title: [Systemic lupus erythematosus with bilateral salivary gland swelling and clouding of consciousness accompanied by hemophagocytic syndrome--a study of serial determination of serum cytokines].
abstract: We investigated changes in various serum cytokines in a case of systemic lupus erythematosus (SLE) accompanied by hemophagocytic syndrome (HPS). The patient, a 15-year-old male, presented in December 1998 with bilateral salivary gland swelling and a history of fever continuing for more than 10 days. After admission, cerebellar ataxia and clouding of consciousness developed. Laboratory examinations revealed leukopenia, thrombocytopenia, high serum LDH and ferritin, hypercytokinemia, and prominent hemophagocytosis in the bone marrow. Given these findings and positive titers of antinuclear antibody, hypocomplementemia, proteinuria and pericarditis, a diagnosis of HPS with associated SLE was made. The patient was treated with high dose methylprednisolone followed by oral prednisolone and cyclosporine. The patient's clinical symptoms, abnormal blood and urine laboratory data consequently improved, and no recurrence of the symptoms has been documented. However, hemophagocytosis in bone marrow recurred with concomitantly increased serum levels of IL-6 and IL-1 beta. This case indicated that aberrant production of these inflammatory cytokines might be involved in HPS in autoimmune disease.
authors.affiliation: Department of Pediatrics, Kinki University School of Medicine.
authors.forename: Naohiko
authors.initials: N
authors.lastname: Moriguchi
authors.forename: Wakako
authors.initials: W
authors.lastname: Sato
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Miyata
authors.forename: Kazuo
authors.initials: K
authors.lastname: Yoshioka
mesh_terms: D000293:Adolescent; D002524:Cerebellar Ataxia / Q000209:etiology; D003244:Consciousness Disorders / Q000209:etiology; D016207:Cytokines / Q000097:blood*; D015616:Histiocytosis, Non-Langerhans-Cell / Q000097:blood / Q000209:etiology*; D006801:Humans; D007375:Interleukin-1 / Q000097:blood; D015850:Interleukin-6 / Q000097:blood; D008180:Lupus Erythematosus, Systemic / Q000097:blood* / Q000150:complications; D008297:Male; D012466:Salivary Gland Diseases / Q000209:etiology / Q000473:pathology*
chemical_list: D016207:Cytokines; D007375:Interleukin-1; D015850:Interleukin-6