A 71 year-old-male was admitted to our hospital because of fever accompanied with leukopenia. He was initially treated with antibiotics, without success. He had worked as a stone mason since the age of 20 was diagnosed as having silicosis at the age of 30. A polyclonal increase of immunoglobulins and elevations of antinuclear factor, anti-DNA antibody, and rheumatoid factor were documented, but anti-topoisomerase I(anti-topo I) antibody and PR3- and MPO- antibeutrophil cytoplasmic antibodies (ANCAs) were negative. HLA-DQB1 was *0301/*0502. Administration of prednisolone was effective for ameliorating fever and leukopenia. These results suggested the immunological pathogenesis for these symptoms with silicosis by interections of silica, antigen presenting macrophages and T-cells through specific HLA, and stimulation of B-cells to produce autoantibodies.