Mixed connective tissue disease is a fairly rare pathology. The clinical picture of this ailment is characterized by a combination of signs of various collagenous diseases. This pathology is also called Sharpe syndrome. Most often, this symptom complex is noted in the puberty and in middle-aged patients. In a neglected form, pathology can lead to serious and life-threatening consequences. In the article, we will consider in detail the symptoms and treatment of mixed connective tissue disease.
What it is
In the past, this pathology was very difficult to diagnose. After all, the signs of Sharpe's syndrome resemble manifestations of a variety of rheumatic ailments. Only relatively recently has this disease been described as a separate autoimmune disorder.
With a mixed disease of connective tissue (TSFST), the patient has certain signs of various rheumatic pathologies:
- systemic lupus erythematosus ;
- dermatomyositis;
- scleroderma;
- rheumatoid arthritis;
- polymyositis.
The patient does not necessarily have a complete clinical picture of all of the above diseases. Usually there are several symptoms characteristic of different autoimmune pathologies.
ICD code
According to ICD-10, a mixed disease of connective tissue is allocated into a separate group of pathologies under the code M35 ("Other connective tissue diseases"). The full code of the SZST is M35.1. This group includes cross rheumatic syndromes. The word "cross" means that with this pathology, signs of various diseases of the connective tissue (collagenoses) are noted.
Causes
The exact causes of Sharpe's syndrome have not yet been elucidated. Mixed connective tissue disease is autoimmune. This means that human immunity for unknown reasons begins to attack its own healthy cells.
What can provoke such a malfunction in the work of the body's defenses? Doctors suggest that prolonged intake of certain medications can affect the functioning of the immune system. A major role in the occurrence of autoimmune reactions is played by hormonal disorders and age-related rearrangement of the endocrine system. For this reason, SZST is often observed in adolescents and in women during menopause.
A negative emotional background can also affect the functioning of the immune system. The psychosomatics of mixed connective tissue disease is associated with serious stress. This pathology is more often observed in people prone to depression, as well as in patients with neurosis and psychosis.
Sharp syndrome is commonly seen in people who have a hereditary predisposition to rheumatic diseases. The impact of adverse factors is only the trigger for the occurrence of autoimmune lesions.
Symptomatology
A mixed disease of connective tissue proceeds in a chronic form and without treatment gradually progresses. This pathology is systemic, it affects not only the skin and joints, but the whole body.
Very often, the initial sign of the disease is a violation of blood circulation in the fingers and toes. This resembles manifestations of Raynaud's syndrome. Due to spasm of blood vessels in humans, the fingers and toes become pale and cold. Then the skin on the hands and feet acquires a bluish tint. Cooling of the extremities is accompanied by severe pain. Such vasospasm can occur several years before the development of other signs of the disease.
Most patients experience joint pain. The fingers are very swollen, the movements become painful. Muscle weakness is noted. Due to pain and swelling, it becomes difficult for the patient to bend his fingers and hold various objects in his hands. This is similar to the initial manifestations of rheumatoid arthritis or systemic lupus erythematosus. However, in this case, bone deformation is very rare. In the future, other articular joints are involved in the pathological process, most often knees and elbows.
Subsequently, a person develops red and white spots on the skin, especially in the area of the hands and face. The condensed areas of the muscles are felt, as in systemic scleroderma. The skin thickens, in rare cases, sores appear on the epidermis.
The patient's health gradually worsens. Joint pain and skin rashes are accompanied by the following symptoms:
- general weakness;
- a feeling of stiffness in the joints after a night's sleep;
- hypersensitivity to ultraviolet light;
- drying out of the oral mucosa and difficulty swallowing;
- hair loss
- unreasonable weight loss with normal nutrition;
- fever;
- enlarged lymph nodes.
In advanced cases, the pathological process extends to the kidneys and lungs. Glomerulonephritis occurs, the protein content in the urine increases. Patients complain of chest pain and shortness of breath.
Possible complications
Mixed connective tissue disease is a rather dangerous pathology. If the pathological process affects the internal organs, then with poor-quality treatment, the following complications may occur:
- renal failure;
- stroke;
- inflammation of the mucous membrane of the esophagus;
- perforation of the intestinal wall;
- myocardial infarction.
Such complications are noted with an unfavorable course of the disease and in the absence of proper therapy.
Diagnostics
The treatment of SZST is carried out by a rheumatologist. Symptoms of a mixed disease of connective tissue are extremely diverse and resemble the manifestations of many other pathologies. Because of this, it is often difficult to make a diagnosis.
Patients are prescribed a serological blood test for antibodies to a nuclear ribonucleoprotein. If the indicators of this study exceed the permissible and at the same time, patients have arthralgia and Raynaud's syndrome, then the diagnosis is considered confirmed.
Additionally, the following studies are prescribed:
- clinical and biochemical blood and urine tests;
- urine research according to Nechiporenko;
- analysis for rheumatoid factor and specific immunoglobulins.
If necessary, an ultrasound of the kidneys is prescribed, as well as an X-ray of the lungs and an echocardiogram.
Treatment methods
The treatment of mixed connective tissue disease is aimed primarily at suppressing the autoimmune reaction. The following medications are prescribed to patients:
- Corticosteroid hormones: Dexamethasone, Metipred, Prednisolone. These drugs reduce the autoimmune response and inflammation in the joints.
- Cytostatics: Azathioprine, Imuran, Plaquenil. Takea medicines also suppress the immune system.
- Nonsteroidal anti-inflammatory drugs: "Diclofenac", "Voltaren". They are prescribed for severe pain and swelling of the joints.
- Calcium antagonists: Verapamil, Diltiazem, Nifedipine. These drugs are prescribed to prevent damage to the cardiovascular system.
- Proton Pump Inhibitors: Omeprazole. Patients with Sharpe's syndrome have to take medication for a long time, and sometimes for life. This can adversely affect the organs of the digestive tract. The drug "Omeprazole" helps protect the gastric mucosa from the aggressive effects of drugs.
Such complex treatment prevents exacerbations of the disease and allows for stable remission.
It is important to remember that drugs for the treatment of TSZST significantly reduce immunity. Therefore, patients need to protect themselves from contact with infectious patients and hypothermia.
Also, patients are not recommended to be in direct sunlight for too long. Exposure to ultraviolet radiation can provoke an exacerbation of the disease.
Forecast
Does Sharpe's syndrome affect life expectancy? The prognosis of this disease is considered conditionally favorable. Dangerous lesions of internal organs with SZST develop less often than with other autoimmune pathologies. A fatal outcome is noted only with advanced forms of the disease and the presence of complications from the heart and kidneys.
However, it should be remembered that this disease is chronic in nature and is not completely cured. Often patients are shown lifelong medication. If the patient adheres to the recommended treatment regimen, then the prognosis of the disease is favorable. Timely therapy helps maintain a normal quality of life for the patient.
Prevention
Specific prevention of this disease has not been developed, since the exact causes of autoimmune pathologies have not been established. Rheumatologists are advised to adhere to the following recommendations:
- Uncontrolled medication should be avoided. A long course of drug treatment can only be carried out under the supervision of a doctor.
- With a hereditary predisposition to autoimmune pathologies, it is necessary to avoid excessive exposure to sunlight and regularly undergo a routine examination by a rheumatologist.
- It is very important to avoid stress whenever possible. Emotionally labile people need to take sedatives and visit a psychotherapist.
- If there is pain in the joints of the limbs and spasms of the peripheral vessels, you must consult a doctor and undergo an examination.
These measures will help reduce the likelihood of autoimmune rheumatic pathologies.