MACROPHAGE ACTIVATION SYNDROME IN LUPUS P ATIENTS

A patient with systemic lupus erythematosus developed unexplained feve r, nonregenerative anemia, leukopenia, and elevations in serum triglyc eride and ferritin levels. Bone marrow studies established the diagnos is of macrophage activation syndrome with active hemophagocytosis. No infectious cause was found but pulmonary nocardiosis developed during the course of the disease. Intravenous gammaglobulin therapy was follo wed by a transient remission. Cyclophosphamide was given subsequently. In lupus patients, macrophage activation syndrome is exceedingly rare and has the same clinical, laboratory, and histologic features as tho se seen in patients with hemopathies, infections, or immune deficienci es. Investigations for an underlying infection are often negative, sug gesting that the macrophage activation syndrome is due to lupus-relate d immune changes. Treatment is not standardized and relapses are commo n. This diagnosis should be considered in lupus patients with febrile pancytopenia.