Autoimmune Syndromes Presenting as a Paraneoplastic Manifestation of Myelodysplastic Syndromes: Clinical Features, Course, Treatment and Outcome

Autoimmune manifestations (AIM) are reported in up to 10-30% of myelodysplastic syndromes (MDS) patients; this association is not well defined. We present herein a retrospective chart review of single center MDS patients for AIM, a case discussion and a literature review. Of 252 MDS patients examined, 11 (4.4%) had AIM around MDS diagnosis. International Prognostic Scoring System scores were: low or intermediate (int)-1 (n = 7); int-2 or high (n = 4). AIM were: culture negative sepsis (n = 7); inflammatory arthritis (n = 3); vasculitis (n = 4); sweats; pericarditis; polymyalgia rheumatica (n = 2 each); mouth ulcers; pulmonary infiltrates; suspicion for Behcet’s; polychondritis and undifferentiated (n = 1 each). AIM treatment and outcome were: prednisone +/- steroid sparing agents, n = 8, ongoing symptoms in 5; azacitidine (n = 3), 2 resolved; and observation, n = 1, ongoing symptoms. At a median follow up of 13 months, seven patients are alive. In summary, 4.4% of MDS patients presented with concomitant AIM. MDS should remain on the differential diagnosis of patients with inflammatory symptoms.