DIFFICULTIES IN DIFFERENTIAL DIAGNOSIS OF SCLERODERMА-LIKE PARANEOPLASTIC SYNDROME AND SYSTEMIC SCLERODERMA AS A FACTOR OF CANCER RISK
DOI:
https://doi.org/10.11603/2415-8798.2018.3.9267Keywords:
cancer, scleroderma, paraneoplastic syndromeAbstract
Systemic sclerosis (SSc) is an autoimmune connective tissue disease of unknown origin, characterized by progressive fibrosis of the skin and internal organs. The characteristic clinical signs of SSc can mask oncological diseases, and the paraneoplastic process in turn is masked by symptoms of SSc.
The aim of the study – to demonstrate the differential diagnosis necessity between scleroderma-like paraneoplastic syndrome and SSc as a risk factor for oncology.
Materials and Methods. The review and analysis of scientific and medical literature data is carried out.
Results and Discussion. Immune reactions taking part in Ssc pathogenesis may contribute to cancer development; therefore patients with risk factors for this disease require observation for a neoplastic process. Patients with SSc and autoantibodies to RNA polymerase III have a significant risk of the cancer developing. The risk of carcinogenesis is highest in the first year after the diagnosis of SSc. On the other hand, the symptoms of SSc can be a mask of various types of cancer. Paraneoplastic scleroderma-like syndrome is not directly associated with the primary tumor and its metastases, but it is caused by complex secondary inflammatory-degenerative changes of systemic, local or distant nature, often occurring as manifestations of "overlap" of rheumatic and tumor diseases. It is assumed that cancer cells can induce the synthesis of a substance that initiates fibrosis of the skin and internal organs. The differentiation between the idiopathic form of SSc, scleroderma-like paraneoplastic syndrome and the development of cancer in the background of the SSc causes many difficulties. This paper presents an analysis of differential diagnostic procedures which were performed and led to the final diagnosis, mentions types of cancers cooccurring with Ssc and suggests a screening scheme for cancer development in patients with a diagnosis of Ssc.
Conclusions. The results of the literature sources analysis prove the urgency of in-depth study of the patient with the newly discovered scleroderma syndrome, especially in the elderly.
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