Immunoglobulin G4-related sclerosing disease: experience with this novel entity in a local hospital

Abstract

Objective: To review the site of involvement, clinical presentation, and treatment outcome of patients having immunoglobulin G4-related sclerosing disease in a local regional hospital.

Design: Retrospective case series.

Setting: Pamela Youde Nethersole Eastern Hospital, Hong Kong.

Patients: All patients with a diagnosis of immunoglobulin G4-related sclerosing disease in the hospital diagnosed in the period from April 2008 to March 2010.

Results: A total of 12 patients with involvement of various organs were identified. There was a male predominance (male-to-female ratio=5:1). The mean age at diagnosis was 65 years. The salivary glands, biliary tract, pancreas, and cervical lymph nodes were the commonest involved sites. The immunoglobulin G4 level was elevated in 83% of the patients. Patients usually appeared to respond well to steroid treatment.

Conclusion: Immunoglobulin G4-related sclerosing disease is a systemic disease and can involve various systems.