Don’t be afraid of the word “wolf”, how to dance with the “wolf”?

May 10th of each year is World Lupus Day.

The most characteristic manifestation of systemic lupus erythematosus is the butterfly-shaped erythema across the bridge of the nose and both cheeks. The name " lupus " comes from the fact that people once believed that the butterfly-shaped erythema on the face of the disease looked like a wolf bite, hence the name " systemic lupus erythematosus ".

When lupus erythematosus lesions are limited to the skin, it is called cutaneous lupus erythematosus ; when the lesions involve multiple organs throughout the body, it is called systemic lupus erythematosus (SLE). We call childhood-onset SLE cSLE (childhood-onset SLE).

Currently, cSLE, like chronic diseases such as hypertension and diabetes, cannot be completely cured, but with the joint efforts of doctors and patients, the disease can be controlled and alleviated, and some cSLE children can completely stop taking medication. Most children can study, work and live normally after their disease is controlled.

We already know that SLE is a disease that affects multiple organs of the body. Therefore, the treatment strategy for SLE needs to fully evaluate the impact of the disease on the child's body, and formulate a personalized treatment plan based on standardized diagnosis and treatment plans, after full communication with the child and his parents, and after comprehensive consideration of family factors, children's academic performance and other social factors, in order to delay the progression of the disease to the greatest extent possible. Therefore, it is recommended that children with SLE receive treatment from a pediatric rheumatologist as soon as possible.

As parents, what they are most concerned about is

Can the child be cured?

Can I stop taking the medicine?

How long should I take hormones?

In fact, this is the goal of SLE treatment - to reduce the dosage of glucocorticoids as much as possible, effectively maintain clinical remission or low disease activity, and prevent aggravation of organ damage and recurrence of the disease. Most of the drugs used to treat SLE have side effects. Simply put, our goal is to reduce the side effects of drugs as much as possible while controlling the disease. Therefore, doctors strive to practice the principle of " least harm, best efficacy ", not only to plan drug treatment, but also to minimize the impact on children's studies and life, and improve their quality of life.

Currently, there are many types of drugs used in cSLE, including glucocorticoids, hydroxychloroquine, cyclophosphamide, mycophenolate mofetil, methotrexate, cyclosporine, tacrolimus , etc. At the same time, biological agents such as rituximab and belimumab have also been proven to be effective.

With so many medicines, how should I choose?

The specific treatment plan needs to be formulated by an experienced pediatric rheumatologist based on the results of a detailed evaluation of the child's condition. The following first introduces several commonly used drugs.

(1) Glucocorticoids

Glucocorticoids are the basic drugs for the treatment of SLE, which are what we often call hormones. They can be injected intravenously or orally. The doctor will determine the route and dosage of medication according to the severity of the child's condition and weight. However, drugs are a double-edged sword. The use of hormones will bring a variety of side effects. They secretly spy and make people restless. After long-term use, weight gain, high blood pressure, arteriosclerosis, blood sugar fluctuations, cataracts, osteoporosis, depression... may follow one after another. But becoming a Buddha or a demon depends on the person holding the sword. While enjoying the benefits brought by hormones, patients should also re-examine this relationship.

Only by facing the problem can we solve it.

The so-called correct approach means respecting facts and maintaining balance. Doctors will develop a comprehensive treatment plan based on comprehensive considerations. Choosing the right dosage and appropriate course of treatment is the way to survive when using glucocorticoids. This is just like a shrewd chef who knows how much heat to use for cooking different dishes. Smart doctors have gradually mastered the "heat" of the treatment plan for children. Nowadays, most of the side effects of hormones can be prevented and treated under the guidance of doctors' skilled techniques.

Is there any way to predict the efficacy and side effects of drugs in advance?

Through the drug genome, we may be able to learn some key information in advance. In clinical practice, it is often found that two children have the same diagnosis, the same general condition, and are treated with the same drug, with the same dosage and usage, but the efficacy is very different! One of the reasons for such differences is the difference in drug genetics. For example, it is currently known that the ABCB1 gene is related to the incidence of adverse reactions, and children with TT genotype and T allele may need other treatment options. When facing glucocorticoids, parents should understand:

1. It has strong immunosuppressive and anti-inflammatory effects, so its effects are wide-ranging and complex, and vary with different doses;

2. It plays an irreplaceable bridge role in the treatment of immune diseases. Doctors will choose the appropriate dose and course of treatment;

3. Nowadays, most side effects of glucocorticoids can be prevented and treated with the cooperation of parents and the skilled techniques of doctors.

Of course, glucocorticoids themselves, as such a different and interesting soul, are still working hard. They certainly not only hope to enjoy the fun of expanding the boundaries of treatment, but also want to be a star in the starry sky of human medical history.

(2) Hydroxychloroquine

Hydroxychloroquine is the basic treatment for SLE; because of its side effects on the eyes, the doctor will check the child's ophthalmological condition before and during use. (3) Immunosuppressants

For SLE patients who do not respond well to hormone combined with hydroxychloroquine treatment, immunosuppressants need to be added; for those with organ involvement, it is recommended to add immunosuppressants during initial treatment. However, for the aforementioned cyclophosphamide, mycophenolate mofetil, methotrexate, cyclosporine, etc., the specific choice of immunosuppressant should be based on the evaluation of the affected organs, the diagnosis and treatment guidelines and the child's physical tolerance. (4) Biological agents

If the standard treatment regimen (hydroxychloroquine combined with glucocorticoids, with or without immunosuppressants) is still ineffective, it is difficult to reduce the dose of hormones, or relapses are frequent, biological agents such as belimumab and rituximab can be added.

The growth and development of SLE children is a hot topic of concern for many parents. It is recommended that parents take their children to assess their growth and development status and trends regularly, and complete assessments of bone age, growth hormone levels, etc. when necessary. For children with special needs and if the condition permits, growth hormone injections may also be considered, but this must be completed after a comprehensive evaluation by multiple disciplines such as pediatric immunology specialists and endocrinology departments, while ensuring safety.

SLE children can go to school normally after their condition stabilizes. If they encounter special periods in their studies, they can discuss with their doctors whether they need to adjust their treatment plans. In summary, we hope that with the joint efforts of parents, doctors, children, and society, we can strive to allow SLE children to have the same wonderful life as normal children.

Tips from Peking Union Medical College:

① Since taking the medicine can suppress the immune system, it will make the patient more susceptible to infection or mask the infection. Therefore, special attention should be paid to long-term use and adverse reactions must be closely observed.

② During medication, blood pressure, weight, blood sugar, blood electrolytes, fecal occult blood should be checked regularly, and an ophthalmological examination should be performed.

③If you need to use glucocorticoids for a long time, you should pay attention to supplementing calcium and vitamin D to prevent osteoporosis and fractures.

④ It is recommended that parents take their children to assess their growth and development status and trends regularly. If necessary, growth hormone injections can be considered, but this must be done under safe conditions after a comprehensive evaluation by a doctor.

The theme of this article comes from the theme of the 2023 issue 3 of the Peking Union Medical College Journal: Pediatric Rheumatic and Immune Diseases

Editor: Liu Yang and Zhao Na

Proofreading: Li Na, Li Yule, Dong Zhe, Li Huiwen

Producer: Wu Wenming

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