September 24, 2010

 20% diagnosed in childhood  Mostly in adolescence  F:M ratio  Prior to puberty - 3:1  After puberty - 9:1  Native Americans > African Americans > Hispanics > Chinese > Filipinos  More severe disease in AA and Hispanics  Multigenic

 Most common symptoms in teenagers  Fever  Rash  Mucositis  Arthritis  Constitutional ▪ Weight loss ▪ Malaise

 Butterfly rash  Malar erythema  Photosensitive  Sparing of nasolabial folds  Discoid lesions  Scalp or extremities  Photosensitive  Maculopapular lesions  Occur anywhere

 Alopecia  Begins frontal  Spreads diffusely  Rarely permanent  Vasculitic  Palmar erythema  Mucosal lesions  Ulcers  Sometimes painless

 2 nd leading cause of morbidity and mortality  Psychiatric  Psychosis  Seizures  HA  Lupus cerebritis ▪ Also difficulty with concentration, memory, depression or decline in school performance  Less commonly  Chorea, neuropathies, transverse myelitis

 Any layer of the heart affected  Pericardium  Most common  Myocarditis  CHF, arrhythmia  Libman-Sacks endocarditis  At risk for SBE

 Raynaud phenomenon  Very common in adolescents  Cold, emotional stress, caffeine, cigarette smoke  At risk for infarction  Premature atherosclerosis

 Pleuropulmonary disease  Pleuritic chest pain ▪ Effusion  60% of adolescent patients will have abnormal PFTs  Other  Pneumonitis  Pulmonary Hemorrhage  Pulmonary HTN

 Abdominal pain is common  Pancreatitis  Mesenteric vasculitis  Peritonitis  Hepatitis  Question #2

 #1 cause of morbidity  75% of children within first 2 years  Signs  Microscopic hematuria and proteinuria  HTN  Decreased GFR  Elevated BUN or Cr

 Renal Biopsy

 Arthralgia  Arthritis  Nonerosive, nondeforming  Small and large joints  Myalgia  Proximal muscle weakness  Myositis  Possible MCTD

 Cytopenia  Any cell line  Anemia 50% ▪ Normocytic normochromic ▪ Anemia of chronic disease or autoimmune hemolytic  ITP

 ANA  Best screening test  Positive in almost all that have active disease  Not specific  More specific if ≥ 1:1,280  Other diseases ▪ JIA, Dermatomyositis, scleroderma, thyroid disease

 Complement (C3, C4)  Reduced levels  May indicate need for aggressive therapy

 Antiphospholipid antibodies  CBC  Electrolytes  BUN and Cr  Hepatic Enzymes  Acute-phase reactants  UA

 Consult with Rhematology  Treatment depends on clinical manifestations  Emerging morbidity is associated with treatment  Close monitoring

 Improving  Juvenile-onset  92% - 5 year survival rate  85% - 10 year survival rate  Major causes of death  Renal disease  Infection  CNS disease