Pediatric Rheumatology Flashcards
Spondyloarthropathies?
Juvenile ankylosing spondylitis
Arthritis associated with inflammatory bowel disease
Reactive arthritis following GI or GU infections
Psoriatic arthritis
Juvenile ankylosing spondylitis? (JAS)
most frequent in older boys, adolescents, and young adults
frequently familial
strong assocation with human leukocyte antigen HLA-B27
Clinical manifestation of JAS?
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)
JAS characteristics?
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
JAS diagnosis?
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
Lab findings of JAS?
Evidence of systemic inflammation (increased ESR, increased WBC, increase platelet count)
Negatives (RF, antinuclear antibodies)
HLA-B27 (90%)
Treatment JAS?
control inflammation
minimize pain
preserve function
methods (anti-inflammatory meds, exercises, physical therapy, pyschosocial support)
JAS complications?
anterior uveitits (up to 25%)
aortic valve insufficiency (rare, but impt)
atlantoaxial subluxation
Juvenile Idiopathic Arthritis?
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
Juvenile Idiopathic Arthritis etiology (JIA)?
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)
Major difference between JIA and RA?
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)
Rheumatoid Nodules?
on extensor surfaces of the elbows and over the Achilles tendons
Unusual and associated with a more severe course
Micognathia?
chronic TMJ disease
Cervical spine involvemnet?
risk of atlantoaxial subluxation
potential neurological sequelae
JIA systemic onset?
Arthritis and prominant visceral involvement
Fever with temps over 39c for over 2 wks (accompanied wth rash)
Koebner phenomenon
Koebner phenomeon?
cutaneous hypersensitvity to superficial trauma
Suggestive of systemic onset disease
Diagnosis of JIA?
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
Lab stuff JIA?
Elevated (WBC, Platelet counts, ESR, (may be normal) C reactive protein)
Decreased (Hemoglobin and Mean corpuscular volume)
Antinuclear antibodies (ANA) pos (40-85%)
JIA treatment?
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)
SLE?
systemic lupus erythematous
Auto-antibodies directed against self antigens
-leads to inflammatory damage of many organs
Genetic predisposition is suggested
SLE presentation?
children present with diverse and often severe manifestations
-fever, fatigue, hematologic abnormalities, arthralgia or arthritis, malar rash, renal disease
SLE Lab findings?
Elevated ANA
Anti-double stranded DNA (more specific for lupus, reflect the degree of serologic disease activity)
Complement C3 and C4 are decreased
Neonatal lupus?
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
Juvenile Dermatomyositis?
Most common of the pediatric inflammatory myopathies
Genetic predisposition and environmental tiggers
Juvenile Dermatomyositis clinical manifest?
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)
Juvenile Dermatomyositis lab findings?
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
JD Juvenile Dermatomyositis treatment?
often responsive to immunosuppresive therapy
unlike many adults, children appear able to repair their vasculature and muscle damage
