Sharp's syndrome is an autoimmune disease characterized by a high titer of autoantibodies to ribonucleoprotein. The syndrome was first identified as an independent disease and described by medical scientists quite recently, in 1972 the first mention of the disease occurs in adults, in 1986 - in children. At the same time, treatment methods were developed, the causes, symptoms and diagnosis of Sharpe syndrome were systematized, and a positive outcome of the disease was predicted with timely comprehensive treatment.
Brief description of the condition
This ailment has a different name that fully characterizes its essence - a mixed, or undifferentiated, disease of connective tissue (SSTS). The reason for this name lies in its symptoms, characteristic of a number of other diseases, among which:
- Systemic lupus erythematosus is a condition in which the human body ceases to “recognize” its own cells and begins to fight them as alien cells.
- Systemic scleroderma. In this disease, the connective tissue also suffers, as a result of which the cardiovascular system, other internal organs, the musculoskeletal system and the skin are affected.
- Dermatomyositis is a pathology of the connective tissue, muscle system and human skin.
- Rheumatoid arthritis is a systemic disease accompanied by damage to the joints, mainly small ones.
Faced with a similar diagnosis, the patient wonders, what are the causes, symptoms of Sharpe syndrome, what is it? To date, the disease has been little studied by domestic doctors, most of the information about it was obtained by doctors from foreign studies. In the 10th revision of the ICD, Sharpe's syndrome is assigned to the subclass “Systemic Connective Tissue Damage” (M35).
Key scientific facts known about the disease:
- the disease is most prevalent among women;
- in most cases affects people from 5 to 80 years old, the average age of the diseased is 37 years;
- a hereditary predisposition to pathology is allowed.
In order not to miss the onset of the disease, or maybe prevent it, it is important to understand what are the causes of the disease, Sharpe syndrome, symptoms of the disease, prevention.
Causes of occurrence
The etiology of the disease in question is not fully understood. There is an opinion that the main cause of the syndrome is heredity. A genetic predisposition develops into a progressive disease under the influence of negative factors, which include the following:
- frequent infection with viral diseases;
- exposure to stress;
- regular severe hypothermia;
- hormonal surges.
It is the above circumstances that become the impetus for the increased production of autoantibodies to nuclear ribonucleoproteins.
First signs of the disease
Early diagnosis and timely treatment of Sharpe's syndrome can allow us to talk about a favorable prognosis of the disease. In order to see a doctor in time, you must be able to independently recognize the first signs of an ailment.
These include:
- Raynaud's syndrome - a disease characterized by the pathology of small arteries and arterioles of the upper extremities;
- joint and muscle pain;
- swelling of the hands;
- manifestations of arthritis;
- low-grade body temperature (37.1–37.9 ° C);
- general weakness.
If you ignore the first symptoms of the disease, the clinical picture begins to expand, and the initial symptoms progress.
Symptoms of SZST
Most often, patients who are diagnosed with a mixed connective tissue disease encounter the following disorders:
- Problems with the musculoskeletal system. Arthralgia, arthritis, degenerative processes in the muscles, osteoporosis, Raynaud's syndrome with subsequent gangrene of the upper extremities - this is not a complete list of pathologies that comprehend the bone and muscle systems of a person with Sharpe syndrome.
- Skin changes. Swelling of the hands, especially of the hands and fingers, rashes, including on the face, hyper- or hypopigmentation, alopecia are the most common manifestations of SZST on the skin.
- Damage to the kidneys. This pathology is observed only in 1 out of 10 cases of the disease and has a favorable prognosis.
- Pulmonary pathology. The characteristic manifestations of the syndrome in the bronchopulmonary system are reduced respiratory volume of the lungs and a decrease in their diffusion capacity.
- Disorders in the cardiovascular system. Exudative pericarditis (inflammation of the pericardium), myocarditis (inflammation of the heart muscle), problems in the aortic valve, myocardial necrosis and other disorders of the heart are common signs of the disease.
- Digestive upset. There are problems with the esophagus, less often - an increase in the liver and spleen.
- Lymphadenopathy is a condition characterized by swollen lymph nodes.
- Trigeminal neuralgia (not more than 10% of cases).
- Sjögren’s syndrome (an autoimmune disease that affects connective tissues and endocrine glands) and Hashimoto's thyroiditis (an autoimmune disease characterized by an inflammatory process in the thyroid gland). These manifestations are extremely rare, therefore, they can not be relied upon in the diagnosis of the syndrome.
Clinical diagnosis
A varied combination of symptoms and several variations in laboratory test results often make it difficult to diagnose Sharp's syndrome.
Methods used to make the diagnosis:
- Examination of the patient. Based only on the external manifestations of the disease (swelling of the hands, Raynaud's syndrome, muscle weakness, enlarged lymph nodes, skin rash, etc.), there is a high probability of making an incorrect diagnosis, since all these symptoms are also characteristic of other systemic autoimmune diseases.
- Laboratory blood test. A study is being conducted to detect antibodies to ribonucleoprotein. And also in patients there is severe anemia (a decrease in hemoglobin level), leukopenia (a decrease in the number of white blood cells) and an increased ESR.
The diagnosis of SSTF can be confirmed provided that there are at least three patient complaints of characteristic symptoms (pain, weakness, etc.) and the detection of autoantibodies.
Disease treatment
The choice of a specific treatment method for an undifferentiated connective tissue disease depends on the severity of the damage to all organs and systems of the body.
The following methods bring good results:
- Hormone therapy. Clinical studies and practical application confirm the positive effect of corticosteroids on the body. In advanced cases, high doses of Prednisolone are prescribed, which can be significantly reduced over time.
- Prescribing immunosuppressants. Indication for such therapy is the lack of effectiveness of steroids.
- In acute articular conditions, such as severe pain and limited mobility, non-steroidal anti-inflammatory drugs are used.
In his research, G. Sharp noted that the administration of corticosteroids can quickly return the patient to normal life with complete drug withdrawal or minor supportive therapy mainly during periods of exacerbation.
In the case of significant damage to the internal organs, the selection of a treatment technique may require consultation of related specialists:
- cardiologist;
- gastroenterologist;
- rheumatologist;
- neurologist.
The above specialists can make their own adjustments to the prescription of drugs. So, depending on the symptoms, cytostatics (antitumor agents) and vasodilators (medications that cause vasodilation and improve blood circulation) can be prescribed.
Possible complications
With the timely identification of causes, symptoms, diagnosis of Sharpe syndrome, as well as with adequate treatment, pathological processes in the body are successfully stopped, allowing the patient to return to a full life. In the case of severe damage to internal organs and systems, it is customary to talk about the aggravated course of the disease.
Possible complications of the disease include:
- stroke;
- myocardial infarction;
- esophagitis (esophageal disease);
- esophageal stricture (narrowing of its lumen);
- renal failure;
- perforation of the colon;
- pulmonary pathology;
- cerebral hemorrhages;
- infections.
Forecast
A favorable prognosis of the disease is due to a relatively mild course (in comparison with other systemic autoimmune diseases). All characteristic conditions respond well to treatment mainly with corticosteroids and, with timely initiation of therapy, rarely lead to serious complications.
According to statistics, mortality from Sharpe syndrome occurs in no more than 7% of cases.
The causes of death in such cases are complications of the syndrome, namely:
- myocardial infarction;
- renal failure;
- damage to the lungs and central nervous system;
- joined infection;
- cerebral hemorrhage.
Prevention methods
Due to the fact that the main reason for the appearance of this disease is considered to be a genetic predisposition, which a person cannot influence, there are no clinical recommendations for Sharpe syndrome to prevent it. But in order not to provoke the onset of the disease, it is important to monitor the state of your nervous and hormonal systems, to protect yourself from hypothermia and frequent colds.