Clinical aspects
IgG4-related disease: features and treatment response in a multi-ethnic cohort in Singapore
W. Fong1, I. Liew2, D. Tan3, K.H. Lim4, A. Low5, Y.Y. Leung6
- Department of Rheumatology and Immunology, Singapore General Hospital; Duke-NUS Medical School, Singapore; and Department of Medicine, Yong Loo Lin School of Medicine, National University of Singapore. warren.fong.w.s@singhealth.com.sg
- Department of Rheumatology and Immunology, Singapore General Hospital, Singapore.
- Duke-NUS Medical School, Singapore; Department of Medicine, Yong Loo Lin School of Medicine, National University of Singapore; Department of Gastroenterology and Hepatology, Singapore General Hospital, Singapore.
- Department of Pathology, Singapore General Hospital, Singapore.
- Department of Diagnostic Radiology, Singapore General Hospital, Singapore.
- Department of Rheumatology and Immunology, Singapore General Hospital; and Duke-NUS Medical School, Singapore.
CER10453
2018 Vol.36, N°3 ,Suppl.112
PI 0089, PF 0093
Clinical aspects
Free to view
(click on article PDF icon to read the article)
PMID: 29846168 [PubMed]
Received: 30/03/2017
Accepted : 27/11/2017
In Press: 24/05/2018
Published: 13/08/2018
Abstract
OBJECTIVES:
To describe the features and treatment outcomes of IgG4-RD in multi-ethnic patients in Singapore.
METHODS:
Retrospective study was performed on IgG4-RD patients identified from patient databases in a tertiary hospital.
RESULTS:
Fourty-two patients (76% male) were included; 79% fulfilled the 2011 comprehensive diagnostic criteria for IgG4-RD for definite IgG4-RD. 81% were Chinese and 19% were Malays. Common initial manifestations included jaundice (52%), abdominal pain (36%) and swollen salivary glands (26%). Only 36% had a history of allergy. 83% had ≥ 1 organ involvement. Erythrocyte sedimentation rate, immunoglobulin E, IgG2 and IgG4 levels were elevated in 84%, 100%, 70% and 44% of patients, respectively. The most common histopathological feature was >10 IgG4+ cells per high power field (66%). 94% (34/36) of patients were treated with moderate to high doses of glucocorticoids, including 17 patients with combination immunosuppressants. Of these, all patients responded to therapy by 3 months. With a median (range) follow-up of 4.1 (0.4-13.8) years, 69% (25/36) needed low dose of glucocorticoids to maintain disease remission. Twenty-six per cent had relapse of disease, of which 82% had disease recurrence in the same organs.
CONCLUSIONS:
Pancreatitis, lymphoadenopathy and cholangitis were the commonest manifestations in Asians with IgG4-RD. All patients responded to glucocorticoid therapy by 3 months, two-thirds required maintenance therapy with glucocorticoids, and one-quarter developed relapse of disease.
