Emma Holmes
9 November 2017
Induced Sputum Analysis in Subjects with Systemic Sclerosis (2016)
Thermography Improves Clinical Assessment in Patients with Systemic Sclerosis Treated with Ozone Therapy (2017)
Familial occurrence frequencies and relative risks for systemic sclerosis (scleroderma) in three United States cohorts (2001)
Systemic sclerosis, also called scleroderma, is a rare autoimmune disease that affects multiple different bodily systems. The disease is characterized by an overproduction of collagen in the skin and around internal organs. One organ commonly affected is the lungs, resulting in a morbidity and mortality rate. Interstitial lung disease is prominent in many systemic sclerosis patients, and induced sputum technology is well established in early detection of lung inflammation and diseases. Induced sputum is a tool used for retrieving cellular materials from lung airways. A study using induced sputum was conducted to test the lung airways of 20 systemic sclerosis patients versus 16 healthy volunteers. The purpose of this study was to test in inflammation of the lungs in patients with systemic sclerosis and to identify possible manifestations of the disease. The testing was done by inducing sputum through inhalation of a hypertonic saline, which was then recovered after 20 minutes through a mouthpiece and nose clip. The results of the testing concluded that patients with systemic sclerosis had a higher amount of neutrophils in their airways, and a lower amount of lymphocytes compared to the healthy subjects. The study also revealed changes in the cellular material that correlated with pulmonary function tests. However, additional testing with more subjects may be necessary for validating the study's results.
Systemic sclerosis, or scleroderma, is an autoimmune disease that affects the skin and internal organs due to fibrous connective tissue build up in the hypodermis and lining of blood vessels. Symptoms can include ischemia, reduced body temperature, skin atrophy, joint stiffness, and limited mobility (fingers become "claw-like"). No cure exists due to the unknown cause of the disease. However, treatments worldwide are designed to slow down to the process of fibrosis and reduce inflammation and blood pressure changes. In 2006, a study in Poland was conducted testing the benefits of ozone therapy using thermography in 42 hospitalized scleroderma patients. The patient's blood pressure, index of skin thickness (skin score), joint mobility, and body temperature were tested before and after the ozone treatment. The patients were then placed in comprehensive air-baths, first without ozone and then with. To determine the effectiveness of the treatment, thermography testing was done after each non-ozone and ozone-fused baths. The results of the study concluded that exposure to the ozone increased the body's surface temperature, and joint mobility, decreased the skin score value and decreased blood pressure. The study determined that these benefits resulted from ozone gas catalyzing an enzyme that has been found to relax the endothelium lining of smooth muscles. Therefore, this treatment relaxes the fibrous blood vessels allowing for an improvement in the symptoms. Although this is not a cure, this treatment has proved effective and beneficial to patients suffering from this disease.
While the cause of scleroderma is still unknown, studies and surveys are conducted to try and understand the etiopathogenesis of the disease. A US study based on three cohorts of first-degree family members (parents and siblings) of patients with scleroderma were surveyed to calculate the familial risks along with determining genetics versus environmental factors. The first cohort was studied in Texas by the Health Science Center at the University of Texas-Houston and consisted of 190 patients with the disease. The second cohort was studied by the Specialized Center of Rheumatology in Scleroderma in Texas and consisted of 175 early onset patients. The third cohort consisted of 338 patients with scleroderma surveyed in Michigan. Medical records of the family members were obtained to determine the history of any other autoimmune disease. The results of the study concluded an average of 1.6% frequency in first degree relatives of scleroderma patients. This puts family members at a relatively low risk (less than 1%), yet is a significantly higher frequency than the general population, which averages 0.026% (2.6 case/10,000 population). The studied also showed higher frequencies in siblings over parent-child relations. This study was consistent with a similar one conducted in Australia, therefore, the conclusion is found to be highly accurate. However, since both percentages are low it is difficult to prove if genetics has an influence or if a family occurrence of the disease is due to exposure to similar environmental factors. More surveys and studies will need to be conducted in order to determine the key factor.
Scleroderma was my main topic of choice because my grandfather is currently suffering from this disease. Due to the rarity of the disease, especially in men, many of my family members do not fully understand the severity of my grandfather's illness. While it is a form of arthritis, scleroderma has a very high mortality rate since it affects internal organs and blood vessels. It has been around four years since his diagnosis and my family is beginning to see a drastic decline in my grandfather's health. Who was once an active man, is now barely able to brush his own teeth (his hands have almost stiffened to the point of complete immobility). Now that I have some extent of background information on systemic sclerosis, I will be able to explain to my grandfather and other family members the anatomy and physiology behind his illness and possibilities of new treatment options. For example, I now understand that scleroderma affects major organs such as the lungs and heart, and keeping these organs healthy is important in prolonging life-expectancy.
References
Litinsky, I., Fireman, E., Paran, D., Polachek, A., Broide, A., Sharabi, A., . . . Elkayam, O. (2016). Induced Sputum Analysis in Subjects With Systemic Sclerosis. Respiratory Care,61(10), 1369-1373. doi:10.4187/respcare.04706
Nowicka, D. (2017). Thermography Improves Clinical Assessment in Patients with Systemic Sclerosis Treated with Ozone Therapy. BioMed Research International, 2017, 1-7. doi:10.1155/2017/5842723
Arnett, F. C., Cho, M., Chatterjee, S., Aguilar, M. B., Reveille, J. D., & Mayes, M. D. (2001). Familial occurrence frequencies and relative risks for systemic sclerosis (scleroderma) in three United States cohorts. Arthritis & Rheumatism, 44(6), 1359-1362. doi:10.1002/1529-0131(200106)44:6<1359::aid-art228>3.0.co;2-s