Systemic juvenile idiopathic arthritis mimics multicentric Castleman's disease.
An 11-year-old girl presented with fever and a large cervical lymphadenopathy. Indicators of inflammation were remarkable: she had extremely high levels of serum interleukin-6 (IL-6) (398 pg/ml) in addition to hypergammaglobulinemia and hypoalbuminemia. Computed tomography (CT) revealed swollen systemic lymph nodes. Two weeks after the onset of symptoms she developed polyarthralgia. Biopsy of the cervical lymph node revealed massive infiltration of plasma cells without hyaline vascular changes. She was diagnosed with systemic juvenile idiopathic arthritis (JIA). The patient's symptoms and hypercytokinemia disappeared soon after corticosteroid treatment was started. This case demonstrates that overproduction of IL-6 is common to systemic JIA and multicentric Castleman's disease.
Osone
Shinya
S
Department of Pediatrics, Kyoto Prefectural University of Medicine, 465 Kajii-cho, Kamigyo-ku, 602-8566 Kyoto, Japan. shinn-o@koto.kpu-m.ac.jp
Morimoto
Akira
A
Tsutsui
Junna
J
Kano
Gen
G
Todo
Shinjiro
S
Sugimoto
Tohru
T
D001171:Arthritis, Juvenile / Q000175:diagnosis* / Q000188:drug therapy; D001707:Biopsy, Needle; D005871:Castleman Disease / Q000175:diagnosis* / Q000188:drug therapy; D002648:Child; D003937:Diagnosis, Differential; D004334:Drug Administration Schedule; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D008198:Lymph Nodes / Q000473:pathology*; D011239:Prednisolone / Q000008:administration & dosage; D018570:Risk Assessment; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
D011239:Prednisolone