Autoimmune Disease as Harbinger of Myelodysplasia Save

Autoimmune manifestations (AIM) are reported in up to 30% of patients with myelodysplastic syndrome. A recent review identified 252 MDS patients from the medical literature and found that AIM was seen in 11 patients (4.4%), most occurring roughly at the same time of the MDS diagnosis.

The median age was 68 years and 9 of 11 had an adverse karyotype on immunogenetics. The severity/prognosis of these patients was either low or intermediate (n=7) or intermediate- high (n=4). The AIM diagnoses included culture negative sepsis (n=7); inflammatory arthritis (n=3); vasculitis (n=4); two each with pericarditis or polymyalgia rheumatic; and one each with mouth ulcers; pulmonary infiltrates; Behcet’s, polychondritis or undifferentiated (n=1 each).

With a median follow up of 13 months, seven patients are alive. Half were treated with prednisone +/- DMARDs, such as MTX, hydroxychloroquine, azathioprine, chlorambucil, gold or dapsone. Three patients resolved, 4 had partial improvement with therapy and 3 have ongoing symptoms. Previous reports suggested that AIM may adversely affect the outcome of MDS; such was not the case in this report.

In summary, 4.4% of MDS patients presented with concomitant AIM. MDS should remain on the differential diagnosis of patients with new undiagnosed inflammatory or autoimmune findings.