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rheumatology

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Question
Answer
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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