Some people have stiff expressions, as if they are wearing masks, but in fact they are suffering from this disease!

Author: Mu Rong, Chief Physician, Peking University Third Hospital

Reviewer: Wu Xueyan, Chief Physician, Peking Union Medical College Hospital

Have you ever noticed this type of people in life? Their expressions are restricted. It is difficult for them to make various expressions and they look very stiff. It is also difficult for them to eat and open their mouths, as if they are wearing a mask. Therefore, they are also called "mask face". Their skin wrinkles become fewer and cannot be pinched. In fact, they have a disease called scleroderma, or systemic sclerosis.

Figure 1 Original copyright image, no permission to reprint

The name of this disease comes from the clinical symptoms, which are that the patient's skin will harden. Of course, in the early stage, the skin may not be hardened, but there will be some edema. In the later stage, fibrosis will gradually appear, and the skin cannot be pinched. Some people describe it as parchment paper. Some patients may not have obvious edema in the early stage, and will directly manifest as skin hardening.

Systemic sclerosis is an autoimmune disease. Its pathogenesis is not very clear so far. It is generally recognized that autoimmune dysfunction leads to vascular inflammation and the occurrence of fibrosis.

The incidence of systemic sclerosis is relatively low, and it is more common in women than in men, which may be related to sex hormone levels. In addition, some people have found that it may be related to some special occupations, such as contact with some organic solvents or silicon-like things, but it has not been recognized so far because quite a number of patients have never been exposed to any special chemical reagents.

The pathological basis of systemic sclerosis is vascular inflammation and tissue fibrosis, which can affect various organs. In addition to skin sclerosis, there are also various clinical manifestations.

1. In addition to skin sclerosis, what other symptoms does systemic sclerosis have?

The most common is Raynaud's phenomenon, which occurs when the peripheral blood vessels in the hands or toes become inflamed or occluded. When you are emotionally excited or exposed to cold, the blood vessels contract and spasm, causing the skin of your fingers or toes to turn white. When you return to a warm environment, the blood vessels dilate, and a large amount of blood stasis returns to the original ischemic area, causing the fingers to turn purple and eventually return to normal color.

Figure 2 Original copyright image, no permission to reprint

Raynaud's phenomenon is not only seen in scleroderma, but can be seen in many other diseases. If Raynaud's phenomenon occurs, you must go to the hospital for examination. Do not be satisfied with the diagnosis of Raynaud's phenomenon alone. Most people may suffer from systemic diseases that cause Raynaud's phenomenon.

If the affected blood vessels of systemic sclerosis are completely blocked and necrotic, it will cause gangrene of the extremities, leading to necrosis of the fingers or toes, and some may even require amputation.

If large blood vessels, such as the pulmonary artery, are affected, it will lead to pulmonary hypertension, which is very high and increases the patient's risk of sudden death. Long-term pulmonary hypertension can lead to heart disease and heart failure.

Vascular inflammation leads to fibrosis. If fibrosis occurs in the internal organs, it will cause hardening of various organs. For example, if the digestive system is affected, it will cause gastroesophageal reflux, atrophic gastritis, diarrhea, intestinal lesions, intestinal obstruction, esophageal motility disorders, etc., and various digestive tract symptoms will appear, and food will be difficult to digest and absorb. If it occurs in the lungs, it will cause pulmonary fibrosis, causing dry cough, decreased mobility, dyspnea, etc. If it occurs in the heart and kidneys, it will cause cardiac fibrosis, abnormal renal tubular function, and finally heart and kidney failure.

2. What examinations are needed for systemic sclerosis?

Systemic sclerosis is an autoimmune disease, so autoimmune-related tests are needed to detect autoimmune antibodies, such as antinuclear antibodies, anti-Scl-70 antibodies, anti-centromere antibodies, and anti-RNA polymerase III antibodies, to help confirm the diagnosis. The pathological basis of systemic sclerosis is vascular inflammation, so vascular-related tests, such as capillaroscopy, are needed.

In addition, systemic sclerosis affects a wide range of organs. In addition to diagnosis, it is also necessary to examine the extent of organ involvement and perform relevant examinations based on clinical symptoms, such as examinations of the digestive tract, lungs, heart, and kidneys, to provide guidance for subsequent treatment.

Although the incidence of systemic sclerosis is not high, it has a great impact on the patient's quality of life. It is the autoimmune disease with the highest mortality rate, so people are paying more and more attention to it. After diagnosis, active treatment should be given to reduce the occurrence of complications.

3. How to treat systemic sclerosis?

So far, there are not many treatment methods. The earlier the treatment, the more options there are. The later the treatment, the more limited the treatment options may be.

At present, the treatment is mainly aimed at autoimmune function, with immunosuppressants as the core drugs, and hormones are used more cautiously. The difference between systemic sclerosis and other autoimmune diseases is that if large doses of hormones are used, renal crisis may be induced, leading to a sharp decline in renal function. Therefore, superb medical skills are required to formulate treatment plans and control the dosage and timing of hormones, so that patients can benefit and it is relatively safe. Therefore, immunosuppressants are mainly used, and hormones are supplemented.

In addition to immunosuppressants and hormones, anti-fibrotic drugs are also being tried in the treatment of systemic sclerosis, which can bring new hope to patients with rapidly progressive pulmonary fibrosis.

Systemic sclerosis is a chronic disease. Because of the disorder of autoimmune function, it must be corrected by medication. If the medication is reduced or stopped without authorization, the condition may gradually worsen. The vast majority of patients need long-term medication. The treatment plan should be adjusted in time according to changes in the condition. It is hoped that under long-term drug control, the patient's quality of life can be improved and his life span will not be affected.

4. What should patients with systemic sclerosis pay attention to in their daily lives?

First, keep warm, because this disease may worsen vascular inflammation when exposed to cold.

Second, avoid mental stimulation. When you are under great pressure or mentally stimulated, your blood vessels will also contract, which is not good for your condition.

3. Avoid smoking, as smoking can also cause vasoconstriction.

4. Avoid infection. Because this disease may affect various organs and is often complicated by infection, avoid going to crowded places.

5. To prevent the disease from getting worse, it is crucial to adhere to medication.

We know that a healthy lifestyle is beneficial to the disease, so patients with systemic sclerosis must ensure adequate sleep and nutrition, and eat a reasonable diet. As long as the disease is under control, they can live like normal people without too much psychological burden.