How to treat dermatomyositis? What should we pay attention to in daily life?

Author: Wu Donghai, Chief Physician of China-Japan Friendship Hospital

Reviewer: Zhang Zhuoli, Chief Physician, Peking University First Hospital

Dermatomyositis is a rare autoimmune disease that primarily affects the skin and muscles, causing muscle weakness and specific skin lesions. Due to its complexity and chronic course, the management of dermatomyositis requires a comprehensive treatment plan and careful care in the patient's daily life.

1. How to treat dermatomyositis?

The treatment plan for dermatomyositis is usually divided into two phases: acute phase and maintenance phase.

In the acute phase, the first choice of treatment is glucocorticoids. For severe patients, short-term high-dose glucocorticoid shock therapy can be used for 3 consecutive days. In addition, in the acute phase, if the condition is severe, intravenous immunoglobulin can also be used for treatment. At the same time, immunosuppressants can be used in combination to enhance the efficacy.

When the acute phase is over, the condition is basically under control and the treatment enters the maintenance phase. At this time, glucocorticoids need to be gradually reduced to the lowest dose that can maintain stable disease. However, during maintenance treatment, it is sometimes difficult to maintain stable disease with hormones alone, and relapses may occur; or attempts to reduce the dosage of hormones may lead to recurrence of the disease. In this case, second-line drugs such as mycophenolate mofetil, tacrolimus, methotrexate, cyclophosphamide, azathioprine, etc. need to be introduced to help control disease progression and help further reduce the dosage of hormones.

For patients who have improved muscle symptoms but still have residual skin symptoms, topical steroid creams, such as hydrocortisone or prednisolone acetate, can be applied to the affected areas. Another effective topical treatment is tacrolimus. In addition, hydroxychloroquine can be used to treat persistent rashes, but it has no significant effect on myositis itself.

The maintenance treatment phase generally lasts 1-3 years. During this period, if the patient does not relapse, the condition tends to be stable. If the patient has no signs of relapse within 5 years, it can be considered that the clinical cure has been achieved, and it is possible to completely stop hormone treatment.

2. What should patients with dermatomyositis pay attention to when reducing or stopping hormones?

Adjusting the dosage of a drug is usually based on changes in the patient's condition and test results. For example, if the symptoms of muscle pain and weakness disappear after taking the drug, and laboratory tests show that muscle enzyme levels return to normal, then the dosage of hormone drugs can be appropriately reduced, but it must be done in a gradual manner.

In the initial stage, if the oral hormone dose is large, the dose can be reduced once every half a month. As the dose gradually decreases, the frequency of dose reduction also needs to be reduced accordingly. For example, in the final stage, the dose may need to be reduced once a month. And the dose reduced each time also gradually decreases. For example, when the patient takes 10 tablets of hormones a day, 2 tablets can be reduced every half a month, but when it is reduced to 2 tablets, 1/4 tablet is reduced every month. This is because as the hormone dose decreases, the main focus shifts from the side effects of hormones to preventing recurrence of the disease. Close observation is required after each dose reduction to ensure that the condition is stable. If the condition fluctuates again after the dose reduction, the dose reduction should be stopped, or even restored to the previous dose.

Therefore, when adjusting the hormone dosage, the degree of reduction needs to be carefully controlled, and this process must be carried out under the supervision and guidance of a doctor.

Figure 1 Original copyright image, no permission to reprint

3. What should patients with dermatomyositis pay attention to in their daily lives?

Although dermatomyositis is challenging to treat, many patients can achieve effective control of the disease or even clinical cure through proper management. Regarding its management, the following points should be noted:

First of all, given that the cause of dermatomyositis is unknown, patients should focus on adjusting their lifestyle, ensuring a good balance between work and rest, getting enough sleep, and maintaining an optimistic attitude, because excessive psychological stress may aggravate the condition or cause recurrence.

Secondly, strictly follow the doctor's orders to take medications. Do not stop or change the dosage without authorization. All increases or decreases in medications should be done under the guidance of a doctor, and regular check-ups should be conducted so that the doctor can detect and deal with potential signs of recurrence in a timely manner.

For patients who have difficulty swallowing, it is recommended to take measures such as inserting a gastric tube as soon as possible, and then remove it after the symptoms improve to prevent food from being accidentally inhaled into the lungs and causing damage.

In addition, since ultraviolet rays may be one of the triggering factors, protective measures should be taken during outdoor activities, such as using an umbrella, wearing a sun hat and applying sunscreen, especially in the summer.

Figure 2 Original copyright image, no permission to reprint

During the remission period, avoid long-term bed rest and encourage patients to engage in moderate activities, but be careful to avoid strenuous exercise to prevent the condition from worsening.

At the same time, since most drugs for treating dermatomyositis are immunosuppressants, these drugs may also bring a series of adverse reactions, such as reduced immunity, while exerting their therapeutic effects. Therefore, patients need to pay special attention to preventing infection, reduce visits to crowded places, and regularly monitor drug side effects, such as hypertension, hyperglycemia, gastric ulcers and osteoporosis, and provide symptomatic treatment in a timely manner once they occur.

Finally, for female patients, contraceptive measures should be taken before the disease is effectively controlled, because some therapeutic drugs such as methotrexate and cyclophosphamide have the risk of fetal teratogenicity. It is recommended to consider pregnancy after the disease has stabilized for half a year, preferably after complete recovery.