Lupus in Children and Teenagers. Arielle Hay, MD Pediatric Rheumatologist Nicklaus Children s Hospital

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2 Lupus in Children and Teenagers Arielle Hay, MD Pediatric Rheumatologist Nicklaus Children s Hospital

3 Systemic Lupus Erythematosus (SLE) Chronic Illness What is lupus? Autoimmune Multisystem Antinuclear Antibodies

4 Chronic Illness - Condition that persists or is long lasting - Lupus tends to have periods where the disease is active flares and inactive quiescent Autoimmune Multisystem What is lupus? Antinuclear Antibodies

5 What is lupus? Chronic Illness Autoimmune - The job of the immune system is to protect us from harmful invaders such as bacteria or viruses - In autoimmune conditions the immune system attacks a person s own body - There are many different types of autoimmune conditions Multisystem Antinuclear Antibodies

6 What is lupus? Chronic Illness Autoimmune Multisystem Can affect any organ in the body Antinuclear Antibodies

7 What is lupus? - Chronic Illness Autoimmune Multisystem Antinuclear Antibodies Hallmark of the disease, more details coming up

8 How is lupus diagnosed? Might be suspected based on certain symptoms Rash, unexplained fever, arthritis, abnormal blood counts First test usually ANA (antinuclear antibody) If this is negative, then highly unlikely to have lupus Pediatric rheumatologist will do a comprehensive history, exam and labs tests to confirm/rule out lupus

9 What is the ANA? Anti Nuclear Antibody: protein made by the immune system whose job is to recognize and neutralize harmful agents known as antigens

10 Anti Nuclear: referring to the cell nucleus Antibody: protein made by the immune system whose job is to recognize and neutralize harmful agents known as antigens

11 In lupus the immune system makes antibodies directed at antigens which are parts of the nucleus of the cell Hence the term: Antinuclear antibodies.

12 Most like a combination of Environmental and Genetic factors Environmental UV light Viral infections Hormones (Women:Men 9:1 Boys:Girls 4:1) Certain medications Diet? Genetic What causes lupus? Occurs more frequently in certain populations (African-Americans, Afro-Caribeans, Hispanics, Native Americans) Identical twin risk 20-30%, Fraternal twins or siblings 2-5% risk Slightly increased risk in first degree relative, but low overall

13 Childhood vs Adult Lupus Much less common in kids: 50/100,000 in adults / 100,000 in kids 10x less common than JIA or Type 1 diabetes Course tends to be more aggressive in kids Higher disease activity More kidney involvement More likely to need immune suppressing medications Kids have the disease longer, more potential for damage from disease and side effects of medications Growth and Development Children are not little adults Impacts on growing skeleton Affects on puberty

14 What symptoms does lupus cause? Everybody s lupus is different! Can affect ANY part of the body Some people have many parts affected Some people only have a few areas affected

15 Skin About 90% of kids with lupus have some kind of skin involvement

16 Skin Raynauds: color changes to fingers when exposed to the cold

17 Joints Joint pain, muscle pain and arthritis are common Arthritis of lupus does NOT cause permanent damage (different from arthritis in JIA)

18 Blood May have anemia (low hemoglobin) May have low platelets May have low white blood cells May have completely normal blood counts

19 Kidneys Known as lupus nephritis Occurs in about 50% of childhood lupus patients Majority will present in the first 2 years of diagnosis One of the few silent features That s why we check urine tests at every follow up visit Can detect protein and/or blood in the urine which may be a sign of kidney involvement

20 Central Nervous System Most common symptom is headache Other RARE symptoms include concentration difficulty, seizures, mood problems, strokes

21 Reminder Just because something CAN happen in a child with lupus does not mean it WILL happen

22 How is lupus treated? Everybody s lupus is different! Many different medications available Type of medications based on which parts of the body are affected Almost everyone with lupus will be prescribed hydroxychloriquine (Plaquenil)

23 How is lupus treated? Hydroxychloroquine (Plaquenil) Helps with fatigue, rash, blood counts, joint pains Might prevent disease flare Glucocorticoids (Prednisone, Methylprednisolone, etc) Used for many different manifestation of lupus Dose may be high or low depending on the problem (low for arthritis, high for kidney disease) Many side effects Try to use lowest doses possible Cyclophosphamide (Cytoxan) Usually reserved for kidney disease or central nervous system disease Mycophenolate mofetil (Cellcept) and Azathioprine (Imuran) Steroid sparing agents Can be used for various manifestations of lupus Anti B-cell therapies Rituximab, Belimumab

24 What to expect Frequent doctor visits More frequent early on When things are stable usually every 3 months Multiple medications might be needed Depends which parts of the body are involved Sometime switching medications is necessary if goals aren t being achieved Getting back to normal daily life Treatments are aimed at controlling disease activity and managing and pain, uncomfortable symptoms or medication side effects

25 Ask questions! What can you do? Ask your doctor to explain recommendations Ask about alternatives Talk about side effects of medications Keep appointments Especially when things are going well Tell your doctor about any complimentary or alternative treatments Remember SUNSCREEN!!! Healthy lifestyle choices Diet, exercise, stress reduction

26 Thank you!