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