Systemic sclerosisis a inveterate autoimmune disease in which there is an modification of collagen ( a set of proteins that support the Hammond organ and tissues of the soundbox ) , which causes the cutis to sclerose , that is , hardens , as well as the different organs that it can affect , especially the lung , kidney , tenderness andgastrointestinal nerve pathway .
It is a rare disease of unknown line , affecting one in 50,000 people . It can seem at any age , being more frequent in middle - cured womanhood and its evolution can be more or less rapid depend on the degree of participation .
During the first twelvemonth , the rheumatologist can usually classify and make a prognosis of the rigorousness of the disease in each case , and monish the patient role 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 mannequin ofcancer . alternatively of this , systemic autoimmune disease are consociate with a higher incidence of lymphoproliferative disorders and some solid tumor . Likewise , multiple late studies have shown an increased risk of genus Cancer in patient with scleroderma , peculiarly hematologic tumors , lung , breast , skin , and esophagus .
The affiliation between Crab and systemic scleroderma has been very controversial , but several epidemiological studies on the subject have been convey out in the last 10 that allow affirming that the existing human relationship between both diseases is veridical .
The risk is greater for the diffuse systemic form and when the disease appear in the male sex . Its kinship with the presence of some specific type of autoantibody is disputed . There are different legal opinion on what is the risk attributable to each character of malignant neoplastic disease in special . Lung genus Cancer , breast cancer , non - melanoma skin cancer , hematologic neoplasms , clapper cancer and hepatocarcinoma are the tumor that are statistically connect to the disease . Most work coincide in pointing to lung malignant neoplastic disease as the malignant disease of greater incidence , with an increased relative risk of infection that varies between 4.4 and 5.9 according to the different studies . From typesetter’s case series and isolated communication , more association arise , such as esophageal and stomachic Crab , among others .
Proposed Mechanisms
Cancer start as a outcome of the tissue paper changes that scleroderma sire . An example of this is lung carcinoma that develops from thepulmonary fibrosisthat the disease causes .
Scleroderma develop because of immunologic alteration induced by the tumor or due to nub unblock directly by it .
There could be a genetic predisposition that determines the coming into court of both diseases , although the possibility that an environmental agent not yet identified is responsible for this common susceptibleness can not be ruled out .
Immunosuppressive therapy such as ultraviolet radiation therapy carries a risk built-in carcinogenicity , and both are often used in systemic dermatosclerosis . In several pillowcase , genus Scleroderma , or a state similar to it ( sclerodermoid ) , appears as a result of thechemotherapytreatment of a tumor . The drug most ofttimes tortuous 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 convinced data rise up .
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 . patient with localized scleroderma may train spinocellular epithelioma , as well as those suffer from Nathan Birnbaum or chronic radiodermatitis with cerebral tissue . Immunosuppressive therapy is an additional risk ( a patient who presented a fibrotic plaque of morphea and was do by with Imuran , spring up a squamous cubicle carcinoma in the scar surface area ) . Azathioprine can help the development of several tumors such as spinocell and basal mobile phone , lung , and kidney .
The literature distinguish morphea colligate to breast cancer and its intervention with radiation with supervoltage . Both IL4 and neoplasm maturation cistron activate the fibroblasts . foreplay of fibroblasts with increased collagen yield after the radiation may be due to the increase of the tumour growth factor and through the foreplay of fibroblast , which bring forth reactive atomic number 8 species . Many tumors let in breast cancer and lymphoreticular neoplasms are associated with stromal fibrosis .
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