Mixed connective tissue disease - causes, symptoms, treatment
- 14 Jun 09:00
- Disease Of The Skin
A mixed connective tissue disease has a second name - Sharpe syndrome. This is a serious systemic disease characterized by the symbiosis of certain signs of polymyositis, scleroderma and red lupus erythematosus. The disease develops predominantly in women.
- 1 Causes of development of
- 2 Clinical picture of
- diagnostic methods 4 Treatment of
- 4.1 Treatment by folk methods
- 5 Forecast and prevention of
- 6 Photo
Causes of development of
The causes of the development of connective tissue disease by this time are unknown. The mechanism of development is the specific impairment of the immune system. As a result of this failure, there is a violation of the production of antibodies and immune complexes, and T-lymphocytes change their immunoregulatory functions. Incidentally, they are the cause of T-cell lymphoma.
In addition, Sharp syndrome is associated with pathological changes in large vessels, leading to signs of Raynaud's disease and pulmonary hypertension.
Clinical picture of
For Sharp disease, a combination of Raynaud's symptoms( tissue damage, swelling, trophic disorder), polyarthritis( inflammation of joints), polymyositis( inflammation of the muscle tissue) is characteristic. In addition, patients experience hypotension and respiratory failure.
Most often, the first sign of connective tissue disease is the manifestation of Raynaud's disease. The disease begins with a feeling of numbness of the fingers and toes, with the development of the disease, severe necrotic changes in the distal phalanges of the fingers are observed. Patients often have a dense swelling of their hands, but the transition to sclerodactyly( thickening of the skin and atrophy of the skin of fingers) is not observed.
When mixed, most patients have polyarthritis of the recurrent course. Approximately 30% of patients are diagnosed with erosive changes in joints and the appearance of rheumatoid nodules.
Symptoms of polymyositis are manifested in 70% of patients, they have myalgia, weakness and seizures of certain muscle groups.
The defeat of the internal organs with connective tissue disease is characterized by the development of pulmonary hypertension, hypotension of the esophagus, which is difficult to swallow and unpleasant sensations in the sternum. Less commonly in patients with Sharp syndrome, diffuse glomerulonephritis develops. In a mixed connective tissue disease, the nervous system is rarely affected, however, cases of aseptic meningitis in patients with severe course and other complications are noted.
Diagnosis of conjunctival mixed disease is based on the evaluation of clinical symptoms and analyzes.
In patients with Sharpe syndrome there is a development of leukopenia, anemia, cryoglobulinemia. These signs, as well as high titres of antibodies to RNP, are diagnostic signs of the disease.
If a patient with Sharpe's syndrome is accompanied by a kidney disease, hematuria and proteinuria are detected in the urine test.
A physician should suspect Sharp syndrome if a patient with symptoms of discoid red lupus erythematosus, polymyositis, scleroderma, rheumatoid arthritis, vasculitis, "cross" manifestations of these diseases are found. In case of suspected Sharpe disease, the patient is referred to a thorough examination of the esophagus, lungs and skeletal muscles. Such research allows to detect changes in these organs at the earliest stages of the disease, when clinical manifestations are not yet present.
The basis for treatment of conjunctival mixed disease is hormonal therapy. Patients are given glucocorticosteroids, and the dose of hormones is selected individually.
As a rule, higher doses are prescribed for acute course and high activity of the disease. On average, in terms of prednisone, the daily dose is 1-1.5 mg per kilogram of body weight of the patient.
At the first stage of Sharp treatment, it is important to choose a dose that can reduce the activity of the pathological process. If during the first two days of treatment no improvement in the patient's condition is observed, it is necessary to increase the dose by 20%.
Usually, treating with high doses of hormones for 1-1.5 months. During this time it is possible to achieve a stable clinical effect. Then begin gradually( once a week) to lower the dose of hormones. Further treatment of Sharpe's disease is the administration of a maintenance dose of hormonal drugs for several years.
If treatment with steroids does not work, cytostatic immunosuppressants - azathioprine, cyclophosphamide or methotrexate - are used to treat connective tissue disease. The course of basic treatment - up to 2 months, then continue taking the drug in a maintenance dose.
Since treatment with large doses of steroids and cytostatics often complicates, it is necessary that the patient is constantly under medical supervision. This will allow you to adjust the dose in a timely manner and pick up symptomatic treatment.
Used for the treatment of Sharp disease and physiotherapy - plasmapheresis. It is necessary to conduct 3-6 procedures at short intervals.
Treatment by folk methods
Mixed connective tissue disease is a serious disease, therefore it is strongly recommended that self-medication be avoided. It is necessary to adhere to the treatment scheme prescribed by the doctor, however, if the doctor considers it possible to supplement as well as folk remedies.
Forecast and prevention of
To date, precise reasons for the development of connective tissue mixed disease have not been established. Therefore, it is not possible to develop preventive measures to prevent Sharpe syndrome.
The prognosis for this disease is uncertain. In general, connective tissue mixed disease proceeds more benignly than all those diseases whose symptoms it duplicates. The outcome of the disease largely depends on the degree of involvement of internal organs. The most severe course of the disease is observed with progressive pulmonary hypertension, damage to the kidneys and nervous system. In patients with minimal damage to visceral organs, perennial remissions are often observed in the context of receiving maintenance doses of corticosteroids or even without medication.