A 64-year-old man was diagnosed with MPO-ANCA-related systemic vasculitis in March, 2004, because of multiple mononeuropathies, an interstitial pneumonia, a rapidly-progressive glomerulonephritis which was demonstrated by a biopsied-specimen from kidney. High dose intraveneous methylprednisolne bolus-therapy following an oral prednisolone were transiently effective. In August, 2004, the serum level of MPO-ANCA elevated again, and a macular purpura appeared in both arms. A hundred mg/day of oral cyclophosphamide was added to the prednisolone. In February, 2005, he was re-admitted to our hospital because of muscle weakness of extremities mainly in the left arm and leg. Laboratory examination showed a pancytopenia, high serum levels of ferritin (more than 3000μg/ml) and of MPO-ANCA. Bone marrow-aspirated smear showed a number of hemophagocytosis by macrophages. His general status worsened with continuous pyrexia and a decreased level of consciousness. He died in April, 2005, in spite of the treatments including several antibiotics and anti-fungal agents. An abscess formation in the right frontal lobe demonstrated by the autopsy seemed to be responsible for his final outcome. Histologically, the narrowing of lumen and thickening of small vessels in several organs such as kidneys and lungs were observed.
In this case, the serum levels of cytokines such as INF-γ, TNF-α, which play an important role for the pathogenesis of hemophagocytic syndrome were undetectable. On the other hand, the serum levels of IL-6, IL-10 and soluble IL-2 receptor were high. These cytokine profiles were compatible with those in compensatory anti-inflammatory response syndrome (CARS) which reflects a cachexic condition but not systemic vasculitis nor hemophagocytic syndrome.
