Systemic autoimmune rheumatic diseases as paraneoplastic phenomena: 3 illustrative case reports and narrative review of the literature

Abstract

ABSTRACT Objectives Systemic autoimmune rheumatic diseases can occur as paraneoplastic phenomena in the context of underlying malignancies. We present three illustrative clinical cases and a narrative literature review focusing on systemic sclerosis, dermatomyositis and palmar fasciitis and polyarthritis syndrome. Methods Medical data of three patients from the University Hospitals Leuven were retrospectively and anonymously obtained and reviewed. A narrative review was performed, searching the databases of PubMed, Embase and Cochrane Library. Results Systemic sclerosis, dermatomyositis and palmar fasciitis and polyarthritis syndrome are systemic autoimmune rheumatic diseases that can present as paraneoplastic phenomena. Systemic autoimmune rheumatic diseases are often associated with the presence of specific autoantibodies, some associated with a high likelihood of underlying malignancy. The presence of anti-ribonucleic acid polymerase III antibodies and anti-transcription intermediary factor 1 gamma antibodies indicates an increased risk of underlying cancer in systemic sclerosis and dermatomyositis, respectively. Individual patient prognosis can be improved through early detection of underlying malignancy, hence the importance of adequate cancer screening. Conclusion Some systemic autoimmune rheumatic diseases can appear as paraneoplastic phenomena, whereby the presence of specific autoantibodies is known to be related to the likelihood of underlying malignancy. We highlight the importance of clinician’s knowledge of these distinct features, as it facilitates early detection and treatment of underlying malignancy, thereby improving individual patient prognosis.