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What is Scleroderma? Why stem cells are now a popular treatment

By Dr. Santiago Saldarriaga

The immune system is an incredibly complex system that affects every part of the body. The body relies on the immune system to keep vital organs and tissues safe from outside invaders. Typically, the immune system does an exceptionally good job in fighting off potential threats. When there is an issue with the immune system, the body can react in strange ways and develop symptoms when the individual is otherwise healthy.

A relatively common autoimmune disorder is known as scleroderma. The immune system sets off a series of reactions that can cause serious health issues. This disease affects the amount of collagen and other proteins that are produced in connective tissue. The increased level of collagen can create structural issues in the tissue, which affects its functionality.

There are more than 300,000 Americans who suffer from scleroderma. There are multiple types of scleroderma, depending on the severity and areas of the body that are affected. Life can be extremely difficult for patients who suffer from systemic sclerosis. Patients may suffer from severe pain and could be embarrassed by the appearance of their skin. There are also several life-threatening complications that can develop due to this condition.

There are currently no cures for scleroderma or effective treatments that target the root cause of the condition. Patients can only treat the symptoms of the condition. The medical community is looking into ways that can improve the outcome of patients who are suffering from scleroderma. Patients could improve their quality of life and life expectancy, if the right treatments become available.

Researchers are beginning to come to the conclusion that stem cell therapy can improve patient outcomes and lead to the regression of the disease. Stem cells have a number of properties that could help solve the underlying causes of scleroderma. Let’s discuss the disease and background to better understand how stem cell therapy may be an effective treatment.

What Is Scleroderma?

Scleroderma is caused by an overproduction of collagen and proteins in tissues throughout the body. Collagen helps to maintain structural integrity in various tissues throughout the body, but too much collagen can create issues. Women are four times more likely to develop the condition.

The skin of patients who suffer from scleroderma changes in texture and appearance. These patients typically develop fibrosis, or fibrotic scarring, which occurs when connective tissue replaces normal tissue at a rate that tissue remodeling and scar tissue formation occurs. When fibrosis occurs on the skin it can reduce the confidence of patients due to the skin’s appearance.

Other organs and systems, such as the lungs, gastrointestinal tract, heart, and kidneys, can be affected by systemic sclerosis. Patients who develop systemic scleroderma can develop fibrosis throughout the body and can even affect various organ systems. There are multiple types of scleroderma that patients can develop.

Types of Scleroderma

Knowing the different types of scleroderma can help patients ensure that they receive the right treatment plan.

Limited Systemic Sclerosis

Most patients only develop localized scleroderma, where the symptoms only affect parts of the skin on various parts of the body. This condition is also known as CREST syndrome. Other organs are generally not affected. It can increase the pressure on arteries throughout the body, which can affect organs. Limited scleroderma progresses slowly and can be complicated by pulmonary hypertension.

Diffuse Systemic Sclerosis

The more serious form of the condition is also known as systemic scleroderma or systemic sclerosis. This type of systemic sclerosis can progress rapidly and cause major complications. The lungs and kidneys can be damaged seriously over time if the disease continues to progress.

Musculoskeletal Issues

Patients with scleroderma can feel symptoms in their joints. Patients can develop arthritis and discomfort in tendons or muscles. The skin thickening or tightening can lead to issues with joint mobility. Muscle weakness is also a potential concern. A grating sound can be felt or heard as tissue moves over each other. Fingers, wrists, and elbows can get stuck in a fixed position due to the scarred tissue.

Gastrointestinal Issues

Diffuse systemic scleroderma can affect any part of the digestive system. The esophagus is one of the most affected parts of the digestive tract. When the esophagus becomes damaged, it can be difficult for some patients to properly swallow food. Patients could suffer from heartburn and additional damage to the esophagus due to acid reflux.

The mobility of the gastrointestinal tract can also be affected where it primarily affects the esophagus and leads to dysphagia and chest pain. The intestines can also be damaged, which can prevent the body from properly absorbing nutrients and cause weight loss.

Heart and Lung Issues

Systemic sclerosis can create scar tissue in both the heart and lungs. Patients may develop interstitial lung disease, which can make breathing more difficult during exercise. The collagen production can interfere with the arteries and blood vessels in the lungs. This can thicken the blood vessel walls, which can lead to the development of pulmonary hypertension. The damaged heart structure can lead to life-threatening heart conditions, such as heart failure and arrhythmias.

Kidney Issues

Patients with systemic sclerosis can suffer from kidney damage. Around 7-9% of patients with scleroderma will suffer from renal crisis and have to address their kidney issues. Patients who suffer from kidney damage related to scleroderma have a poor prognosis, but some patients are able to improve over time.

The life expectancy of patients who suffer from localized scleroderma is typically not reduced, but patients who develop the systemic version have a reduced life expectancy. The fibrosis that develops in organs can cause structural damage that affects the organ’s ability to function. Patients with this type of scleroderma have a survival rate of 65% over 10 years after the diagnosis is made. Patients who suffer from kidney disease may develop a unique form of systemic sclerosis.

Researchers do not fully understand why patients develop the condition. There are some theories. Patients with a family history of systemic sclerosis are at an increased risk of developing the condition themselves.

What Causes Systemic Scleroderma?

The main symptom of systemic scleroderma is the overproduction of collagen. Most researchers agree that the immune system signals the body to produce more collagen than is necessary, but there is some debate as to how that happens. The immune system then attacks the kinetochore of the chromosomes, which can cause genetic damage to cells. The T-cells that accumulate in the skin secrete factors that stimulate collagen production.

Researchers believe that there are three processes that result in the symptoms of scleroderma. These processes are all likely the result of immune system dysfunction.

1) The severe fibroproliferation of vascular lesions on small arteries and arterioles. This can lead to the prgoressive deterioration of the normal function and structure of the skin and organs.

2) An excessive and prgoressive production of collagen and other macromolecules in the skin and other organs.

3) And the alteration of cellular immunity.

Researchers do not fully understand which of these processes are more responsible for the development and progression of the disease. Studies have shown that there is a certain sequence of events that are triggered by an unknown factor in patients that are genetically inclined to develop scleroderma. The factor triggers endothelial cell abnormalities to increase production of various potent mediators and/or the reduction of important cell compounds.

The cell dysfunction attracts inflammatory cells and fibrocytes from the bloodstream and bone marrow into the surrounding tissues. T-cells and B-lymphocytes also secrete cytokines and growth factors into the tissues. This results in a chronic inflammatory process. This process creates the perfect environment for the development of scleroderma.

The medical community has attempted to develop various treatments to improve the lives of patients with scleroderma.

Current Treatments For Systemic Scleroderma

Patients do not have any treatment options that can prevent the continued overproduction of collagen and other proteins. The goal of treatment plans is typically to improve symptoms and prevent complications.

Medication

There are no medications that can prevent the progression of scleroderma or reverse the progress of the condition. Certain medications can help treat specific symptoms and organs. Corticosteroids can be useful in reducing joint stiffness and pain. Long term steroid use can adversely affect cartilage in joints, which can increase pain levels.

Non-steroidal anti-inflammatory drugs can help patients manage their level of pain. This type of medication can cause gastrointestinal problems for some patients. Topical treatment for skin changes can improve pain levels and the skin’s appearance. Anti-inflammatory drugs can reduce the level of inflammation present in the skin. Skin tightness and skin thickness can both be treated with different topical medications.

Immunosuppressive drugs can reduce the symptoms of this condition. The immune system’s reaction can be minimized, but there are potential consequences. Patients who use immunosuppressive drugs are much more susceptible to diseases, as their immune system is weakened.

Physical Therapy

Patients could work with a physical therapist to improve their strength and mobility. This could help reduce stiffness and pain in various joints that are affected by the condition. Patients could handle more of their everyday tasks, if they engage in a physical therapy program. Physical therapists will develop a customized treatment plan for patients to strengthen specific muscles and work on flexibility.

Surgery

Surgery is used to treat symptoms of the condition, not the root cause. Patients may have to amputate fingers that have too much dead tissue. Patients who have suffered from lung damage can potentially receive a lung transplant.

The medical community has not been able to develop effective treatments for scleroderma. Patients continue to suffer from the effects of this disease with little or no hope. Researchers are hopeful that stem cell therapy could be an effective treatment for patients who are suffering from all types of scleroderma.

Stem Cell Therapy for Systemic Sclerosis

Researchers are becoming more convinced that stem cell therapy is going to be the future of systemic sclerosis treatment. Stem cells have certain properties that make them attractive for therapeutic treatments for autoimmune conditions.

Researchers know that stem cells have the ability to modify the immune system’s response. Multiple studies have shown that stem cells have immunomodulatory effects. Researchers do not fully understand how stem cells regulate the immune system, but there is promising evidence that this could help patients with scleroderma. Stem cells may be able to balance cytokine and growth factors in scleroderma, adjust the immune cellular balance, and abate the ongoing fibrosis.

Generally, stem cell therapy is a safe treatment for patients. Stem cells are the building blocks of the body and the immune system does not recognize stem cells as a foreign invader. Multiple studies have shown a majority of patients do not suffer from adverse reactions when they are treated with stem cells. The body will utilize stem cells to regenerate damaged tissue and cells and potentially reduce inflammation.

The medical community is researching how stem cell therapy could benefit patients who are suffering from various forms of scleroderma.

Promising Research

The medical field has been looking into how stem cell therapy can improve the outcome of patients who are suffering from various types of scleroderma. The main method of stem cell therapy that researchers are looking into is hematopoietic stem cell transplant. Researchers are hopeful that this treatment method can reset the immune system and ensure positive outcomes for patients with scleroderma.

Researchers at Duke University Medical Center looked into how patients with diffuse systemic sclerosis would respond to stem cell therapy. The researchers were hopeful that stem cells could change the way that the immune system reacts. 75 patients with diffuse systemic sclerosis were recruited for the study.

39 patients received the normal treatment of immunosuppressants. The other 36 patients were given autologous stem cell transplants.The immune system was destroyed through chemotherapy and total-body irradiation and then the patients were given the transplants to restore the immune system.

The patients were then followed over the course of 4.5 years. Patients were evaluated based on their disability score, survival rate, vital capacity, and event-free survival. The group that received stem cell transplants fared significantly better than those who underwent the typical treatment protocols.

The group of patients who received stem cell transplants had better outcomes than the group who only received immunosuppressants. 67% of the stem cell transplant patients experienced significant improvements, while only 33% of patients in the other group saw significant improvements. 79% of the stem cell patients did not show any signs of disease progression, compared to 47% in the other group.

Only 9% of the patients in the transplant group required additional treatment of anti-rheumatic drugs, compared to 44% in the other group. 86% of the patients who underwent the stem cell transplant survived, while only 51% of the patients who underwent the typical treatment regimen survived. There were patients who died from complications related to the stem cell transplant.

Another long-term study looked into the effects of stem cell transplantation on patients with scleroderma. Stem cells have the potential to keep patients with scleroderma off certain drugs and can even make them disease free. Researchers were able to follow up with patients 11 years after they had received their treatment.

Patients who had received stem cell transplants had improved musculoskeletal function and gained weight. Organ failure was recorded in two patients who had received stem cell therapy, while six patients who received the typical treatment had organ failure. 92% of the stem cell transplant patients did not need to take anti-rheumatic drugs, while only 61% did in the other group. 80% of the patients who received stem cell transplants survived, whereas only 52% of patients in the other group survived.

Researchers are confident that stem cell transplantation is a viable treatment method for patients with scleroderma in the long run. Stem cells are able to modify the immune response in a way that prevents further disease and potentially causes remession of the disease.

There are some risks to this treatment method. The potential issue with destroying the entire immune system is that it leaves the patient susceptible to diseases and viruses. Patients will not be able to defend themselves against various pathogens as there is no immune system to protect them. This should be considered when a patient chooses a treatment.

If a patient has a more mild form of scleroderma, then the potential risks may not be worth it. A patient may not want to risk being susceptible to pathogens. If stem cells can be utilized to change the way the immune system responds, then there is no need to destroy the entire immune system. Patients can receive stem cells through an IV or injection where they can reduce the immune system’s reaction.

Another study looked at utilizing umbilical cord stem cells to treat two patients who were suffering from progressive scleroderma. The patient's quality of life had deteriorated from the condition and no treatments were working. The patients received a dose of 2 million stem cells.

Both patients improved remarkably after the procedure. The first patient was suffering from difficulty breathing while walking, severe joint pain, and skin contracture to the point where she could not hold a spoon or fork to feed herself. After the procedure, the patient reported that she got her freedom back. She was able to walk more than 300 feet without labored breathing and could hold a spoon or fork to feed herself. Her pulmonary pressures normalized as well.

The second patient also had difficulty breathing when walking and significant joint pain. She also reported similar results as the first patient. There was a significant relief of pain and she was no longer suffering from pulmonary hypertension. Her mobility and ability to exert herself returned to normal. She was even able to return to her job as a nurse.

It is clear that stem cell therapy is a potentially effective treatment for patients who are suffering from scleroderma. Stem cells have the ability to change the immune system’s response and prevent it from causing the chain reaction that results in scleroderma. BioXcellerator is proud to work with patients who are suffering from various autoimmune diseases and disorders. Our team is always focused on achieving the best possible outcomes for all of our patients.

Golden Stem Cells

The BioXcellerator team understands the importance of quality in regards to the treatments that patients receive. We always strive for the best outcomes for our patients, which is why we always go the extra mile. Some patients may be worried about traveling to Colombia to receive stem cells. We understood this concern for patients, which is why we decided to try and ease our patients minds.

BioXcellerator has developed an exceptional quality control process that we believe is the first of its kind. Our team spent countless hours researching the characteristics and features of stem cells that had high potential for therapeutic uses. These characteristics gave our team a roadmap to develop a quality control process.

We can then use these biomarkers to filter out stem cells that are not as potent. The quality control process uses the identifying characteristics of stem cells with high therapeutic potential to ensure that our patients receive the highest quality stem cells. Our team can use this process on any group of stem cells.

The resulting group of stem cells should have a higher therapeutic potential than stem cells that do not go through the same process. Our team likes to call this group of stem cells ‘golden cells.’ Golden cells could have the potential to change how various conditions and diseases are treated. Our team believes that scleroderma patients may be able to utilize golden cells to improve their symptoms and prevent further disease progression.

Reach out to your healthcare provider today to learn more about potential treatments for systemic sclerosis. Stem cell therapy could improve the outcome of patients who are suffering from various forms of scleroderma. Stem cells have the potential to change the way that the medical community treats scleroderma and could result in fewer deaths.

Tags: scleroderma, stem cell treatments

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