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GreenC2 - Rheum

rheumatology

QuestionAnswer
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
Created by: fl_sun80
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