Progressive systemic sclerosis, or scleroderma, is one of the autoimmune inflammatory diseases that affects the connective tissue. It is characterized by a staged course and a huge list of clinical manifestations, which are mainly associated with damage to the skin. The disease also affects some internal organs and the musculoskeletal system.
This kind of inflammation is based on a widespread cascade of circulatory disorders, an inflammatory process and generalized fibrosis. The life expectancy of a patient with systemic sclerosis depends on the nature of the course of the disease, the stage and extent of damage to internal organs and systems.
Classification of scleroderma
In medicine, there are several types of scleroderma, each of them is characterized by its own signs and characteristics of the course:
- Diffuse differs in that it mainly affects the skin of the arms, legs, face and torso. The characteristic lesions of this form progress during the year, and after the firstvisible lesions of the disease affects almost all parts of the body. At the same time that this form affects almost the entire skin, patients also have Raynaud's syndrome - this is a vascular disease that makes them especially sensitive to cold or heat. This form is characterized by rapid damage to almost all internal organs.
- Cross systemic sclerosis combines the symptoms of not only scleroderma, but also other rheumatological ailments.
- Prescleroderma, or as many doctors call this disease with caution, is true scleroderma, and all because it is characterized by isolated Raynaud's syndrome and the presence of autoantibodies in the blood.
- Limited form is a typical autoimmune disease that is expressed by Raynaud's syndrome, only after a long period of time minor skin lesions appear, most often on the feet, hands or face. A little later, the disease also affects the internal organs.
- Visceral systemic sclerosis is different in that it only affects the internal organs.
The juvenile form, which develops mainly in childhood, is considered separately.
According to the nature of the course, scleroderma happens:
- chronic;
- tune;
- spicy.
According to the activity of development, three stages of the disease are distinguished:
- minimum;
- moderate;
- maximum.
Only a specialist will be able to make an accurate diagnosis and choose a treatment after a series ofresearch.
Causes provoking the development of the disease
To date, the exact causes of the development of the disease have not been clarified. It is believed that the pathology develops due to genetic factors. There is a hereditary predisposition to the development of autoimmune diseases. But this does not mean that immediately after birth the disease will begin to develop. In people prone to developing systemic sclerosis (according to ICD 10 code M34), the disease can be triggered by such factors:
- past infectious diseases;
- hormonal disorders;
- hypothermia, especially with frostbite of the limbs;
- molecular mimicry of microorganisms, which provokes high activity of lymphocytes;
- intoxication with chemicals and drugs;
- living in an ecologically unfavorable region;
- working at a chemical plant.
Human he alth and the environment are interconnected. That is why, most often, systemic sclerosis (according to ICD 10 code M34) provokes an unfavorable atmosphere in which a person lives. This is especially true of the form that develops in early childhood. The following substances can have a harmful effect on the body:
- silica;
- white spirit;
- welding gases;
- solvents;
- ketones;
- trichlorethylene.
But not all people who frequently come into contact with these substances develop scleroderma. But if there ishereditary predisposition, the risk of developing the disease in people working with chemicals is quite high.
Symptomatics
The main sign of the development of the disease (systemic sclerosis) is an increase in the function of fibroblasts. It is these connective tissue cells responsible for the synthesis of collagen and elastin that provide the tissue with high strength and elasticity. During the increase in function, fibroblasts begin to produce more collagen. As a result, foci of sclerosis appear in organs and tissues. Also, such changes affect the walls of blood vessels, which thicken. As a result, an obstacle to normal blood flow is created, as a result of which blood clots form and ischemic processes develop.
Connective tissue is present in all organs and systems, which is why the disease spreads throughout the body and has a variety of symptoms.
In the acute form, sclerotic changes in the skin and fibrosis of the internal organs develop during the first years after the onset of the disease. The first sign of the development of the disease is high body temperature and sudden weight loss. The mortality of patients with this form is high.
Chronic classification of systemic sclerosis manifests itself in the form of Raynaud's syndrome, which affects the joints and skin. Symptoms may be hidden for several years.
The most characteristic symptom of the disease is the defeat of the skin on the hands and face. You can also determine the development of the disease by vascular disorders and joint damage.
If the disease affects the musculoskeletal system, then the following symptoms are observed:
- pain in muscles and joints;
- stiffness and limited movement, especially in the morning;
- deformed fingers;
- nail extension;
- Accumulation of calcium in the fingers, expressed as whitish spots around the joints.
If scleroderma affects the lungs, it may manifest as:
- interstitial fibrosis;
- pulmonary hypertension;
- inflammatory process in the pleura.
When the heart is affected, the symptoms of systemic sclerosis, the treatment of which requires an integrated approach, appear as follows:
- appreciably thickening of the heart muscle;
- pericarditis or endocarditis;
- heart discomfort;
- heartbeat quickens;
- shortness of breath;
- heart failure.
Kidney damage is characterized by the following symptoms:
- high blood pressure;
- decreased urine output per day, in some patients there is a complete absence of it;
- rapidly increasing kidney failure;
- urine protein levels increase;
- visual impairment;
- passing out intermittently.
When the intestines and stomach are affected, the disease is expressed by the following symptoms:
- swallowing is disturbed;
- burping and heartburn;
- ulcers on the walls of the esophagus;
- peristalsis decreasesintestines;
- diarrhea or constipation;
- discomfort in the stomach.
When the central nervous system is affected, pain in the limbs is observed, the sensitivity of the skin in the arms and legs is disturbed.
In addition, progressive systemic sclerosis can affect the endocrine system, causing malfunction of the thyroid gland.
Diagnostic methods
To make an accurate diagnosis, you will have to undergo a comprehensive examination, since the disease can affect all internal organs and systems. Initially, the patient is examined by a rheumatologist who will study the medical history of the patient himself and his immediate family. Collects an anamnesis and conducts an examination that allows you to assess the condition of the skin and joints. An examination is also carried out using a phonendoscope, which allows you to determine the stage of progression of the disease.
The patient is recommended to undergo laboratory tests, but they are uninformative and only help to identify organ dysfunction when they are damaged.
Also, people with scleroderma need to undergo instrumental diagnostic methods:
- x-ray of bones and internal organs;
- ECG and EchoCG;
- CT and MRI;
- Ultrasound of the heart and other organs;
- biopsy of skin and tissues of internal organs.
In addition, consultation of narrow specialists will be required.
Therapeutic interventions
So far, the causes of systemic sclerosis have not been precisely clarified, but if individualsigns, it is possible not only to improve the condition of the body, but also the functioning of organs and systems. As a result, it is possible to extend the life of the patient and improve its quality.
Treatment of this disease is based on the elimination and mitigation of symptoms. Drug treatment is divided into three groups:
- anti-fibrotic drugs: Colchicine, Diucifon;
- vascular, which dilate blood vessels and prevent blood clots, these include Nifedipine, Trental;
- anti-inflammatory drugs and medicines that suppress some of the body's immune responses.
In addition, the following drugs are used to relieve the symptoms of systemic sclerosis:
- aminoquinolines: Delagil, Plaquenil;
- lowering blood pressure: Captopril, Kapoten.
For the treatment of scleroderma, the main drug is "D-penicillamine", which allows you to stop the development of the disease in an aggressive course. This tool allows you to reduce the thickening of the skin, the symptoms of Raynaud's syndrome, does not allow the development of severe forms of diseases of the internal organs.
Immunosuppressants prevent the development of life-threatening complications. Most often, experts recommend taking Azathioprine or Chlorambucil.
Glucocorticosteroids are used only for exacerbation of chronic scleroderma.
To improve the walls of blood vessels and skin, "Lidase" is injected.
You can eliminate spasms in Raynaud's syndrome with the help of such means: Prazosin, Nifedipine and Reserpine.
Drugs forelimination of symptoms of systemic sclerosis are selected by a specialist individually for each patient, depending on the course and stage of the disease.
Additional treatments
Be sure to prescribe physiotherapy for scleroderma, thanks to which it is possible to improve joint mobility.
To prevent the development of inflammation, as well as relieve pain, use non-hormonal anti-inflammatory drugs, such as Prednisolone, but not more than 10 mg per day.
A course of massage using oils is prescribed, which allows you to maintain the mobility of the limbs and the elasticity of the skin.
To speed up the healing of ulcers on the fingers, it is recommended to use occlusive bandages, wash the ulcers, remove dead tissue using enzymes. The skin is lubricated with nitroglycerin ointment, and "Reserpine" or "Octadin" is used to dilate blood vessels.
If the ulcers are infected, then antibacterial agents are used, for example, Stellanin.
In order to prevent drying of the skin, it is recommended to use mild, alkali-free products containing oils for washing.
Vitamin B10 and E, as well as "Penicillamine" or "Colicin Alkaloid" will help improve skin condition.
A detailed description of the treatment of progressive systemic sclerosis, the symptoms of which give the patient a lot of inconvenience, will be done by the doctor after he conducts an examination and finds out which organs are affected by the disease.
Preventive methods
If there is a suspicionto the fact that a person develops scleroderma, then he just needs to seek qualified help and undergo a full examination by all specialists. Primary preventive methods for individuals who are at risk include:
- regular examinations by a specialist, dispensary observation, especially for children during puberty;
- carrying out the necessary laboratory tests, mandatory urine and blood donation;
- necessarily the patient must be examined by a cardiologist once a year, while an electrocardiogram is done, a neurologist and a psychotherapist;
- the patient is recommended to undergo regular X-rays and ultrasound examination of internal organs;
- a person should choose for himself a job that will not provoke the development and progression of systemic sclerosis, no injuries, hypothermia, overexertion;
- necessarily a patient with scleroderma should eat rationally, completely stop smoking and drinking alcohol, exclude negative factors that destroy the structure of the walls of blood vessels;
- completely avoid hypothermia, overwork and stress.
If systemic sclerosis is not treated in time, the disease can provoke serious and irreversible consequences.
Complications
Scleroderma is a serious disease that, without proper treatment, can provoke a lot of complications in the body. The disease causes inflammation of small vessels, provoking themgrowth of fibrous tissue. And when the vessels are affected, this leads to disruption of the work of all organs. The main threat to the patient is considered to be a severe disorder of blood flow, which nourishes the tissues and cells of internal organs with oxygen.
Complications most often develop in those patients in whom the diagnosis of systemic sclerosis is unspecified or diagnosed late. Among the complications more often detected:
- tissue necrosis;
- pulmonary fibrosis;
- pulmonary hypertension;
- proteinuria;
- heart failure and arrhythmias;
- gangrene of hands and feet.
If you do not follow the recommendations for the treatment of scleroderma, the disease actively destroys the walls of blood vessels, tissues of internal organs, joints and internal systems of the body, which lead to the fact that life expectancy of patients is reduced.
Forecasts
The most favorable prognosis is in the juvenile form, which manifests itself mainly in children. When treatment recommendations are followed, almost all symptoms subside and do not appear for many years.
Chronic form of systemic sclerosis, the photos in the article indicate vivid manifestations of the disease, in adolescents it proceeds benignly, without causing any inconvenience to the young patient. Patients with the chronic form survive more than 84%, while those with the subacute form only 62%.
But people with scleroderma, which causes abnormal chromosomes, have a poor prognosis.
To slow down the progression of the disease today, only "D-penicillamine" helps. This tool significantly improves the prognosis. For 6In recent years, 9 out of 10 patients survived who took this drug and followed the recommendations exactly. But without proper treatment, out of 10 patients, 5 die.
An important factor for the survival and recovery of patients with scleroderma is rational employment:
- in acute and severe forms, the patient is transferred to disability;
- in the chronic form, the patient is completely freed from hard work, it is also important to exclude hypothermia and contact with chemicals.
If you correctly approach the treatment and choose an adequate place of work, then the prognosis for patients with scleroderma is favorable. It enables a person to maintain normal performance and an active lifestyle.
Conclusion
Scleroderma is a serious disease that requires early diagnosis and complex treatment with drugs, as well as additional therapies. The causes of the development of the disease to date have not been clarified, so it is difficult to correctly diagnose and prevent development.
That is why, at the first alarming symptoms, it is better to consult a doctor, especially for patients with a predisposition to this disease. The prognosis is favorable only for those who seek help in time and follow all the recommendations.
