Flashcards in Pediatric Rheumatic Disease Deck (64):
Which gender is more affected with JIA?
What is the pathology that occurs with JIA?
hyperplasia of the synovial lining, causing edema, and hyperemia
What are the s/sx that are specific to JIA? (5)
-Limp / refusal to bear weight
-joint edema, erythema, and warmth
What are some of the systemic signs of JIA?
What are the two major kinds of JIA
What is the polyarthritis form of JIA? How does RF positivity / negativity correlate with symptoms?
RF + = like adult RA
RF - = 10% have destructive joint dz
What is the oligoarthritis form of JIA?
1-4 joints affected
What percent of systemic JIA will remit within 1 year?
What is the systemic form of JIA?
characterized by arthritis, fever, which typically is higher than the low-grade fever associated with polyarticular and a salmon pink rash.
What percent of JIA cases are polyarthritis? Oligoarthritis? Systemic?
Polyarthritis = 40%
Oligoarthritis = 50%
Systemic = 10%
What percent of cases of polyarthritis, oligoarthritis, and systemic arthritis have uveitis?
Polyarthritis = 5%
Oligoarthritis = 40%
Systemic = rare
What percent of cases of polyarthritis, oligoarthritis, and systemic arthritis are Rh factor +?
Polyarthritis = 10%
Oligoarthritis = rare
Systemic = rare
What percent of cases of polyarthritis, oligoarthritis, and systemic arthritis are ANA +?
Oligoarthritis = 80%
Systemic = 10%
What are the classic joints involved in polyarthritis, oligoarthritis, and systemic arthritis respectively?
Polyarthritis = all large joints
Oligoarthritis = knees and ankles
Systemic = any joint
What is the level of systemic involvement in polyarthritis, oligoarthritis, and systemic arthritis respectively?
Polyarthritis = moderate
Oligoarthritis = rare
Systemic = Severe
If a JIA patient has a positive ANA, what must they be screened for, and by whom?
Uveitis, and requires an ophthalmology exam
What are the long term effects of uveitis 2/2 untreated JIA?
Damage the uvea, leading to poor eyesight
JIA can only be diagnosed in kids less than what age?
16 years old
What is the duration needed to diagnose JIA
What are the major s/sx of arthritis in JIA pts?
What are the characteristics of the rash seen with JIA?
Diffuse macular rashes that is salmon colored
What are the CBC findings in a kid with JIA?
What happens to ESR and CRP levels with JIA?
True or false: an ANA is needed to diagnose JIA in most cases
What is the pharmacotherapy for JIA? (4)
What are the only disease modifying treatment available for JIA?
What is the prognosis for JIA? What if it's Rh factor +?
Infrequent exacerbations, with long periods of remissions
Worse if Rh factor +
If severe, leads to joint destruction
What is psoriatic arthritis?
Form of JIA, that consists of chronic arthritis and psoriasis OR
Arthritis with dactylitis, nail pitting, or onycholysis
What is the pharmacotherapy for psoriatic arthritis?
What is enthesitis?
Inflammation of the enthesis (site of attachment of muscle or ligament to bone)
Which gender is more affected with enthesitis-related JIA? What is the age affected?
Males older than 8
What is the HLA haplotype that is associated with enthesitis-related JIA??
What might enthesitis-related JIA progress to?
What is neonatal lupus?
When the mother transfers antibodies to the fetus, causing thrombocytopenia, cutaneous rash, and complete heart block
What is the hematologic abnormality that can arise from neonatal lupus?
What is the cardiac abnormality that can arise from neonatal lupus?
Complete heart block
What is the liver abnormality that can arise from neonatal lupus?
Hepatomegaly or hepatitis
What is the treatment / prognosis for neonatal lupus?
Most symptoms will resolve w/in 6 months, but complete heart block will require permanent pacemaker
True or false: mothers are rarely asymptomatic with SLE when a child develops neonatal SLE
what, generally, is Henoch-Schonlein purpura?
Idiopathic IgA mediated vasculitis that causes non-thrombocytopenic purpura and vasculitis
What type of purpura are had with HSP?
What is the age range that is usually affected with HSP?
What are the areas affected with HSP? (4)
Where on the skin are the purpura found with HSP? Is it usually symmetric or asymmetric?
Dependent areas (buttock + LE)
What are the GI symptoms of HSP?
Intermittent, colicy abdominal pain
What is a severe GI sequelae of HSP?
What is the diagnostic study needed to diagnose intussusception?
Ba contrast enema
What are the joint symptoms of HSP? (What are not?)
-Tenderness with ROM
(NOT erythema, edema)
What type of kidney pathology does HSP cause: nephrotic or nephritic syndrome?
What will and CBC show with HSP?
What will a PT/PTT show with HSP?
What infectious disease should be r/o with HSP?
What are the kidney labs that should be monitored with HSP?
BUN/Cr and UA
What is the treatment for HSP?
Steroids for abdominal pain
What is the usual course for HSP?
Self limiting, but may have recurrences
What is the treatment for severe abdominal pain 2/2 HSP?
What is the classic triad with HUS?
-Microangiopathic hemolytic anemia
What is the infectious agent that causes HUS?
Shiga toxin producing strains of E.coli O157:H7
What is the major difference in the hematologic abnormalities between HSP and HUS?
HUS has thrombocytopenia, whilst HSP does not
What is the usual presentation of HUS?
Abdominal pain + Bloody diarrhea, followed by:
-pallor (2/2 anemia)
-Sz / coma
What is the role of antimotility agents in kids?
What is the role of abx in the treatment of E.coli dysentery?
Not used-may precipitate HUS
What type of transfusions are used in HUS?
pRBCs, but avoid platelet transfusions