Systemic sclerosis is a chronic autoimmune disease in which there is an alteration of collagen (a set of proteins that support the organs and tissues of the body), which causes the skin to sclerose, that is, hardens, as well as the different organs that it can affect, especially the lungs, kidneys, heart and gastrointestinal tract.
It is a rare disease of unknown origin, affecting one in 50,000 people. It can appear at any age, being more frequent in middle-aged women and its evolution can be more or less rapid depending on the degree of involvement.
During the first year, the rheumatologist can usually classify and make a prognosis of the severity of the disease in each case, and warn the patient about what complications are possible, how to monitor them and the most appropriate treatment for them.
Is Scleroderma A Form Of Cancer?
Scleroderma is not a form of cancer. Instead of this, systemic autoimmune diseases are associated with a higher incidence of lymphoproliferative disorders and some solid tumors. Likewise, multiple recent studies have shown an increased risk of cancer in patients with scleroderma, especially hematological tumors, lung, breast, skin, and esophagus.
The association between cancer and systemic scleroderma has been very controversial, but several epidemiological studies on the subject have been carried out in the last decades that allow affirming that the existing relationship between both diseases is real.
The risk is greater for the diffuse systemic form and when the disease appears in the male sex. Its relationship with the presence of some specific type of autoantibody is disputed. There are different opinions on what is the risk attributable to each type of cancer in particular. Lung cancer, breast cancer, non-melanoma skin cancer, hematological neoplasms, tongue cancer and hepatocarcinoma are the neoplasms that are statistically linked to the disease. Most works coincide in pointing to lung cancer as the malignant disease of greater incidence, with an increased relative risk that varies between 4.4 and 5.9 according to the different studies. From case series and isolated communications, more associations arise, such as esophageal and gastric cancer, among others.
Proposed Mechanisms
Cancer begins as a result of the tissue changes that scleroderma generates. An example of this is lung carcinoma that develops from the pulmonary fibrosis that the disease causes.
Scleroderma arises because of immunological alterations induced by the tumor or due to substances released directly by it.
There could be a genetic predisposition that determines the appearance of both diseases, although the possibility that an environmental agent not yet identified is responsible for this common susceptibility cannot be ruled out.
Immunosuppressive therapy such as ultraviolet radiation therapy carries a risk inherent carcinogenicity, and both are often used in systemic scleroderma. In several cases, scleroderma, or a state similar to it (sclerodermoid), appears as a result of the chemotherapy treatment of a tumor. The drugs most frequently involved are taxol, bleomycin, INF-α, and doxorubicin.
It is considered that a complete interrogation and physical examination is sufficient, and complementary studies will be requested when positive data arise.
Localized Scleroderma and Cancer
The frequency of the types of cancer was similar to those of the general population: carcinoma of vulva, breast, and lung. Patients with localized scleroderma may develop spinocellular epithelioma, as well as those suffering from burns or chronic radiodermatitis with cerebral tissue. Immunosuppressive therapy is an additional risk (a patient who presented a fibrotic plaque of morphea and was treated with azathioprine, developing a squamous cell carcinoma in the scar area). Azathioprine can facilitate the development of several tumors such as spinocell and basal cell, lung, and kidney.
The literature describes morphea related to breast cancer and its treatment with radiation with supervoltage. Both IL4 and tumor growth factor activate the fibroblasts. Stimulation of fibroblasts with increased collagen production after the radiation may be due to the increase of the tumor growth factor and through the stimulation of fibroblasts, which produce reactive oxygen species. Many tumors including breast cancer and lymphoreticular neoplasms are associated with stromal fibrosis.
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