Question | Answer |
must be < 16yoa and have symptoms for at least 6wks | diagnosis of JIA |
what are the 3 types of JIA? | Systemic/oligoarthritis/polyarthritis |
affecting 4 or fewer joints | oligoarthritis |
morning stiffness that improves with movment later in the morning | JIA |
leg-length discrepancies; enuresis may occur | JIA |
requires daily fever occuring usually in the evenings | systemic JIA |
when fever gone, child appears better; a/w arthalgias/myalgias | systemic JIA |
peak age 5-10yrs; boys=girls | systemic JIA |
mild rubbing/scratching of th skin may bring out the rash | kobner phenomenon |
migratory rash, pink to salmon colored; uveitis is RARE | systemic JIA |
on labs: leukocytosis, thrombocytosis, elevated ESR/CRP, anemia, RF & ANA negative | systemic JIA |
elevated serum transaminases, D-dimer +, prolonged PTT, platelets & ESR drop precipitously, hemophagocytosis in bone marrow | macrophase activation syndrome |
triggered by viruses, sulfa drugs, NSAIDS in systemic JIA | macrophage activation syndrome |
age 1-7, ave is 5yoa; F:M is 3:1 | oligoarticular JIA |
a/w positive ANA and asymptomatic UVEITIS | oligoarticular JIA |
peak age is 1-3yrs(usu RF neg); adolescents can be RF positive | polyarticular JIA |
if patient ANA positive and < 7yrs then need to screen for uveitis | polyarticular JIA |
NSAIDS are first line for treatment | JIA |
more severe disease can be treated by methotrexate | JIA |
arthritis plus 2 of the following: dactylitis, nail findings and first degree relative with this dz | juvenile psoriatic arthritis |
DIP joints commonly affected | juvenile psoriatic arthritis |
can have uveitis and ANA positive, common in young girls or adoslecent boys | juvenile psoriatirc arthritis |
this marker is common in ERA | HLA-B27 |
Name at least 2 other features a/w ERA (enthesitis) | sacroiliac joint tenderness; HLA-B27; 1st or 2nd degree relative w HLA-B27 dz; anterior uveitis; onset of arthrisi in a boy after 8yoa |
pain in joints of lower extremities, low back and buttocks are commonly seen | ERA |
triamcinolone joint injections if no improvement from NSAIDs | ERA treatment |
arthritis flares occur with gut flares and improve with gut treatment | IBD associated arthopathy |
typically occurs 1-4wks after GI infxn with yersenia, salmonella, or capylobacter or Chlamydia or ycoplasma | reactive arthritis |
arthritis with conjunctivitis and uveitis | reactive arthritis |
last 3-6wks, fever is common and it commonly affects large, weight bearing joints | reactive arthritis |
borrelia burgdorferi | Lyme disease |
Ixodes scapularis tick or Ixodes pacificus | Lyme disease |
Erythema migrans | Lyme disease |
Lyme disease - neurologics symptoms | peripheral neuropathy, bell's palsy, foot drop |
DON'T do serology if suspecting this disease | Lyme disease |
heart block is a later finding | lyme disease |
treatment for lyme disease | doxycycline (x 2wks) |
treatment for lyme carditis or meningitis | ceftriaxone |
true or false: prophylaxis for hikers/campers is indicated | false |
most commonly dx vasculitis of childhood | HSP |
how does HSP occur? | IgA mediated |
Name some HSP triggers | GABHS, Legionella, mycoplasma, yersinia, PCN, cephalosporins, MMR, yellow fever, food additives, insect bites |
rash: small wheals or red maculopapules that become petechia and purpura on lower extremities and buttocks | HSP |
may also see this classic rash (palpable purpura) on face and ears of young children | HSP |
Name the 3 most common manifestations of HSP in order | 1. skin 2. joint 3. GI |
which joints are commonly affected in HSP | large joints (periarthritis) |
can result with ileoileal intussusception | HSP |
can result with Hydrops gallbladder | HSP |
what will you find on urine dip for HSP? | isolated microscopic hematuria/proteinuria |
lab values for HSP | high WBC, ESR, IgA levels; Normal platelets and coagulation |
How do you treat HSP | supportive |
what is the likelihood of HSP reoccurance? | 40% from 6wks- 2yrs |
"Pulmonary-renal" syndrome | Wegener granulomatosis |
will have necrotizing granulomatous vasculitis of small vessels of the upper and lower respiratory tract and the kidney | Wegener granulomatosis |
nasal deformity; c-ANCA positive | Wegener granulomatosis |
how to acutely tx wegener's? | steroids, methotrexate, cyclophosphamide |
what can you use to prevent relapse of Wegener's? | TMP/SMX |
vasculitis of the arteries AND veins | Behcet disease |
What is the classic triad of Behcet disease? | painful oral ulcers/painful genital ulcers/inflammatory eye disease |
may commonly have erythema nodosum; pathergy | Behcet disease |
Treatment for Behcet disease? | steroids, sometimes colchicine |
Mean age for HSP | 4yrs; 75% under age 7 |
20% of pediatric SLE is diagnosed under age? | 18 |
2 most common labs in SLE | ANA and Anti ds-DNA |
complement levels are high or low in SLE? | LOW |
Classic xray findings of SLE | normal |
type of arthritis of SLE | polyarticular of small and large joints |
Skin manifestations of SLE | malar rash, discoid rash, photosensitivity, alopecia |
CNS manifestations of SLE | seizures, CHOREA, cranial nerves, psychiatric disease |
Hematologic manifestations of SLE | pancytopenia, coombs-positive hemolytic anemia, PT/PTT prolonged |
Cardiac manifestations of SLE | pericarditis, Libman-Sacks endocarditis |
Must have how many of the 11 criteria for diagnosis of SLE | 4 of 11 |
What are the diagnostic criteria for SLE | malar rash; discoid rash; photosensitivity; oral ulcers; arthritis; serositis; renal d/o; neurologic d/o; hematologic d/o; Immunologic d/o; positive ANA |
How do you treat SLE? | Hydroxychloroquine for skin and joint flares (check eyes 1-2x/yr); azathioprine, cyclophosphamide; corticosteroids (flares) |
Side effects of SLE meds | Avascular necrosis; osteoporosis; growth failure; glaucoma; DM; HTN; accelerated atherosclerosis |
girls present with raynaud phenomenon, fever, arthritis, dorsal hand edema, rash and myositis | mixed connective tissue disease |
over time, this disease acts more like scleroderma with esophageal disease and dysphagia | mixed connective tissue disease |
Classic findings of sjogren syndrome | keratoconjunctivitis sicca; xerostomia; lyphocytic infiltrates on minor salviary gland bx; labs (one of the following: RF, ANA, Anti-Ro or Anti-La) |
suspect this in children with recurrent parotitis and keratoconjunctivits sicca | sjogren syndrome |
"bilateral evening or nighttime pains" | growing pains |
heliotrope rash | juvenile dermatomyositis |
autosomal recessive disorder; on chromosome 16 (regulates PMN inflammatory response) | familial mediterranean fever |
presents before age 10yrs; fever cycles for 3-5 days/month | familial mediterranean fever |
severe abdominal pain; pleuritis; pericarditis; scrotal swelling | familial mediterranean fever |
how to treat familial mediterranean fever? | colchicine |
3 body areas of pain for at least 3 months | Fibromyalgia |
stiffness in the morning, basically all day; all labs are normal | Fibromyalgia |
can treat with amitriptyline or cyclobenzapine for nighttime sleep disturbances | Fibromyalgia |
what does PFAPA stand for? | Periodic fever, aphthous-stomatitis; pharyngitis; cervical adenitis |
this fever lasts for 5-7 days and stops by teenage yrs | PFAPA |
can try cimetidine or tonsillectomy | PFAPA |
swimming is good therapy; most commonly seen in girls | hypermobility syndrome |
what to look for with hypermobility syndrome | marfan's, ehlers-danlos |
most common cause of recurrent limb pain in children | growing pains |
deep aching in non-articular areas (usually awaken from sleep) | growing pains |
known as morphea; more common in children | Localized scleroderma |
CREST syndrome | calcinosis; raynaud's; esophageal dysmotility; sclerodactyly; telangiectasias |
more common in girls; rash with muscle weakness | juvenile dermatomyositis |
gottron papules; treatment is prednisone | juvenile dermatomyositis |
Which 2 cancers seen with Sjogren syndrome | MALT lymphoma and non-Hodgkin B-cell lymphoma |
hypergammaglobulinemic purpura | sjogren syndrome |
how to treat sjogren syndrome | artifical tears; antimalarial agents |