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48 Cards in this Set
- Front
- Back
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What test can be done to dx rheumatic disease?
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There is NO specific dx test for rheumatic dz. You have to exclude other causes first.
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What is the clinical manifestation of JRA?
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*AM stiffness & gelling
*easy fatigability *objective joint swelling *joints are warm, resist full ROM, & are painful on motion *usually little to NO redness |
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What is the epidemiology of JRA and what is it?
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*Common in children; age onset <16
*Major cause of chronic disability in children *Synovitis of the peripheral joints manifesting in soft tissue swelling & effusion |
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What are the 3 different types of JRA?
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1- oligoarthritis/pauciarticular
2- polyarthritis/polyarticular 3- Systemic disease |
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Describe oligoarthritis/pauciarticular JRA.
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*usually affects lower extremities; <4 inflamed joints
*often single jt at onset *hip is ALMOST NEVER a presenting sign |
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Describe polyarthritis / polyarticular JRA.
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*small & lg joints of both upper & lower extremities; >5 inflamed jts
*May have rheumatoid nodules *Micrognathia reflects TMJ involvement |
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What is systemic disease type JRA?
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arthritis w/characteristic fever
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Is it possible for one rash to be the only one that causes a specific rheumatic disease?
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NO... BS! B/c all rheumatic diseases cause rashes
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Which rheumatologic condition causes muscle weakness?
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Juvenile Dermatomyositis
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What is the most common pediatric inflammatory myopathy?
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Juvenile Dermatomyositis
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Describe Juvenile Dermatomyositis.
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*characteristic rash & symmetric muscle weakness
*hx of infection in 3 months prior to dz onset |
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What is the most common infection that precedes juvenile dermatomyositis? 2nd most common?
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1- URI
2- Gastroenteritis |
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What two bugs have been implicated with juvenile dermatomyositis?
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* Coxsackievirus B
*Group A strep |
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Which rheumatologic condition causes skin tightness & fibrosis?
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Scleroderma
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What is scleroderma?
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*injury to the endothelium
*fibrosis affecting the dermis, arteries of the lung, GI & kidneys |
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Scleroderma is classified based on what two things?
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* skin involved
* internal organ |
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Which rheumatologic condition(s) causes pulmonary lung disease?
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*Systemic scleroderma can cause pulmonary fibrosis
*SLE can cause atelectasis, pleural effusions, & pulmonary infiltrates |
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Which rheumatologic condition causes aneurysms?
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*Kawasaki disease
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What is the peak age of Kawasaki disease?
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18 months - 2 years
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What is the most common rheumatic disease in children in the US?
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Juvenile arthritis (JA)
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What is the key feature of JA?
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Arthritis (swelling or effusion, limitation of motion, tenderness or pain on motion, or increased heat) for >6 weeks in one or more joints
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What important factor is it important to consider in taking a clinical hx on a child w/arthritis? Why?
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*age & developmental level
*b/c arthritis in children can begin at any age from 8 months through adolescence |
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What is true synovitis?
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Joint swelling or joint pain & limitation of motion
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What physical signs are characteristic of the systemic form of JA? What other findings are associated w/systemic JA?
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1- fever & rash
2- tachycardia, tachypnea, irritability |
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What is macrophage activation syndrome?
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A potentially fatal condition that occurs in a minority of children w/systemic JA.
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What is macrophage activation syndrome often triggered by?
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Drugs or intercurrent viral illness
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What is macrophage activation syndrome manifested by?
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*DIC
*Hepatic, pulmonary, & CNS involvement |
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What ophthalmic complications may be associated w/JA?
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*subacute anterior uveitis
*synechia (irregular pupil margins) *cloudy anterior chambers *cataracts |
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What is the typical cell count & differential in synovial fluid of JA? How does this contrast w/septic arthritis?
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JA- WBC (10-50,000); Primarily neutrophils, few RBCs
Septic- >WBC (50-100,000) |
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What radiographic findings may be associated w/long-standing or severe disease?
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*cartilage loss
*periarticular osteoporosis *erosions *sclerosis *subchondral lucency & deformity |
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Describe the agents used in the management of JA?
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1- NSAIDs
2- Low-dose methotrexate 3- Etanercept- TNF receptor IgG fusion protein 4- Corticosteroid injections |
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What three major conditions should be considered in the differential dx of JA?
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1- trauma
2- infection 3- neoplasm |
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What is the major difference between the childhood & adult forms of SLE?
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The childhood form has more frequent & more severe organ involvement.
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What lab studies are useful for monitoring SLE disease activity?
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Regular checking of complement components C3 & C4.
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What is the key management principle in managing childhood SLE?
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Matching the intensity of the tx to the severity of dz
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Which agents are used in the INITIAL Tx of childhood SLE?
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1- NSAIDs
2- Corticosteroids (i.e. pred) 3- Hydroxychloroquine |
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What are the major causes of M&M in children with SLE?
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Infectious complications
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______ antibodies are the most common antibodies associated with hypercoagulability in SLE.
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antiphospholipid
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MRI & CT scans may be normal in pts w/ generalized _______.
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cerebritis
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______ involvement is the most powerful predictor of morbidity in children w/SLE.
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Renal
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What are the two most common infections that may mimic SLE?
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*Infectious mononucleosis
*Streptococcal infections |
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________ are required to control the disease of SLE in the majority of pts.
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corticosteroids; & then should be tapered to the minimum dose
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What is the more common form of scleroderma in children?
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Localized -- limited to focal areas of the skin & subcutaneous tissues
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What is the typical skin lesion of localized scleroderma?
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A painLESS, NON-pruritic skin lesion that appears very similar to a scar
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Which lab tests are generally positive in scleroderma?
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ANAs --
*Anti-scleroderma 70 *Anticentromere antibodies |
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What is the mainstay of therapy in juvenile dermatomyositis?
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corticosteroids
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What is one of the longer term complications of juvenile dermatomyositis?
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subcutaneous calcifications or calcinosis
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What does diffuse scleroderma usually begin with?
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Sclerodactyly & Raynaud's syndrome
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