A case of successful treatment of adult onset Still's disease with high dose immunoglobulin therapy.
- Author:
Kwang Won SEO
1
;
Byung Chul KIM
;
Jee Hyun PARK
;
In Du JEONG
;
Jong Soo LEE
;
Jae Hoo PARK
;
Seung Won CHOI
Author Information
1. Department of Internal Medicine, Ulsan University Hospital, Ulsan University College of Medicine, Ulsan, Korea. choisw@uuh.ulsan.kr
- Publication Type:Case Report
- Keywords:
Adult onset Still's disease;
Intravenous immunoglobulin
- MeSH:
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*
- From:Journal of Asthma, Allergy and Clinical Immunology
2002;22(3):608-613
- CountryRepublic of Korea
- Language:Korean
-
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.
