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Scleroderma: Etiology, Pathology and Treatment

Selected Disease

Being diagnosed with scleroderma is often viewed as a crisis, even when its overall impact can be positively influenced by compassion, input, and education from the medical team. When patients diagnosed with scleroderma are equipped with knowledge about the disease, it becomes easy for them to act as advocates of their health. They, therefore, start becoming critical and inform partners during their treatment. It is crucial to have a practical approach that will be important in identifying scleroderma patients. Scleroderma is rare but a chronic autoimmune illness known for replacing one’s normal tissue with a thick one containing extra collagen. It is an ailment that can quickly attack different parts of the body. This paper seeks to discuss scleroderma by exploring its etiology, pathology, clinical signs and symptoms, and the drugs used for its treatment.

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Disease definition

There is still no study that has managed to provide its leading causes regarding scleroderma’s etiology. However, several environmental factors seem to relate to sclerodermas, such as illnesses which include exposure to epoxy resins, silica dust, vinyl chloride, and other organic solvents (“Scleroderma – an Overview | Sciencedirect Topics,” n.d.). Some proof of geographic clustering has also been indicated in various researches. The evidence in the studies appears to be consistent with various possible environmental risk factors related to scleroderma. According to Ayello & Sibbald (2018), scleroderma may be best described as a disease that contains two components: a trigger event and genetic susceptibility. For instance, exposure to organic solvents or silica dust particles. Singh et al. (2019) note that some studies have shown support for the idea of the existence of systemic infections in patients, which act as the trigger for scleroderma. Other studies have also tried to suggest a possible connection between Lyme disease and scleroderma, with the latter being viewed as a potential trigger in vulnerable patients (Ayello & Sibbald, 2018). However, the above suggestion is weak because of the absence of any direct studies that link Lyme disease and scleroderma.

In terms of its pathology, the scleroderma disease is known for targeting the connective tissue, vasculature-producing cells, namely myofibroblasts or fibroblasts. It also targets the components of an individual’s adaptive and innate immune system (Singh et al., 2019). Singh et al. (2019) further state that the pathological and clinical presentations of the illness are exacerbated by fibroblast dysfunction, which produces excess collagen deposition and other matrix components in the internal organs and skin. Thet are the defects of the innate and adaptive immune system which stimulates cell-mediated autoimmunity and autoantibodies, and the improper working of the small vessel fibroproliferative microvascular and vasculopathy endothelial cell. Each of the above three processes affects each other because of their constant interaction.

In its clinical presentation, scleroderma is usually heterogeneous, making it likely to reflect different provoking or genetic factors. Its triggering factor significantly influences the connective tissue cell, immune system, and vasculature tissue cell (Singh et al., 2019). Research on the function of other pervasive molecular entities like the impact of the endocannabinoid system on the vasculature, connective tissues, and immune system offers a glimmer of hope for scleroderma therapeutic strategies.

Although there are several other signs such as heart, kidney, lung, and digestive system failures that indicate the presence of scleroderma, the first specific clinical symptom that indicates its diagnosis is skin thickening (Singh et al., 2019). The thickening starts as swelling (puffiness) of the hands and fingers (“Scleroderma – an Overview | Sciencedirect Topics,” n.d.). The worst time for anyone diagnosed with scleroderma is in the morning when the swelling is usually painful but reduces later in the day, especially when the disease is in its early stages (“Scleroderma – an Overview | Sciencedirect Topics,” n.d.). If left untreated, the affected skin later becomes leathery, hard, and shiny. However, it is essential to note that the disease’s signs and symptoms vary, depending on the parts of the body affected.

Drugs Used to Treat Scleroderma

Although there is still no perfect drug or therapeutic approach to scleroderma, several are thought to reduce the effects, as shown in this section. The first drug is prednisone, which is used for treating conditions such as severe allergies, eye problems, and arthritis. Its trade is Deltasone, while its generic name is prednisone. It is primarily classified as one of the corticosteroid drugs (“Scleroderma Treatment Options,” n.d.). With regard to its indication, it is used in the treatment of the immune system. The drug’s action primarily helps reduce inflammation and dampen down the immune system to assist in the autoimmune of various diseases (“Scleroderma Treatment Options,” n.d.). The side effects of the drug include heartburn, dizziness, headache, and increased sweating. The drug dosage varies from 5mg to 60mg per day, depending on the disease being treated.

Azathioprine is the second drug that can be used in managing scleroderma. The drug is usually sold under the trade names Imuran and Azasan. It is classified under immunomodulators, DMARDS, and immunosuppressants, while its generic name is Azathioprine tablet (Singh et al., 2019). Azathioprine is indicated in immunosuppressive regimens as an adjunct to the immunosuppressive agent that usually forms a mainstay of treatment. In terms of action, Azathioprine functions as an agent that suppresses the immune response (Singh et al., 2019). Some of the drug’s side effects include blood and lymphatic system disorder, neoplasms and benign, and immune system disorders. In terms of administration, the drug is administered orally, where the oral dosage of 50 mg/ml includes extemporal compounding by a chemist.

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Nintedanib is the last drug, which trades by the names of Vrahgatef and Ofev. It is classified under Tyrosine Kinase inhibitor and pulmonary drugs. Its action includes the treatment of idiopathic pulmonary fibrosis and other specific types of chronic fibrosing interstitial lung diseases (Singh et al., 2019). The drug is primarily indicated for the idiopathic pulmonary fibrosis treatment in adults. Some of the drug’s side effects include nausea, chest pain, vomiting, and weight loss (Singh et al., 2019). Oral administration is recommended when using Nintedanib. It should not be taken without food, and all the capsules must be wholly swallowed with liquid. However, it is not advisable to crush or chew the tablets. Any missed dosage must be taken at the next scheduled time.

Conclusion

In conclusion, this paper has sufficiently discussed scleroderma by thoroughly exploring its etiology, pathology, clinical signs and symptoms, and three drugs that can be used in its treatment. From the discussion, it is clear that there is a need for further research to determine the leading causes of the disease and the right drugs to use in its treatment. Knowing the leading causes will be significant in developing the right medications and therapeutic approaches.

References

Ayello, E. A., & Sibbald, R. G. (2018). Scleroderma and person/patient-centered concerns. Advances in Skin & Wound Care, 31(10), 437. Web.

Scleroderma treatment options. (n.d.). Web.

Scleroderma—An overview | sciencedirect topics. (n.d.). 2020, Web.

Singh, D., Parihar, A. K., Patel, S., Srivastava, S., Diwan, P., & Singh, M. R. (2019). Scleroderma: An insight into causes, pathogenesis, and treatment strategies. Pathophysiology, 26(2), 103–114. Web.

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