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Abstract: Myelodysplastic syndromes (MDS) can be associated with systemic or autoimmune diseases. Vasculitides (leucocytoclastic, periarteritis nodosa, micropolyangeitis, Wegener''s granulomatosis), relapsing polychondritis, and Sweet''s syndrome are the most commonly reported. Refractory anemia with excess of blasts (RAEB), transformed RAEB evolving to an acute leukemia, and chronic myelomonocytic leukemia (CMML) are preferentially associated with these vasculitides or systemic diseases. Corticosteroids are generally effective. Immunosuppressive drugs expose these patients to infectious complications and increase the risk of transformation into acute leukemia. Occurrence of relapsing polychondritis in a patient older than 60years is associated with a myelodysplastic syndrome in 40% of the cases. Sweet''s syndrome is associated in 10% of the cases with acute myeloid leukemia and MDS. Polyarthritis or oligoarthritis with systemic manifestations that include fever, skin rash, and more rarely serositis or haemolytic anemia can occur contemporarily to a MDS. Behçet''s disease with intestinal involvement has been reported in patients presenting with trisomy 8 associated MDS. Pathogenic mechanisms underlying the association between MDS and autoimmune or systemic disorders remain to be elucidated. [Copyright &y& Elsevier]