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Does IgG4 level at the time of diagnosis correlate with disease outcome in IgG4-Related disease?
- Source :
- Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.]. 19(1)
- Publication Year :
- 2018
-
Abstract
- Background/Objectives: Serum IgG4 level is used as a diagnostic criterion for immunoglobulin G4-related disease (IgG4-RD) but whether it predicts disease progression is unclear. Aim of the study was to investigate if serum IgG4 level at the time of diagnosis correlates with disease outcome. Methods Patients with a definitive diagnosis of IgG4-RD were included in this study. They were divided into two groups – Group 1 : Elevated serum IgG4 at diagnosis and Group 2: Normal serum IgG4 at diagnosis. Outcome parameters including multiple organ involvement, exocrine and endocrine dysfunction, relapse and mortality were compared. Data was subanalysed for outcomes on 2 levels of serum IgG4 cut-off – A: The upper limit of normal (ULN) and B: Twice the ULN. Results Of 47 patients, 31 (66%) patients had elevated serum IgG4 at diagnosis. There was no statistically significant difference between the two groups in any of the outcome parameters. Data analysed with the serum IgG4 levels > ULN showed no difference between the 2 groups for any of the outcome parameters. However, when the serum IgG4 cut-off was set to twice the ULN, there was a significantly higher rate of disease relapse (42.9% vs 11.5%, p = 0.02) and pancreatic exocrine insufficiency (PEI) (76.2% vs 42.3%, p = 0.041). Conclusion Raised serum IgG4 greater than two times the ULN was significantly associated with disease relapse and PEI in patients with IgG4-RD. Larger multicentre studies with longer follow-up are required to corroborate these findings and define the role and cut-off value of serum IgG4 in outcomes of IgG4-RD.
- Subjects :
- Adult
Male
medicine.medical_specialty
Disease outcome
Endocrinology, Diabetes and Metabolism
Disease
Gastroenterology
Elevated serum
03 medical and health sciences
0302 clinical medicine
Internal medicine
parasitic diseases
medicine
Endocrine system
Humans
skin and connective tissue diseases
Autoimmune pancreatitis
Aged
Aged, 80 and over
integumentary system
Hepatology
biology
business.industry
fungi
Pancreatic Diseases
Middle Aged
medicine.disease
030220 oncology & carcinogenesis
Immunoglobulin G
biology.protein
030211 gastroenterology & hepatology
IgG4-related disease
Rituximab
Female
Immunoglobulin G4-Related Disease
Antibody
business
medicine.drug
Subjects
Details
- ISSN :
- 14243911
- Volume :
- 19
- Issue :
- 1
- Database :
- OpenAIRE
- Journal :
- Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.]
- Accession number :
- edsair.doi.dedup.....432650c3cdee9da88fe09a2d1947c804