Paraneoplastic systemic sclerosis: About 3 cases and review of literature

Abstract

Objective: Association between cancer and systemic sclerosis (SSc) has been described. However, paraneoplastic SSc is not well known. The aim of this article is to describe cases of paraneoplastic systemic sclerosis and to compare them to other cases of the literature, to find characteristics that can make suspect a paraneoplastic mechanism when SSc is diagnosed.

Methods: We retrospectively analyzed patients, in our department who, over the last 15 years, presented with Raynaud's phenomenon with a diagnosis of SSc (including cancer during the period of SSc). Treatment of cancer had to be concomitted with an improvement of sclerosis and/or negativation of antinuclear antibodies. Review analysis of other cases was made with Pubmed.

Results: Three patients responded to the criteria. Two of them had an ovarian cancer with peritoneal carcinomatosis and a concomitted SSc. One had a colon cancer with an ovarian metastasis diagnosed 1 year before SSc. None of them had anti-ENA antibody. One had a normal capillaroscopy. After treatment of cancer, all of them had an improvement of sclerosis and a negativation of antinuclear antibodies.

Conclusion: We believe that physicians should be aware of paraneoplastic SSc when presentation of SSc is atypical: no anti-ENA (anti-SCl70 or anti-centromere) or normal capillaroscopy, in particular gynecologic cancer in women.

Keywords: Cancer; Paraneoplastic; Paranéoplasique; Sclérodermie systémique; Systemic sclerosis.