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Ped lupused

Ped lupused



this i take from i cant remember the website. i find it interesting and easy to understand and i upload it, so that i can read it anytime i want without losing it..... lets us together read.

this i take from i cant remember the website. i find it interesting and easy to understand and i upload it, so that i can read it anytime i want without losing it..... lets us together read.



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    Ped lupused Ped lupused Presentation Transcript

    • Lupus in children and teens Lupus Education Day October 29, 2011 Bethany Marston, MD Rheumatology and Pediatric Rheumatology University of Rochester Medical Center
    • Children get lupus too • Accounts for ~15% of all lupus patients. • More common in girls than boys. • More common in Asian-, African-American, and Hispanic than white American children. • Rare in children under 5; more common in adolescents. • May have more severe disease at onset and a more aggressive course than in adults.
    • How does lupus present in children? • Common initial presentations: – Unexplained fevers or prolonged general illness without explanation – Swollen lymph nodes – Changes in blood counts: • Low platelets • Low white blood cells • Anemia – Rashes, skin changes, or ulcers • Classic malar “butterfly” rash is only seen in up to ½ of patients. • Discoid rashes are relatively rare, but are more likely to progress to systemic disease. – Joint pain, swelling, or stiffness • Often in the hands and wrists but can affect other joints too.
    • How does lupus present in children? • Other initial presentations: – Kidney disease • Though occurs in up to 80% of patients eventually. – Neurologic symptoms • Seizures, psychosis, movement problems, etc. • Some neurologic symptoms occur in up to 20 or 30% of pts, usually within the first year. – Blood clots • Antiphospholipid antibodies are relatively common. – Other • Heart disease • Lung disease • Organ enlargement
    • Diagnosis of lupus in children • Similar to clinical criteria used in adults. Most patients can be diagnosed by history, exam, and lab testing. • Not uncommon to have “partial” presentations in children, with unclear initial diagnoses. These may progress over time. • Younger age at diagnosis (esp. before puberty) may imply more severe disease and worse prognosis. • Photosensitivity Symptoms: •Malar rash •Discoid rash •Oral ulcers •Arthritis •Pleurisy or pericarditis, •Seizures or psychosis •Raynaud’s phenomenon •Hair loss Lab tests: • Proteinuria, hematuria, other urine abnormalities • Low white blood cells, low platelets, hemolytic anemia •Positive ANA •Positive anti-Smith or anti-dsDNA •Anti-Ro, anti-La, anti-RNP, anti-histone •Coombs test •Low complements •Elevated inflammatory markers
    • What else could it be? • Depends on the presenting features. – Leukemia, lymphoma, or other malignancy – Infections – Juvenile arthritis • Can present with polyarticular joint symptoms. Many children with juvenile arthritis have a positive ANA. – Organ-specific autoimmunity • Thyroid disease • Idiopathic thrombocytopenia (low platelets) – Many other possibilities depending on presentation.
    • What causes lupus in children? • Genetics affect risk – Children with close relatives who have lupus are at higher risk, but no genetic test predicts disease perfectly. Risk increases with more affected relatives. • Environmental exposures? • Medications: – Minocycline (an antibiotic often used for acne in adolescents) is a well-known cause of positive ANA and lupus-like syndromes. Symptoms often resolve after discontinuation. – Antiseizure medications, antihypertensive (blood-pressure), and several other medications can have similar effects, though are less commonly used in children and teens. – Stimulants prescribed for ADHD can cause Raynaud’s phenomenon or can make it worse.
    • Treatment of lupus in children • Generally similar to that of adults. – Many patients require corticosteroids (e.g. prednisone or SoluMedrol) to control symptoms, especially early. – Mild lupus • Often responds to hydroxychloroquine (Plaquenil). • May benefit from NSAIDs (ibuprofen, naproxen, etc.) for musculoskeletal symptoms. – Moderate lupus • May require the addition of azathioprine (Imuran) or mycophenolate (CellCept). These are often used for hematologic or renal involvement of the disease. – Severe lupus • May be treated with cyclophosphamide (Cytoxan) or sometimes rituximab (Rituxan), for involvement of the central nervous system or for severe kidney or hematologic disease.
    • Special treatment considerations in children • May be approached more aggressively. • Corticosteroids in children • (prednisone, prednisolone, Medrol, SoluMedrol) – Growth effects – Body image • Cyclophosphamide – Fertility? Cancer risks? • Rituximab – Future immune function, vaccine effectiveness • Other medication issues
    • Some challenges in pediatric lupus • Family involvement – Medications – Monitoring and office visits – Stress, family dynamics, financial strain • School accommodations – – – – Medication Academics Gym class and athletics Absences
    • Transition to adulthood • Often very challenging. – – – – – Adolescents are “invincible.” Change of primary care physician and specialists. Change of physical location (for college or job). Change of insurance coverage. Balancing adult responsibilities with demands of managing a chronic illness. – Family changes, relationships, and pregnancy.
    • Some patient stories These are real patients. Some identifying information may have been changed.
    • B was a 16 year old white young woman. – Developed a rash on her face in mid-spring. – Started to feel progressively ill in summer, with abdominal pain, decreased appetite, fatigue, dizziness, fevers, hair loss, and puffiness. – Was diagnosed with lupus in August and treated for skin, joint, and kidney involvement. • Positive ANA, anti-dsDNA, anti-Smith, antiphospholipid antibodies, low complements, and hematuria and proteinuria. – Complicated by a blood clot in her abdomen, found several weeks later. – Lupus in teens is often similar to that in adults, with many of the same clinical features.
    • K was a 9 year old African American girl. – Started having nosebleeds in the winter. – After several ED and doctor visits, was found to have very low platelets. – Further blood and urine testing showed many signs of lupus: • positive ANA, anti-dsDNA, anti-Ro, anti-La, low complement levels, and proteinuria. – Initially did well, but treatment recently has been complicated by pronounced weight gain due to steroids. – Diagnosis sometimes isn’t made immediately. – Family challenges can complicate treatment and recovery. – Steroid side effects can be extremely challenging for early and mid-adolescents.
    • R was an 11 year old Asian boy. – Developed a diffuse rash, high blood pressure, and blood and protein in his urine. Admitted and diagnosed with lupus with nephritis. • Positive ANA, anti-dsDNA, anti-RNP, anti-Smith, antiphospholipid antibodies, very low complements, variable low white blood cells, red blood cells, and platelets, abnormal kidney function (Cr), and urinary blood and protein. – Treated with steroids and Cytoxan with good response. Maintenance has been with hydroxychloroquine and mycophenolate. – Course has been complicated by multiple blood clots. – Presentation in pre-adolescent patients, especially boys, can be severe and can require very aggressive therapy. – Antiphospholipid antibodies and blood clots can be devastating complications if not recognized.
    • G was a 15 year old girl. – Developed joint pain (hands, wrists, and knees) and swelling with morning stiffness. Also had general fatigue and felt cold. – ANA positive. – Taking minocycline for acne. – Symptoms have resolved after discontinuation of the medication and 6 months of hydroxychloroquine (Plaquenil). – Recognition of medications implicated in drug-induced lupus is important for appropriate management.
    • M was a 17 year old young woman. – Diagnosed with polyarticular juvenile arthritis at about age 15 • noted to have a positive ANA and incidental positive anti-dsDNA. – Treated for arthritis with methotrexate. Had an allergic reaction to hydroxychloroquine. – 2 yrs later, developed rapidly worsening arthritis, new mouth sores, and then a sudden onset movement disorder. – Symptoms improved with high dose steroids. – She has since remained well on mycophenolate for 4 years. – Lupus in children and teens can evolve over time, and can be quite rapidly progressive, but can also go into remission for long periods of time. – It’s important to make sure she transitions successfully to an adult care team and that all future providers know about her diagnosis and history.
    • Questions? Pediatric Rheumatology at Golisano Children’s Hospital University of Rochester Medical Center 585-275-4733