J Asthma Allergy Clin Immunol.  2002 Sep;22(3):608-613.

A case of successful treatment of adult onset Still's disease with high dose immunoglobulin therapy

• Seo KW
• Kim BC
• Park JH
• Jeong ID
• Lee JS
• Park JH
• Choi SW

Abstract

Adult onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. It is characterized by spiking fever, evanescent skin rash, arthritis, and various systemic manifestations. Liver involvement is common in AOSD, with up to three-quarters of the patients exhibiting elevation of hepatic enzymes or hepatomegaly. The treatment of AOSD is depends on the severity of the disease or the organ involvement. Numerous drugs have been used including nonsteroidal antiinflammatory drugs (NSAIDs), corticosteroid, and immunosuppressive agents. However, the use of intravenous immunoglobulin (IVIG) was rarely reported, and its efficacy is still controversial. We describe a patient with AOSD who developed acute severe hepatitis refractory to corti costeroid, but it was successfully treated with IVIG. An 18-year-old woman developed malaise, jaundice and nausea. One month ago, she was diagnosed as AOSD and treated with prednisolone and naproxen. Laboratory tests demonstrated marked increase of transaminase, bilirubin and ferritin. Etiologic evaluation for viral hepatitis and other causes showed negative result. In spite of methylprednisolone pulse therapy, hepatitis aggravated rapidly. After IVIG (0.4 g/ kg/day) was administered for 5 days, her systemic symptoms and hepatitis were much improved. We considered that IVIG may be a potential alternative for the treatment of AOSD, particularly refractory to conventional therapy.

Keyword

Adult onset Still's disease; Intravenous immunoglobulin

MeSH Terms

Adolescent
Adult*
Arthritis
Bilirubin
Exanthema
Female
Ferritins
Fever
Hepatitis
Hepatomegaly
Humans
Immunization, Passive*
Immunoglobulins*
Immunoglobulins, Intravenous
Immunosuppressive Agents
Jaundice
Liver
Methylprednisolone
Naproxen
Nausea
Prednisolone
Still's Disease, Adult-Onset*
Bilirubin
Ferritins
Immunoglobulins
Immunoglobulins, Intravenous
Immunosuppressive Agents
Methylprednisolone
Naproxen
Prednisolone