, has normal viscosity and WBC 5000 and LE cells. What is it?

Back 95

SLE

Front 96

Synovial Fluid is yellow and cloudy, has decreased viscosity and WBC 15000-20000. What is it?

Back 96

JRA

Front 97

Synovial fluid is yellow and cloudy and has decreased viscosity and WBC 5000. What is it?

Back 97

Rheumatic Fever

Front 98

Synovial fluid is yellow and opaque and has decreased viscosity and WBC 20000. What is it?

Back 98

Reiter

Front 99

Synovial fluid is yellow and has variable viscosity and WBC 50000 - 300000 and low glucose and low bacteria. What is it?

Back 99

Septic arthritis.