Lupus And The Child

Systemic lupus in children and adolescents in ten key points: Editor: Dr. B. Bader-Meunier, Reference Center "Juvenile Arthritis", Necker Hospital, Paris) Editors: Drs Quartier (Reference Center "Arthritis Juveniles", 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, especially the skin, joints, blood cells and kidneys. SLE is a chronic disease which means it can last a long time. Autoimmune means that it is a disease of the immune system that will cause an attack on the patient's own organs. Systemic means that it can reach several organs of the body. The word lupus comes from the Latin word wolf because of the characteristic aspect of the cutaneous involvement of the face with a red plaque in the shape of the mask which is called "wolf".

The management of pediatric lupus should be coordinated by a specialized hospital center in connection with the local treatment and / or hospital physician. The treatment aims to treat the outbreaks of the disease, to prevent the occurrence of thrust and the occurrence of sequelae, to treat pain when it is present. The background treatment most often comprises platinum, sometimes combined with treatment with nonsteroidal anti-inflammatory drugs, corticosteroids and / or other immunosuppressants (imurelR, cellceptR, endoxanR in particular), and analgesics.

Scheduled consultations should be done at least every 2 to 3 months depending on the nature of the events. In particular, they will make it possible to ensure that Lupus is well controlled, to possibly adapt the treatment, and to look for complications, in particular renal, by researching the presence of proteins in the urine. You will find a more detailed description of the manifestations and treatments of Systemic Lupus in references 1 and 2.

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

Unusual signs may result from an outbreak of the disease, a side effect of treatment or infection (especially in case of treatment with corticosteroids and / or immunosuppressants), and should lead to a rapid medical examination. This may include, for example, fever, headache or severe stomach pain, pallor, easy bruising or bleeding through brushing, behavior change (but this list is not exhaustive).

3 What to avoid

1. Sex exposure: It can cause an eruption, sometimes serious, on the areas of the skin exposed to the sun, and sometimes also cause a relapse of the disease. The wearing of long sleeves and a hat, and the regular application (every 2 hours and after each bath) of powerful sunscreen creams (index at least equal to 50) is therefore necessary in all children and adolescents with a Lupus.
2. Smoking: It decreases the effectiveness of PlaquenilR and increases the risk of cardiovascular complications in adulthood.
3. The abrupt discontinuation of treatment, especially cortisone can lead to a serious complication (acute adrenal insufficiency). Stopping other treatments may result in Lupus flare.
4. The cessation of specialized follow-up, especially during adolescence or when transferring Pediatric care to an adult medicine service. Specific care for each medical team can then be useful.
5. Take oestroprogestative oral contraception (the most usual) without discussing it with the doctor responsible for the management of Lupus.

4 Is a diet necessary?

A diet is necessary in case of treatment with corticosteroids. A plan sheet will be given for this. A diet low in sugar is essential to avoid excessive weight gain if cortisone doses are important. A low salt diet may be helpful depending on your child's condition and cortancyl dose.

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

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

Vaccinations against influenza, pneumococcus and vaccination against cervical papillomavirus infections in adolescents before the first sexual intercourse are recommended according to the usual pattern. Vaccine efficacy may be decreased by concomitant immunosuppressive therapy, and booster may be necessary after discontinuation of immunosuppressive therapy. Vaccination should be avoided during an outbreak of the disease.

6 Which contraception to use?

The oestroprogestative pill (most often prescribed) is most often contraindicated and can only be considered after a specialized evaluation. Progestin-only pills are generally 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 care can be done, but it should be noted all treatments in progress. Precautions are necessary in case:

1 / treatment with cortisone and / or immunosuppressants: antibiotic treatment before and after the operation may be proposed in some cases because there is an increased risk of infection

2 / prolonged treatment with cortisone: it should never be interrupted 3 / treatment with antivitamin K in cases of anti-phospholipid syndrome: treatment with oral anticoagulant should be stopped just before the operation (including tooth extraction) and resumed immediately after and replaced by subcutaneous anticoagulation according to medical prescriptions. In some cases, antibiotics will be prescribed for dental care.

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

There are no contraindications to travel (make sure you have enough medicines for long trips). If anti-phospholipid antibodies are present in the blood, it may be advisable in some cases to give a subcutaneous injection of low molecular weight heparin one hour before departure if 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 schooling is possible in the vast majority of cases. In case of significant absenteeism, home schooling or other facilities may be offered through the MDPH (House of Disability).

Regular physical activity should be encouraged in children during periods of remission. However, it is necessary to take precautions under certain conditions: 1° violent sports are to be avoided in case of drop of platelets or anticoagulant treatment, which promotes bleeding 2 ° sports activities are to be avoided in case of painful joint damage and the more often during Lupus flares.

10 What financial aid is possible?

Systemic Lupus is recognized as a long-term condition (ALD) with 100% coverage based on the social security tariff for Lupus care, treatment and transport costs. Depending on the impact of your child's illness on daily life, additional help may be requested from the MDPH.