Sharp’s syndrome or mixed collagenosis is what doctors call a disease of the connective tissue with a disturbed immune system. The symptoms of several collagen diseases (connective tissue diseases) characterize Sharp’s syndrome.
What is Sharp Syndrome?
Sharp syndrome or MCTD (“mixed connective tissue disease”) is a rheumatoid disease of the connective tissue. Like all rheumatic phenomena, Sharp’s syndrome is an autoimmune disease. For cavernoma in dictionary, please visit dictionaryforall.com.
Here, MCTD is one of the collagen diseases. This name is derived from “collagen”. The protein is secreted by connective tissue cells and stabilizes the tissue matrix. Mixed collagenosis is another term for Sharp’s syndrome because of the overlapping symptoms of several autoimmune diseases.
This is why the term “overlap syndrome” is sometimes used. The term “Sharp Syndrome” is mainly used in Anglo-American countries and refers to the person who first described the disease: the US doctor andresearcherdr Gordon C. Sharp was the godfather of Sharp Syndrome.
Causes
Sharp syndrome is caused by an autoimmune reaction. The immune system produces antibodies against the tissue of its own organism, which it is actually supposed to protect. The tolerance towards one’s own body learned in the course of maturation is eliminated.
The reasons for this are unknown and an important issue in basic medical research. According to a working hypothesis, genetic factors should play the main role in the development of MCTD. According to another opinion, side effects of medication could also be among the triggers of mixed collagenosis. Psychological factors or hormonal disorders may also be involved in the overall process.
In the case of collagenosis, the immune system can attack almost all organs, because connective tissue can be found in many places in the organism. These diverse symptoms are particularly pronounced in Sharp’s syndrome.
Symptoms, Ailments & Signs
Sharp Syndrome is a disease of the connective tissue. A wide variety of symptoms can occur. Therefore, “mixed” symptoms are also spoken of. Sharp syndrome often manifests itself in the small finger joints. In this context, a positive rheumatoid factor can be detected in the blood. There is a risk that the inflammation will spread to other joints.
Inflammation of the knee joints can also occur. The knee swells and hurts. However, the symptoms do not spread to other joints. Sharp syndrome is not only expressed in the form of pain and impairment of joint mobility. There may also be external signs of the disease.
The skin may turn red or form wheals in certain areas. Skin areas that are exposed to prolonged exposure to the sun are often affected. If vascular inflammation occurs, circulatory disorders occur. Other symptoms can be nerve inflammation or kidney dysfunction.
The occurrence of pneumonia is also typical of Sharp’s syndrome. The disease occurs in episodes. In many cases, the symptoms first appear a few weeks after delivery. Symptoms worsen with prolonged exposure to the sun.
Diagnosis & History
Sharp syndrome usually begins with blood vessel involvement. Circulatory disorders are particularly evident in the hands and feet. These are symptoms typical of collagenosis in Raynaud’s syndrome. The specific antibodies that indicate mixed collagenosis are detected in the blood laboratory. In many cases, the rheumatic factors are also increased.
Hardening of the connective tissue of the skin occurs as the disease progresses, and finally polyarthritis can develop with painful changes in the joints, mostly on the fingers. In addition, the patient sometimes has inflammatory symptoms in the muscles (polymyositis). A pathological proliferation of the connective tissue ( fibrosis ) occurs primarily in the lungs, which can be seen in the X-ray image.
Lung involvement is secondary to pulmonary hypertension ( see Hypertension Due to a Constricted Lung). Furthermore, inflammation of the pericardium and renal dysfunction characterize Sharp’s syndrome as possible complications.
Complications
Various complications and long-term effects can occur in the course of Sharp syndrome. If the disease remains untreated, hardening of the skin connective tissue and finally polyarthritis with painful joint changes and vascular diseases occur. In addition, the patient is occasionally burdened by inflammatory phenomena in the muscles.
It can also lead to a pathological proliferation of connective tissue. A typical complication is the development of eczema. These can become inflamed as the disease progresses and, in the worst case, scar. Possible long-term consequences of Sharp’s syndrome are arthritis and myositis. If the course is severe, internal organs are also attacked – kidney, digestive tract and lung disorders occur.
The heart or nervous system is also rarely affected. Treating the autoimmune disease also carries risks. When using antirheumatic drugs, asthma attacks can occur in addition to the typical side effects and interactions. Immunosuppressive drugs can cause high blood pressure, weight gain, and serious conditions such as diabetes and gastrointestinal problems. With a weakened immune system, the preparations can promote tuberculosis, pneumonia or encephalitis. Allergic reactions to the agents and materials used cannot be ruled out either.
When should you go to the doctor?
Sharp syndrome should always be treated by a doctor. Further complications and symptoms can only be avoided by proper medical treatment, so treatment by a doctor is essential for this disease. The earlier a doctor is consulted, the better the further course of this disease is usually. Inflammation in the joints is the primary indication of Sharp’s syndrome. If these occur permanently and do not go away on their own, a doctor must be consulted in any case.
Self-healing cannot occur in this case. Furthermore, circulatory disorders can indicate this syndrome and must be examined by a doctor. Many of those affected also suffer from inflammation of the lungs or nerve inflammation. Relapsing symptoms can also indicate Sharp’s syndrome. The syndrome can be recognized by a general practitioner. Further treatment is then carried out by a specialist. It cannot be universally predicted whether this will lead to a complete treatment of Sharp’s syndrome.
Treatment & Therapy
Sharp syndrome requires drug therapy. First of all, the non-steroidal anti- inflammatory drugs (NSAIDs) come into question here. These are pharmaceuticals that do not belong to the cortisone-like anti- inflammatory drugs.
NSAIDs include well-known preparations such as aspirin and ibuprofen. If violent flare-ups occur in the course of mixed collagenosis, the doctor will also prescribe glucocorticoids. These steroid drugs are chemically comparable to cortisone and, like this, reduce inflammatory reactions. The effect is that an endogenous hormonal process is imitated.
If internal organs are affected by MCTD, immunosuppressants are also used. These drugs reduce the performance of the immune system and can be used in parallel with medication with glucocorticoids. However, doctors avoid over-treatment, since cortisone analogues in particular cause serious side effects when used continuously.
The sequelae of mixed collagenosis must also be considered. Pulmonary hypertension or renal hypofunction are in need of treatment. Due to the discussed psychosomatic aspects of mixed collagenosis, holistic approaches are of great importance in specialist clinics. What is then ultimately required is a lifestyle change on the part of the patient with Sharp’s syndrome.
Prevention
Sharp syndrome is a disease with little prophylactic potential. Genetic causes would be difficult to control and the overall mechanism of all autoimmune diseases is poorly understood. However, if stress factors play a decisive role, everyone can make a certain contribution if they lead a balanced life wherever possible. This includes a healthy diet as well as exercise and traditional Chinese medicine (TCM) measures such as Chi Gong or meditation. This is always recommended, not only with regard to Sharp’s syndrome.
Aftercare
Follow-up care for Sharp syndrome includes a physical exam, patient interview, and medication cessation. Since the disease is a chronic condition, follow-up care is often only useful for individual symptoms. Depending on the causative disease, follow-up care can include, for example, an examination of the skin, neurological examinations or imaging procedures.
As a rule, typical symptoms such as rigid facial expressions or a microstomy, in which the mouth can no longer be opened wide, are checked. Once these symptoms are cured, the only way to relieve pain is with medication. If necessary, surgical interventions can also be considered.
Depending on which measures are chosen in detail, aftercare also includes consultations or the prescription of suitable medication. Aftercare for Sharp syndrome is carried out by the responsible specialist. It usually takes place several years after the diagnosis, when the condition has been cured and a purely symptomatic therapy is initiated.
If necessary, the doctor in charge can call in other doctors, such as dermatologists or neurologists. Sharp’s syndrome can take a variety of courses. In some cases, it comes to a standstill for years. Therefore, the doctor must be consulted regularly even after the aftercare. Patients should visit the specialist clinic at least every three months so that the necessary examinations can be carried out.
You can do that yourself
Many people with Sharp Syndrome are under a lot of stress. However, human genetic causes can also trigger the disease. The course of the disease cannot always be traced exactly. In everyday life, the main thing is to relieve acute pain.
It helps to reduce stress with relaxation exercises. Balancing activities also lead to more inner balance. As a result, the painful symptoms occur less frequently. Many people can hardly avoid the stress of everyday life, because work and family demand full commitment. Those affected should then pay attention to a balanced diet and exercise. This increases well-being and stabilizes the cardiovascular system. Meditative exercises such as yoga or chi gong can also be helpful. Even if these self-help measures should not have a direct effect on the disease, they ensure better body awareness and optimize the quality of life.
Long-term physiotherapy and psychological methods of pain management can also be integrated into everyday life. Medicines, which the doctor adapts to the respective symptoms, help against acute pain. Sometimes, however, the gentle exercises of occupational therapy are enough to overcome small attacks of pain. This reduces the consumption of medication.
