Rheum Part 3-Paulson (Exam 2) Flashcards
A child might have a form of JIA if they have at least ___ affected joint(s) for ___ week(s).
at least 1 joint
for at least 6 weeks
JIA can manifest in many different ways, but the main mechanism of the disease if ________________________.
persistent joint swelling that comes from synovial fluid increase (with inflammatory cells) + expansion of pannus=joint damage
This subtype of JIA is most common in ages 1-7yrs, involves 4 joints or less (asymmetrically); most commonly seen in the knee joint. This kiddos might also have prominent morning stiffness.
Oligoarticular JIA (aka pauriarticular JIA)
Children with oligoarticular JIA (esp ones with positive ANA) have an increased risk of developing what type of eye disorder?
Uveitis (can be asymptomatic and/or occur without the joint symptoms)
This subtype of JIA affects 5 or more joints and typically has an elevated ESR.
Polyarticular JIA
If a child with polyarticular JIA has a positive Rf, it is referred to as ____________.
Seropositive
___________(seropositive or seronegative) is the more aggressive form of polyarticular JIA and resembles the adult version of RA.
Seropositive
T/F? Pt’s with Seronegative polyarticular JIA usually have other extraarticular sx’s like rheumatoid nodules, vasculitis, and lung dz.
False. Seronegative does not have extraarticular sx’s, while seropositive does.
The other/ technical name for “Still’s Disease is _________________.
Systemic Onset JIA (SOJIA)
Children with SOJIA are likely to have a daily ________ fever. What does this mean?
Daily quotidian fever, is a fever that spikes then resolves spontaneously, can go up and down
If you see a child with a “salmon-colored evanescent macular rash” on their trunk and extremities, what rheumatologic disease would you think about?
Systemic Onset JIA (SOJIA)
You are working up an ill-appearing 10 yr old child, who you suspect has SOJIA due to his clinical presentation. You decide to run labs. What labs do you choose and what do you expect them to reflect if he does indeed have SOJIA?
Labs: CBC, ESR and Iron panel
Results would show: High leukocytosis, very high ESR, elevated ferritin
______ ________ syndrome is a potentially fatal complication that can occur in kiddos with SOJIA. This syndrome occurs due to excess circulating ___________, which can cause seizures/DIC/bleeding.
MAS (Macrophage activation syndrome)
Cytokines
The following are characteristics of what type of JIA? Arthritis in at least 1 joint, DIP synovitis, dactylitis, nail pitting.
Psoriatic Arthritis
With enthesitis-related JIA, you would expect tenderness of the ____________ and a positive HLA-B27.
Sacroiliac
Which type of JIA includes those with juvenile-onset spondylitis, reactive arthritis, and arthritis associated with IBD?
enthesitis-related JIA
How do you diagnose JIA?
Clinically diagnosed; no specific lab/imaging to definitely confirm the diagnosis’, but they can guide you in the right direction and exclude other possibilities.
What are the mainstays of treatment for JIA?
NSAIDS (ibuprofen, naproxen, meloxicam) Joint injections (triamcinolone acetate)
Children with SOJIA may need a __________ intially for sx control.
steroid
Pt’s with which type of JIA should be treated aggressively with a DMARD?
Seropositive polyarticular JIA
Which form of JIA has the highest mortalilty?
SOJIA
_______ ________ is a chronic, systemic autoimmune disorder with fibrosis of the skin and other organs.
Systemic sclerosis (Scleroderma)
There are 2 forms of Scleroderma; _________ which includes CREST syndrome, and _________.
Which is more common?
Limited (affects face, distal to knees/elbows)
Diffuse (throughout he body)
MC=Limited (80%)
What does CREST Syndrome consist of?
Calcinosis cutis- Ca deposits in the skin
Raynaud phenomenon(usu 1st sign, can start years before any other sx’s)
Esophageal dysmotility
Sclerodactyly-thickening/tightening of skin on toes/finger
Telangiectasia
There is a higher incidence of scleroderma in which two specific populations?
African americans
Choctaw Native Americans
What are some common skin manifestations of scleroderma?
Initially, edema + pruritis Later, thick, hide-like skin Hyperpigmentation or depigmentation Digital ulcers Pitting of fingertips Loss of appendicular hair Telangiectasias Calcinosis
Scleroderma can also manifest in other important organs. One thing that has been found on autopsy is vascular ectasia of the stomach antrum, aka ___________ ___________.
Watermelon stomach
________ _______ ______ is a complication of Scleroderma and is the sudden onset of malignant HTN that can lead to renal failure and death.
Scleroderma Renal Crisis (SRC)
With Scleroderma pt’s will have a ________(pos/neg) ANA 95% of the time.
_________ is another lab that is positive in 1/3 of pt’s with diffuse dz and 20% with CREST (Indicates poor prognosis).
POSITIVE
Anti-SCL-70
What is the tx for scleroderma?
No proven disease-modifying drug to treat scleroderma.
Treatment is targeted to organs affected.