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What You Need To Know About Scleroderma
Scleroderma also knew as systemic sclerosis is an autoimmune disease that causes alterations in some organs of the body. Scleroderma usually affects organs such as the liver, lungs, muscles, cardiovascular system, skin and so on. This disease usually presents in two forms; It’s either limited to the skin or affect multiple organs of the body. Some of the symptoms of the disease include the thickening of the skin, fatigue, and inadequate blood flow to the extremities. Generally, patients affected by this disease present with the CREST syndrome. The CREST syndrome stands for Calcinosis, Raynaud’s syndrome, Esophageal dysmotility, sclerodactyly, and telangiectasia.
The exact cause of this disease is not yet known, however, there are some factors that increase the chances and contributes to the development of this disease. The most important risk factors are heredity and environmental factors. This disease is diagnosed based on the presenting symptoms of the patient and confirmed by some specific procedures. This disease has no cure, however, medications are given by physicians to help the affected people live a comfortable life, and to possibly increase their lifespan.
What Are The Signs And Symptoms Of Scleroderma?
The signs and symptoms of scleroderma largely depend on the part of the body the disease affects. Below are some of the symptoms of this disease;
Extremities: One of the initial signs of scleroderma is the way the body extremities react to cold temperatures or a sudden change in weather. The extremities, especially the fingers and toes often change color and become numb. The patient might also experience some pain. This is referred to as the Raynaud syndrome.
Skin reaction: Almost everyone affected by scleroderma presents with a thickening of their skin. In addition, there might be lesions and patches on the skin. These patches might sometimes take the shape of an egg or just appear as straight lines. These patches can appear on certain parts of the body such the trunk and limbs. The skin might look tight and shiny, this is because of the tightening and thickening of the skin.
Gastrointestinal system: Scleroderma causes gastroesophageal acid reflux. This is a condition in which acid moves from the stomach back into the esophagus. Apart from this, this disease may also cause malabsorption. It does this by reducing the bowel movement. It causes a reduction in the way the intestines move and absorb nutrients.
Pulmonary system: Scleroderma leads to the gradual deterioration of the muscles used in respiration. This eventually leads to shortness of breath, chest discomfort and pain. Also, patients might develop interstitial lung disease, that might lead to unproductive dry coughing.
Reproductive system: This disease might lead to the development of erectile dysfunction. Also, affected people might experience pain when having sexual intercourse.
Musculoskeletal system: Patients experience pain in the muscles and joints of the body. In addition to this, they might also present with a reduction in the range of motion of their joints. Carpal tunnel syndrome is also a common occurrence.
Others: There are some symptoms that are not common, but have been reported to be present in some cases. Some of these symptoms are headache, stroke, tiredness and so on.
What Is The Cause Of Scleroderma?
Scleroderma is mostly as a result of excess collagen in the body tissues. Collagen is the building substance of the connective tissues, which includes the skin. The exact mechanism of how this disease isn’t known yet, however, the immune system has been suggested to play an important role in t eh development of this disease. People who are genetically predisposed to having this disease might be triggered by some factors. Some of these factors are chemicals in the form of insecticides, chlorinated solvents and so on.
What Are The Complications Of Scleroderma?
The complications usually vary in different people. It can also be mild, moderate and severe. Below are some of the complications of scleroderma, especially if left untreated;
Pulmonary system: Scleroderma can lead to the impairment of the lung tissues. This could lead to the reduction in the ability to breathe. In addition, patients might develop hypertension in the pulmonary arteries. All this occurrence can be attributed to the accumulation of collagen in the lung tissues.
Skin: Raynaud’s disease, one of the components of the CREST syndrome can sometimes get very severe, that it could cease the flow of blood to the tissues, especially in the extremities. The fingers and toes are largely affected by this condition. If this is left untreated, the patient might end up having gangrene. The treatment of gangrene is surgery. This shows how severe scleroderma can be.
Renal system: Accumulation of collagen tissues in the kidney and renal system due to scleroderma can lead to hypertension. In addition to this, patients might experience proteinuria. In severe cases, especially in not well managed, patients might end up with kidney failure.
Cardiovascular system: Scleroderma in the heart usually leads to the scarring and thickening of the heart muscles. This could lead to the development of other heart-related conditions such as arrhythmia, cardiac failure, pericarditis and so on.
Reproductive system: This disease causes erectile dysfunction in men. This is probably due to the hardening of the penile vessels, making it difficult for blood to engorge it when aroused. However, women affected with scleroderma often present with reduced vaginal lubrication. In addition, their vaginal opening might become constricted.
Digestive system: Scleroderma causes gastroesophageal acid reflux. This is a condition in which acid moves from the stomach back into the esophagus. Apart from this, this disease may also cause malabsorption. It does this by reducing the bowel movement. It causes a reduction in the way the intestines move and absorb nutrients. Patients might also present with constipation, that alternates with diarrhea.
How Is Scleroderma Treated?
This disease does not have a cure yet, however, there are some medications that ease the pain, and gives the patient a more comfortable life. These medications are aimed at reducing the pain, giving the patient more strength, especially in the affected part, and also making life comfortable for the patient.
References
I103. Scleroderma Management. (2015). Rheumatology.
Kreuter, A. (2012). Localized scleroderma. Dermatologic Therapy, 25(2), pp.135-147.
Moinzadeh, P., Hunzelmann, N., Kreuter, A. and Krieg, T. (2016). Localized scleroderma: a review. Journal of Scleroderma and Related Disorders, 1(3), pp.286-297.
Scleroderma: management. (2014). Clinical Pharmacist.
