Systemic lupus erythematosus complicated by cytomegalovirus-induced hemophagocytic syndrome and pneumonia

Abstract

  A 58-year-old female with systemic lupus erythematosus (SLE) and anti-phospholipid syndrome (APS) was referred and admitted to our hospital because of fever and pancytopenia. She had been taking small dose of prednisolone and azathiprine since 2003 with stable SLE and APS. Two weeks before the admission, she developed fever and common cold-like symptoms and was admitted to a hospital. Antibiotics were ineffective and thrombocytopenia manifested. Under a diagnosis of progressive SLE, bolus methylprednisolone was administrated. However, she became pancytopenic and was transferred to our hospital. A bone marrow aspirate showed the hemophagocytosis and chest CT scanning revealed interstitial pneumonia. Cytomegalovirus (CMV) antigenemia test gave a positive result. A diagnosis of CMV-induced hemophagocytic syndrome (HPS) and CMV pneumonia was made. Gancyclovir resolved the pancytopenia, pneumonia, and fever. There have been only 3 reported cases of CMV-related HPS in collagen disease.