Pediatric Rheumatology

Question 1

Spondyloarthropathies?

Answer 1

Juvenile ankylosing spondylitis
Arthritis associated with inflammatory bowel disease
Reactive arthritis following GI or GU infections
Psoriatic arthritis

Question 2

Juvenile ankylosing spondylitis? (JAS)

Answer 2

most frequent in older boys, adolescents, and young adults
frequently familial
strong assocation with human leukocyte antigen HLA-B27

Question 3

Clinical manifestation of JAS?

Answer 3

Oligoarthritis (legs more than arms)

Enthesitis (Inflammation at the sites of attachment of lig, tendons, fascias, capsules to bones) (may progress to calcification lig and fusion of joints)

Hip joint arthritis is particular suggestive of JAS

Loss of spinal mobility may eventually be noted (loss lumbar lordosis)

Question 4

JAS characteristics?

Answer 4

disease course may be characterized by long periods of apparent disease remission

systemic symptoms of low grade fever and weight loss, raise possibility of occult inflammatory bowel disease

Question 5

JAS diagnosis?

Answer 5

Older boy with oligoarthritis mainly affecting lower extremities
-loss of normal lumbar lordosis, inability to touch the toes while legs are straight, pain on palpation or compression of pelvis

Confirmation (radiographic evidence of sacroilitis) not always present early, confirmatory

Question 6

Lab findings of JAS?

Answer 6

Evidence of systemic inflammation (increased ESR, increased WBC, increase platelet count)

Negatives (RF, antinuclear antibodies)

HLA-B27 (90%)

Question 7

Treatment JAS?

Answer 7

control inflammation
minimize pain
preserve function
methods (anti-inflammatory meds, exercises, physical therapy, pyschosocial support)

Question 8

JAS complications?

Answer 8

anterior uveitits (up to 25%)
aortic valve insufficiency (rare, but impt)
atlantoaxial subluxation

Question 9

Juvenile Idiopathic Arthritis?

Answer 9

disease formerly known as Juvenile Rheumatoid Arthritis (JRA)

most common rheumatic disease of children

3 principle types of onset:
-oligoarthritis/pauciarticular disease, polyarthritis, systemic-onset disease

Question 10

Juvenile Idiopathic Arthritis etiology (JIA)?

Answer 10

etiology unknown
possible external triggers
-Viruses (parovirus B19, rubella, EBV)
-Host hyperractivity to specific self antigens (type II collagen)
-Enhanced T cell reactivity to bacterial or mycobacterial heat shock proteins)

Question 11

Major difference between JIA and RA?

Answer 11

JIA mainly affects the lower extremity
morning stiff, easy fatigue, joint pain later in day, joint swelling, involved joints (warm, resist full range of motion, painful on motion, not usually erythematous)

Question 12

Rheumatoid Nodules?

Answer 12

on extensor surfaces of the elbows and over the Achilles tendons
Unusual and associated with a more severe course

Question 13

Micognathia?

Answer 13

chronic TMJ disease

Question 14

Cervical spine involvemnet?

Answer 14

risk of atlantoaxial subluxation

potential neurological sequelae

Question 15

JIA systemic onset?

Answer 15

Arthritis and prominant visceral involvement
Fever with temps over 39c for over 2 wks (accompanied wth rash)
Koebner phenomenon

Question 16

Koebner phenomeon?

Answer 16

cutaneous hypersensitvity to superficial trauma

Suggestive of systemic onset disease

Question 17

Diagnosis of JIA?

Answer 17

age at onset under 16 yrs
arthritis in one or more joints
duration of disease 6 weeks or longer
exclusion of other forms of juvenile arthritis

Question 18

Lab stuff JIA?

Answer 18

Elevated (WBC, Platelet counts, ESR, (may be normal) C reactive protein)

Decreased (Hemoglobin and Mean corpuscular volume)

Antinuclear antibodies (ANA) pos (40-85%)

Question 19

JIA treatment?

Answer 19

Treat pain with non-steroidal anti-inflammatory meds (treat refractory pain with steroids and immunosuppressants)

Non-pharmacological intervention is just as important (Physical and occupation therapists, social workers, specialized nurses)

Question 20

SLE?

Answer 20

systemic lupus erythematous
Auto-antibodies directed against self antigens
-leads to inflammatory damage of many organs
Genetic predisposition is suggested

Question 21

SLE presentation?

Answer 21

children present with diverse and often severe manifestations
-fever, fatigue, hematologic abnormalities, arthralgia or arthritis, malar rash, renal disease

Question 22

SLE Lab findings?

Answer 22

Elevated ANA
Anti-double stranded DNA (more specific for lupus, reflect the degree of serologic disease activity)
Complement C3 and C4 are decreased

Question 23

Neonatal lupus?

Answer 23

Maternal transfer of IgG auto-anitbodies
only a small percentage develops neonatal lupus
Symptoms
-Congential heart block
-Cutaneous lesions
-Hepatitis, Thrombocytopenia, Neutropenia
Treatment is supportive

Question 24

Juvenile Dermatomyositis?

Answer 24

Most common of the pediatric inflammatory myopathies

Genetic predisposition and environmental tiggers

Question 25

Juvenile Dermatomyositis clinical manifest?

Answer 25

history of infection 3 months prior to disease onset
rash in sun exposed areas develop in 50% of cases
Proximal weakness
Dysphagia
Infrequent findings (heptosplenomegaly, retinitis, iritis)

Question 26

Juvenile Dermatomyositis lab findings?

Answer 26

Elevated muscle derived enzymes (creatinine kinase, lactic acid dehydrogenase, may be normal for the first 4-5 months after disease onset)

ESR is usually normal

RF negative

Question 27

JD Juvenile Dermatomyositis treatment?

Answer 27

often responsive to immunosuppresive therapy

unlike many adults, children appear able to repair their vasculature and muscle damage