In this real word of autoimmune diseases, Scleroderma is one of the complex and often misinterpret disease. It is a persistent condition that impacts the connective tissues within the body, resulting in the skin and other organs becoming thicker and harder. It is necessary for those affected by this serious illness, along with their caregivers and healthcare providers, to have a proper understanding about the illness. Looking further into the real-world of this illness inculcates examining and determining its symptoms, treatment options and causes. It is very essential for the patients to have deep understanding about the treatment options available.
What exactly is Scleroderma?
Systematic Sclerosis or commonly known as Scleroderma is an uncommon autoimmune disease that occurs due to the excessive production of collagen within the body of an individual. The extra collagen present in the body causes the skin, blood vessels, and internal organs to thicken and they become hard with time. The term "scleroderma" is originated from the Greek words "sclero" (that means hard) and "derma" (means skin), which describes the main symptom of this illness accurately.
Types of Scleroderma
There are two primary forms of Scleroderma which are known worldwide: localized and systemic. Systemic Scleroderma not only effects the skin but also has major impact on the internal organs like heart, kidney, digestive system and lungs of a person. Whereas, Localized Scleroderma majorly targets the underneath tissues and skin which results in thick and visible discoloured patches on the body. There are many other types of scleroderma which are seen rarely. Let’s read about them in detail-
1. Localized Scleroderma
Morphea or Localized Scleroderma (common name) that predominantly affects the skin and underlying tissues of the skin. This type of scleroderma has very less effect on person’s internal organs as compared to systematic scleroderma and it is also less severe. The skin changes associated with localized scleroderma typically present as patches of hardened, discoloured skin are of different size and shape. Luckily, in most of the cases, localized scleroderma does not involve other areas of the body while effecting.
2. Systemic Sclerosis Systemic sclerosis is a very severe variant of scleroderma that has the strong potential to impact not just the skin, but also major internal organs like the lungs, heart, kidneys, and digestive system. This type of scleroderma is further classified into two subtypes, that depends on the degree of skin involved. The subtypes are-
I. Limited Cutaneous Systemic Sclerosis: It oftenly occurs with the additional symptoms like Raynaud's phenomenon, which includes the diminishing of blood vessels in the toes and fingers because of stress or cold. This type of systematic scleroderma is prominent due to the thickening of the skin, which is mainly found in the hands, forearms, feet and face.
II. Diffuse Cutaneous Systemic Sclerosis: On the other hand, diffuse cutaneous systemic sclerosis is known due to the extensive thickening of the skin, which can majorly impact body parts like trunk and limbs. This particular type of the illness spread at a faster speed and has a higher rate of affecting internal organs of an individual. 3. Sine Scleroderma Diagnosing this type of scleroderma in its early stages can be specifically difficult due to the presence of very less visible skin symptoms, which may hamper the accurate detection of the illness. People with sine scleroderma may experience symptoms linked with organ dysfunction, also includes digestive system problems or pulmonary complications. Sine scleroderma, is a very less common form of the Scleroderma, it is differentiated by internal organ involvement without significant modification in the skin of the individual affected.
4. Overlap Syndrome
Overlap syndrome is a medical term used to explain a condition where a person shows features of both scleroderma and another autoimmune connective tissue diseases, such as rheumatoid arthritis or lupus. This type of scleroderma leads to difficulties in examining and treating the condition due to the overlapping symptoms and difficulties involved in understanding multiple autoimmune disorders simultaneously.
5. Juvenile Scleroderma Timely diagnose and effective treatment of juvenile scleroderma plays a crucial role in reducing the risk of long-term difficulties and enhancing the overall effect for those who are affected by this disease. Juvenile scleroderma, also known as childhood-onset scleroderma, is an uncommon illness that predominantly influence children and teenagers. This type of scleroderma can be seen as localized or systemic variations and may involve unique characteristics as compared to scleroderma that is seen in adults.
6. Calcinosis
The treatment for calcinosis is truly based on lighten pain and inflammation, while also targeting to hamper further calcification with the help of medication and lifestyle adjustments in the daily life of an individual. Calcinosis is a potential illness that may take place in certain individuals with scleroderma, generally occurs due to the development of calcium deposits in the soft tissues and skin. These deposits have the ability to induce pain, skin ulceration and inflammation, thereby additional obstacles are added in the management of severe scleroderma symptoms. 7. Raynaud's Phenomenon
Raynaud's phenomenon rarely takes place in individuals with scleroderma, this is basically decrease in blood circulation towards the fingers and toes that is triggered by cold or stress. This illness may lead to changes in skin colour, numbness or tingling sensation, and very discomfort when vasospasms occur. Treatment for Raynaud's phenomenon majorly includes lifestyle changes like staying away from cold weather conditions and wearing protective clothes, in addition to medicines the main aim is at increasing the blood flow and lowering the symptoms of Raynaud’s.
8. Pulmonary Involvement It is very important to monitor the symptoms and condition of a person regularly and take steps early in order to manage the complications associated with scleroderma on time, thereby the best therapy is maximizing the results and improving the quality of life they are living currently. Pulmonary difficulties are a major topic of concern in systemic sclerosis, with the ability to result in either pulmonary arterial hypertension or pulmonary fibrosis. These medical conditions have a great impact on the lungs and pulmonary blood vessels, that can lead to respiratory impairment and potentially life-frightening outcomes.
9. Gastrointestinal Manifestations Scleroderma has the strong ability to affect the digestive system or gastrointestinal system, resulting in problems like acid reflux, swallowing complications, bloating, and vast changes in bowel movements of the person affected. If left unattended gastrointestinal difficulties in scleroderma can result in malnutrition, weight loss, and a decrease in the living condition. Controlling these symptoms may need dietary changes, medications for symptoms relief, and intermediation to target particular gastrointestinal problems related to the illness.
10. Renal Complications Scleroderma has the great potential to affect the kidneys, resulting in situations like scleroderma renal crisis. This difficulty is different by the urgent emergence of severe hypertension and fast advancing kidney failure, that needs immediate medical treatment to stop the irreversible harm. Regular examination is very important to monitor kidney function and effective control of blood pressure are crucial aspects to look after in the control and prevention of renal difficulties in individuals suffering with this illness.
Symptoms of Scleroderma
The disease Scleroderma can include various changes based on the type and severity of the disease. Typical indications inculcate:
1. Thickening and hardening of the skin and tissues beneath
2. Raynaud’s phenomenon (fingers and toes turns white or blue due to cold and stressful conditions)
3. Digestive or gastrointestinal difficulties
4. Loss of Breath
5. Rigidity in body and Joint pain
6. Tiredness
Causes of Scleroderma
The accurate cause of Scleroderma is still not known; however, experts says that it is a mix of genetic, environmental, and immune system elements that could potentially lead to scleroderma. Specific response of stimuli, like exposure to toxins or infections, may also be a cause of initiating the autoimmune reaction seen most of the cases of Scleroderma.
Diagnosis and Treatment
Diagnosing Scleroderma is a very challenging task due to its vast symptoms and time to time changing features. Confirming after the examination of an individual often includes doing a comprehensive physical examination, blood tests, and imaging studies which plays a vital role. The treatment approach for Scleroderma basically revolves around symptom control, controlling complication, and slowing down the spread of the diseasing the body. Some commonly used treatment options are:
1. Using immunosuppressants to manage the immune response of the body.
2. Using vasodilators to speed up the blood flow.
3. Including physical therapy to gain joint movement and decrease joint pain.
4. Intake of medicines to lower down particular symptom.
Living with Scleroderma
Actively joining support groups and making connections with individuals who share similar experiences can offer invaluable opinions and also helps in motivating each other. It is necessary for individuals detected with Scleroderma to take a comprehensive approach towards their well-being and managing their daily lifestyle, which incorporates routine medical examinations, a nutritious diet, physical activity, and emotional well-being too.
Research and Future Perspectives
Currently ongoing research related to Scleroderma trying harder to decipher fundamental mechanisms of Scleroderma, discovery of potential biomarkers for early detection is very much needed, and invention more efficient and reliable treatment approaches is still going on. The clinical trials going on among researchers globally offer hope to individuals affected.
Conclusion
In summary, By imparting knowledge, empathy, and cooperation, each and every individual can truly make a great difference in the lives of those who are affected by Scleroderma. Scleroderma is known to be a complex autoimmune disorder that needs a comprehensive approach for its diagnosis and treatment. By promoting awareness, keeping hope in ongoing research, and empowering individuals affected by Scleroderma, whole community or world can work towards the betterment and well-being of the people suffering all around.
FAQ’s
1. In which part of the body scleroderma is seen commonly?
Scleroderma is commonly seen in hands, feet, face, fingers and toes.
2. Which age group is at higher risk of scleroderma?
Teenagers to adults (20yrs- 50yrs) of people are at high risk of being affected by Scleroderma.
3. Is scleroderma a genetic disease?
Scleroderma is said to be mix of genetic and environmental conditions but it doesn’t pass from parents to their offsprings.
4. Is Scleroderma a chronic disease?
Yes, it is a lifelong disease but changes can be seen in the severity of symptoms if a person changes his/her lifestyle.
5. Who discovered Scleroderma?
Carl Cruzio was the first person to discover Scleroderma in 1753.