By | June 11, 2021

Systemic lupus in children and adolescents in ten key points: Editor: Dr. B. Bader-Meunier, Reference Center “Juvenile Arthritis”, Necker Hospital, Paris) Publisher: Drs Quartier (Reference Center “Juvenile Arthritis”, Paris), Dr. Ranchin (Pediatric Nephrology, Lyon)

1 What is Systemic Lupus? How is Lupus treated and supervised in children and adolescents?

SLE is an autoimmune disease that can affect one or more organs, including the skin, joints, blood cells, and kidneys. SLE is a chronic condition, which means it can last for a long time. Autoimmune means that it is an immune system disorder that causes an attack on the patient’s own organs. Systemic means that it can affect several organs in the body. The word lupus comes from the Latin word wolf because of the characteristic appearance of the skin involvement of the face with a red spot in the form of a mask called “wolf”.

Treatment of pediatric lupus must be coordinated by a specialized hospital center in collaboration with the local and / or treating hospital doctor. Treatment aims to treat flare-ups, prevent flare-ups and sequelae, and treat pain when it is present. Disease-modifying treatment most commonly includes platinum, sometimes combined with treatment with nonsteroidal anti-inflammatory drugs, corticosteroids, and / or other immunosuppressants (particularly ImurelR, cellceptR, EndoxanR) and analgesics.

Scheduled consultations should be carried out at least every 2 to 3 months, depending on the nature of the events. In particular, they will make it possible to ensure that the lupus is well controlled, possibly adjust treatment, and check for complications, particularly of the kidney, by looking for proteins in the urine. For a more detailed description of the manifestations and treatments of systemic lupus, see References 1 and 2.

2 What are the signs that should lead to prompt medical advice?

Unusual signs may result from onset of illness, treatment side effects, or infection (especially with corticosteroid and / or immunosuppressant treatment) and should prompt a medical examination. These can be, for example, fever, severe headache or abdominal pain, paleness, easy bruising or bleeding when brushing your teeth, changes in behavior (however, this list is not exhaustive).

3 What to avoid

  1. Sexual exposure: It can cause a sometimes severe rash on sun-exposed areas of the skin, and sometimes cause the disease to relapse. Wearing long sleeves and a headgear as well as the regular use (every 2 hours and after every bath) of strong sunscreens (index at least equal to 50) is therefore necessary for all children and adolescents with lupus.
  2. Smoking: It decreases the effectiveness of PlaquenilR and increases the risk of cardiovascular complications in adulthood.
  3. Suddenly stopping treatment, especially cortisone, can lead to serious complications (acute adrenal insufficiency). Stopping other treatments can lead to a flare-up of lupus.
  4. The end of specialist follow-up care, especially in adolescence or when pediatric care is transferred to an adult medical service. Specific support for each medical team can then be useful.
  5. Take oral combined contraceptives (the most common) without discussing with the doctor who treats lupus.

4 Is a diet necessary?

Diet is required when using corticosteroids. You will be given a card sheet for this purpose. A low-sugar diet is essential to avoid excessive weight gain when cortisone doses are important. A low-salt diet may be helpful depending on your child’s condition and dose of cortancyl.

5 Which vaccines are recommended? What are the contraindicated vaccines?

The usual vaccination schedule must be followed in accordance with the applicable vaccination recommendations. In the case of treatment with an immunosuppressant, however, live vaccines with biotherapy and / or high-dose corticosteroids (measles, rubella, mumps, yellow fever, oral polio, BCG vaccine) are contraindicated.

Vaccinations against influenza, pneumococci and vaccinations against infections of the cervix with papillomaviruses in adolescent girls before the first sexual intercourse are recommended according to the usual schedule. Concomitant immunosuppressive therapy may reduce the effectiveness of the vaccine, and a booster vaccination may be required after immunosuppressive therapy is stopped. Vaccination should be avoided during an outbreak of the disease.

6 Which contraception to use?

The combined hormonal pill (usually prescribed) is mostly contraindicated and can only be considered after a specialist examination. In general, progestogen-only pills are preferred, such as chlormadinone acetate (Luteran®), cyproterone acetate (Androcur®), nomegestrol acetate (Lutenyl®) and desogestrel (Cerazette®).

7 Are there any special precautions to consider before surgery, dental care?

Any surgery and dental treatment can be performed, but any ongoing treatments should be recorded. Precautions are required when:

1 / Treatment with cortisone and / or immunosuppressants: in some cases antibiotic treatment can be offered before and after the operation, as there is an increased risk of infection

2 / Prolonged treatment with cortisone: should never be interrupted 3 / Treatment with antivitamin K for antiphospholipid syndrome: Treatment with oral anticoagulants should be discontinued shortly before the operation (including tooth extraction) and resumed immediately afterwards and replaced by subcutaneous anticoagulation according to the doctor Regulation. In some cases, antibiotics are prescribed for dental treatment.

8 Are there any special precautions to consider before traveling abroad?

There are no contraindications to travel (make sure you have enough medication for long trips). If antiphospholipid antibodies are present in the blood, in some cases it may be advisable to give a subcutaneous injection of low molecular weight heparin one hour before departure if you are traveling by plane to avoid the occurrence of thrombosis. The list of specialized centers abroad is available on the PRINTO website (Ref. 1).

9 Is it possible to have normal schooling? A sports activity ?

Normal school attendance is possible in the vast majority of cases. In the event of significant absences, home tuition or other facilities can be offered through the MDPH (Maison du Handicap).

Regular physical activity should be encouraged in children who are in remission. However, precautions must be taken under certain conditions: 1 ° violent sport should be avoided in the case of a platelet drop or anticoagulant treatment that promotes bleeding 2 ° sporting activities in painful joint damage and more frequently during flare-ups.

10 What financial aid is possible?

Systemic lupus is recognized as a long-term disease (ALD) with 100% coverage based on the social insurance tariff for the costs of lupus care, treatment and transport. Additional help from the MDPH may be requested depending on the impact of your child’s condition on daily life.

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