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. Also, the disease affects some internal organs and the musculoskeletal system.
The basis of this kind of inflammation is a common cascading disturbance of blood circulation, the 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.
Scleroderma Classification
In medicine, several types of scleroderma are distinguished, 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 hands, feet, face and body. Characteristic lesions of this form progress within a year, and after the first visible lesions the disease affects almost all parts of the body. Simultaneously with the fact that this form affects almost the entire skin in patients, Raynaud's syndrome is also noted - it 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 cautiously call this ailment, is true scleroderma, and all because it is characterized by isolated Raynaud's syndrome and the presence of autoantibodies in the blood.
- The limited form is a typical autoimmune disease, which is expressed by Raynaud's syndrome, only after a long period of time minor lesions of the skin appear, most often on the feet, hands or face. A little later, the disease affects the internal organs.
- Visceral systemic sclerosis is characterized in that it affects exclusively internal organs.
Separately considered juvenile form, which develops mainly in childhood.
By the nature of the course, scleroderma is:
According to the development activity, there are three stages of the disease:
An accurate diagnosis and treatment can only be made by a specialist after a series of studies.
The causes of the development of the disease
To date, the exact causes of the disease have not been elucidated. It is believed that 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 the development of systemic sclerosis (ICD 10 code M34), the disease can be triggered by such factors:
- infectious diseases;
- hormonal disorders;
- hypothermia, especially with frostbite of the limbs;
- molecular mimicry of microorganisms, which provokes a high activity of lymphocytes;
- intoxication with chemicals and drugs;
- living in an environmentally disadvantaged region;
- work in a chemical enterprise.
Human health 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. Such substances can have a harmful effect on the body:
- silica;
- White Spirit;
- welding gases;
- solvents;
- ketones;
- trichlorethylene.
But not all people who often come in contact with these substances develop scleroderma. But if there is a hereditary predisposition, then the risk of developing the disease in people working with chemicals is high enough.
Symptomatology
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 tissue with high strength and elasticity. During increased function, fibroblasts begin to intensively produce 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 is created for normal blood flow, 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 development of the disease. The first sign of the development of the disease is high body temperature and sudden weight loss. The mortality rate of patients with this form is high.
The chronic classification of systemic sclerosis is manifested in the form of Raynaud's syndrome, which affects the joints and skin. Symptoms may be hidden for several years.
The most characteristic sign of the disease is damage to 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:
- muscle and joint pain;
- stiffness and limited movement, especially in the morning;
- finger deformation;
- nail extension;
- the accumulation of calcium in the fingers is expressed as whitish spots around the joints.
If scleroderma affects the lungs, then this can manifest itself in the form of:
- interstitial fibrosis;
- pulmonary hypertension;
- inflammatory process in the pleura.
With heart damage, symptoms of systemic sclerosis, the treatment of which requires an integrated approach, are manifested as follows:
- noticeable compaction of the heart muscle;
- pericarditis or endocarditis;
- discomfort in the heart;
- heart palpitations;
- shortness of breath is observed;
- the work of the heart is disturbed.
Kidney damage is characterized by the following symptoms:
- high blood pressure;
- decrease in urine excreted per day, in some patients its complete absence is observed;
- renal failure is rapidly increasing;
- urine protein levels increase;
- impaired vision;
- periodically lost consciousness.
With damage to the intestines and stomach, the disease is expressed by the following symptoms:
- the swallowing process is disturbed;
- belching and heartburn;
- ulcers on the walls of the esophagus;
- intestinal motility decreases;
- diarrhea or constipation;
- discomfort in the stomach.
With lesions of the central nervous system, pain in the limbs is observed, the sensitivity of the skin in the arms and legs is impaired.
In addition, progressive systemic sclerosis can affect the endocrine system, which causes malfunctions in 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. Gathers an anamnesis and conducts an examination 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 dysfunctions when they are affected.
Also, people with scleroderma need to undergo instrumental diagnostic methods:
- X-ray of bones and internal organs;
- ECG and echocardiography;
- CT and MRI;
- Ultrasound of the heart and other organs;
- biopsy of the skin and tissues of internal organs.
In addition, consultation with specialized specialists will be required.
Therapeutic measures
Until now, the causes of systemic sclerosis have not been exactly clarified, but if individual signs are treated, then you can not only improve the condition of the body, but also the work of organs and systems. As a result, it is possible to extend the life of the patient and improve its quality.
The treatment of this disease is based on the elimination and mitigation of symptoms. Drug treatment is divided into three groups:
- antifibrotic drugs: "Colchicine", "Diutsifon";
- vascular, which dilate blood vessels and prevent the formation of blood clots, such as "Nifedipine", "Trental";
- anti-inflammatory drugs and drugs that suppress some of the body's immune responses.
In addition, to relieve the symptoms of systemic sclerosis, the following drugs are used:
- aminoquinolines: Delagil, Plaquenil;
- lowering blood pressure: Captopril, Capoten.
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 internal organs.
Immunosuppressants do not allow the development of complications dangerous to human life. Most often, experts recommend taking Azathioprine or Chlorambucil.
Glucocorticosteroids are used only for exacerbation of the chronic form of scleroderma.
To improve the walls of blood vessels and skin integument, “Lidase” is administered.
You can eliminate cramps with Raynaud's syndrome using the following means: Prazosin, Nifedipine, and Reserpine.
Preparations for eliminating the 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, due to which it is possible to improve joint mobility.
To prevent the development of inflammation, as well as relieve pain, non-hormonal anti-inflammatory drugs are used, such as Prednisolone, but not more than 10 mg per day.
A massage course is prescribed with the use of oils, which allows you to maintain mobility of the limbs and skin elasticity.
To accelerate the healing of finger ulcers, it is recommended to use occlusive dressings, rinse ulcers, and remove dead tissue using enzymes. The skin is lubricated with nitroglycerin ointment, and for the expansion of blood vessels use Reserpine or Octadine.
If the ulcers are infected, then antibacterial agents are used, for example, Stellanin.
To prevent drying out of the skin, it is recommended to use mild detergents without alkali in which oils are present.
Vitamin B10 and E, as well as Penicillamine or Alkoloid Colicin will help improve skin condition.
A detailed description of the treatment of progressive systemic sclerosis, the symptoms of which cause a lot of inconvenience to the patient, will be made by the doctor after he examines and finds out which organs are affected by the disease.
Preventative methods
If there is a suspicion that a person is developing scleroderma, then he simply needs to seek qualified help and undergo a full examination from all specialists. Primary preventive methods in people who are at risk include:
- regular examinations by a specialist, dispensary observation, especially for children during puberty;
- conducting the necessary laboratory tests, urine and blood are mandatory;
- a patient must be examined by a cardiologist once a year, with an electrocardiogram, a neurologist and a psychotherapist;
- the patient is advised to undergo regular x-rays and ultrasound examinations of internal organs;
- a person must choose for himself a job that does not provoke the development and progression of systemic sclerosis, no injuries, hypothermia, overstrain;
- the patient with scleroderma must eat rationally, completely stop smoking and drinking alcohol, eliminate negative factors that destroy the structure of the walls of blood vessels;
- completely avoid hypothermia, overwork and stress.
If you do not receive treatment for systemic sclerosis in time, then 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 the small vessels, causing the growth of fibrous tissue in them. And when the vessels are affected, this leads to disruptions in the functioning of all organs. The main threat to the patient is considered to be a severe blood flow disorder, which nourishes the tissues and cells of the internal organs with oxygen.
Complications most often develop in those patients for whom the diagnosis of systemic sclerosis is unspecified or identified late. Among the complications are more often detected:
- tissue necrosis;
- pulmonary fibrosis;
- pulmonary hypertension;
- proteinuria;
- heart failure and arrhythmia;
- gangrene of arms and legs.
If you do not follow the recommendations for the treatment of scleroderma, then 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 the life expectancy of patients is reduced.
Forecasts
The most favorable prognosis is in the juvenile form, which manifests itself mainly in children. Subject to treatment recommendations, almost all symptoms subside and do not appear for many years.
The chronic form of systemic sclerosis, the photo in the article indicate vivid manifestations of the disease, in adolescents proceeds benignly, without causing any inconvenience to the young patient. Patients with a chronic form survive more than 84%, and with a subacute only 62%.
But in people with scleroderma, which provokes abnormalities in the chromosomes, the prognosis is poor.
To slow down the progression of the disease today only "D-penicillamine" helps. This tool significantly improves the prognosis. Over the past 6 years, 9 out of 10 patients who took this drug and precisely followed the recommendations survived. But without proper treatment, out of 10 patients, 5 die.
An important factor for the survival and recovery of patients with scleroderma is the rational employment of:
- in acute and severe forms of the patient are transferred to disability;
- in the chronic form of the patient, they are completely exempted 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 a normal working capacity and an active lifestyle.
Conclusion
Scleroderma is a serious disease that requires early diagnosis and comprehensive treatment with the use of drugs, as well as additional methods of therapy. The reasons for the development of the disease have not yet been clarified, so it is difficult to correctly diagnose and prevent development.
That is why, at the first alarming symptoms, it is better to seek the advice of a doctor, especially patients with a predisposition to this ailment. The forecast is favorable only for those who in time will seek help and will follow all the recommendations.