What Is Systemic Sclerosis?

A condition of unknown cause leading to organ fibrosis, which mainly invades the skin, blood vessels, and internal organs, which in turn leads to a series of pathological and physiological changes that affect the patient's life.

Systemic sclerosis

A condition of unknown cause leading to organ fibrosis, which mainly invades the skin, blood vessels, and internal organs, which in turn leads to a series of pathological and physiological changes that affect the patient's life.

Western Medicine Name

Systemic sclerosis

The main symptoms

Organ fibrosis

Main cause

unknown reason

Contagious

Non-contagious

Epidemiology: Scleroderma does not differ by race or region. The incidence in the United States ranges from 4 to 20 per million people. The prevalence is between 30 and 50 years old, and women are between 3 and 50 years old. 4 times.

Etiology and pathogenesis:

At present, the true cause of this disease is still unknown, but it is generally believed that it should be caused by multiple factors.

1. Basic genetic factors: Studies have shown that HLA-DR1, DR3, and DR5 may be closely related to the disease.

2.Environmental factors: Many workers get scleroderma-like lesions due to occupational relationships, such as silica, silicide, and Vinyl choloride. In addition, drinking a poisonous Spanish oil and eating supplements containing L-tryptophan can produce crust. Clinical symptoms of disease.

3. Cell immune factors: When cells are activated by genetic or environmental factors, causing the release of immune mediators, resulting in endothelial cell damage, fibroblast proliferation and collagen proliferation, and endothelial cells will release more cells after injury Hormones make the immune response continue, eventually leading to blood endothelial fibrosis and thickening, skin hardening and internal organ damage.

Systemic sclerosis

a. Scleroderma

b. CREST syndrome

c.Overlap Syndrome

Localized sclerosis

a. Hard spot (Morphea)

b. Linear scleroderma

3. Sclerosis caused by chemicals

4. Eosinophilic myositis

5. Pseudoscleroderma

This article focuses on scleroderma and the CREST syndrome.

1. Early symptoms:

Mainly Raynaud's phenomenon, which is the earliest symptom in about 70% of patients, followed by swelling of the fingers and back of the hand.

2. Skin lesions:

Generally can be divided into three stages of edema, sclerosis and atrophy. You can see painless edema at the back of the fingers in the early stage, and then the skin will become bright and tight, and occasionally redness. The symptoms will spread from the fingers to the face and upper arms and then the whole body's skin. The mouth cannot be fully opened due to the sclerosis of the skin, and the skin is atrophied in the most advanced stage.

3. Skeletal muscle disease:

Morning stiffness and joint pain are common symptoms, but true arthritis is not common. Due to Raynaud's phenomenon, ischemia of the extremities can lead to shortening or thinning of the phalanx after a long time. In severe cases, the tip of the finger can be dissolved and absorbed. Due to the atrophy of the skin, the patient will feel weak muscles at the distal and distal ends. For every slight increase in muscle sera in the serum, the electromyogram also shows the phenomenon of muscle lesions.

4. Digestive system lesions:

Mainly the invasion of the esophagus is the main cause of peristaltic abnormalities in the lower third of the esophagus, which makes swallowing difficult, and the reflux esophagus makes the patient feel heartburn and discomfort behind the sternum. Symptoms of the small intestine are intermittent abdominal pain and diarrhea. In severe cases, intestinal obstruction can occur.

5. Lung disease:

Patients have difficulty breathing after exercise. When there is no sputum cough, attention must be paid to whether scleroderma invades the lungs. The common lesions are progressive pulmonary interstitial fibrosis. Chest X-rays show infiltration and pulmonary function. Carbon monoxide diffusibility is significantly reduced. Due to the intimal hyperplasia of the small and medium arteries in the lung, about 30% of patients are complicated by pulmonary hypertension, and severe cases cause right heart failure.

6. Heart disease:

Scleroderma can cause myocarditis, endocarditis, and pericarditis. The clinical symptoms were chest tightness, palpitations, and irregular breathing. Patients may develop hydropericardium, myocardial infarction, and arrhythmia, so care must be taken to evaluate and prevent treatment.

7. Kidney disease:

Renal disease is the most common cause of death in patients with scleroderma. When proteinuria, hypertension, and elevated urea nitrogen appear clinically, renal disease must be suspected. It can be divided into two types: acute and chronic. It occurs in patients with early diffuse scleroderma. The clinical symptoms are sudden onset, rapid formation of malignant hypertension and renal insufficiency, and hyperreninemia. This is called "renal crisis of scleroderma". This is an internal medical emergency. One of them must be treated immediately. At present, ACE inhibitor is considered to be the most effective drug. Chronic kidney disease occurs two to three years after the illness. The symptoms are mild proteinuria, hematuria and hypertension under the microscope, and mild renal insufficiency.

8.CREST syndrome

Is a collective term for five clinical symptoms, including

CalcinosisSubcutaneous calcium deposition

Raynaud's pheromeron

Esophageal dysmotilityEsophageal dysmotility

Sclerodactly-hardened fingers or toes

TelangiectasiaSkin Capillary Dilation

It is a benign localized lesion in scleroderma with a slower progression. Anti-centromere Ab in blood is closely related to this syndrome, and there have been reports that primary biliary cirrhosis is slightly related to this syndrome.

Antinuclear antibodies were positive in the blood of 1.95% of patients.

2. Anti-toposiomerase 1 (Scl-70) can be found in the blood of 20 to 40% of patients.

3. Mild to moderate red blood cell sedimentation rate increased, slightly anemia.

4. Microangiopathy hemolytic anemia can be found in patients with "sclerosis renal crisis".

5. Proteinuria and hematuria mainly depend on the degree of kidney disease.

6. Sometimes the blood muscle muscle (CPK) will rise.

7. Nail and wrinkle microvascular microscope can make an effective assessment of the condition.

Treatment principles:

The organs affected by scleroderma are treated separately because there is still no one drug that can change the natural course of the disease. The following describes each organ separately.

1. Vascular disease: Calcium blockers are effective for some Raynaud's phenomena, but the cold environment should basically be avoided.

2. Skin: D-Penicillamine has some effects on early skin lesions, but other drugs cannot prove the actual effect.

3. Gastrointestinal system: mainly for symptomatic treatment, including not lying down when eating and drinking, and raising head when sleeping. Taking antacids to control reflux esophagitis, Cisapride can partially improve gastrointestinal motility, with some effects.

4. Renal disease: Angiotensin-Converting Enzyme (ACE) inhibitors is currently considered to be the most effective drug for controlling renal disease and mortality.

5. Cardiopulmonary disease: vasodilators can be used to treat pulmonary hypertension and heart failure, but there is no special drug to control pulmonary fibrosis.

What should I pay attention to? What about diet?

Once the patient is injured, it is not easy to recover.

Secondly, if you have symptoms of sclerosis, you should pay attention to functional exercise, more activities, especially hands, otherwise it can cause deformities.

Diet is mainly to avoid hard, hot, difficult to digest diet.