• Epidemiology
    • Up to 30% of patients with MDS may have autoimmune manifestations (AIM)112-114
      • The association between MDS and autoimmune disorders is poorly understood
      • AIM may precede MDS diagnosis by up to many years or may present as a paraneoplastic manifestation of MDS
      • AIM may occur in all MDS risk groups
    • Compared to MDS patients without AIM, patients with AIM were younger, more frequently male, less frequently low IPSS risk, more frequently of adverse karyotype, and less frequently had RARS and RAEB2115
    • Progression to AML and overall survival was similar between MDS patients with and without AIM115
    • AIM may affect virtually every organ system and may be categorized as:
      • Acute systemic vasculitis
      • Chronic immune syndromes
      • Connective tissue disorders (e.g. SLE, relapsing polychondritis, Sjorgren’s syndrome, Raynaud’s syndrome, Behcet’s-like syndrome)
      • Immune-mediated cytopenias
      • Serological abnormalities (e.g. ANA, the lupus anticoagulant, direct antiglobulin test)
  • Management
    • AIM may respond to immunosuppressive medications such as prednisone or cyclosporine116
    • In higher risk MDS, there is some evidence that AIM may respond to azacitidine115,117
    • Data with lenalidomide are conflicting, with some reports suggesting AIM may be exacerbated by lenalidomide.118-123 However, a recent case series suggests improved survival in 6 patients with AIM treated with lenalidomide, compared to 9 patients not receiving lenalidomide (p=0.017) with response of AIM in 5 of 6 patients124