Lupus in children and teens

Lupus Education DayOctober 29, 2011

Bethany Marston, MDRheumatology 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.

Symptoms:• Photosensitivity•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 HospitalUniversity of Rochester Medical Center

585-275-4733