Abstract
Background
IgG4-related disease (IgG4-RD) is a recently recognized condition defined by characteristic
histopathologic findings in affected organs. Serum IgG4 concentration is often but
not always elevated. The sensitivity and specificity of serum IgG4 vary greatly across
studies and has been anecdotally associated to ethnicity. Our study was conducted
to investigate the difference in serum IgG4 levels between Asian and non-Asian patients
with IgG4-RD.
Methods
This is a single-center retrospective study of 26 Asian and 10 non-Asian patients
with histologically confirmed IgG4-RD. Serum IgG4 levels, clinical features and other
laboratory findings were compared between the 2 groups, 31 Asian and 11 non-Asian
patients with non-IgG4-RD rheumatic diseases were randomly identified to evaluate
test characteristics of serum IgG4 measurement.
Results
Median serum IgG4 at time of diagnosis was significantly higher in Asian (median =
11.2 g/L, interquartile range: 4.6-19.7) than non-Asian patients (median = 2.9 g/L, interquartile range: 0.7-5.4, P = 0.0094), as well as the median serum IgG and total protein. Asian patients had
more eosinophilia and polyclonal hypergammaglobulinemia than non-Asian patients (P = 0.016 and 0.001, respectively). Test sensitivity was higher in Asian (96%) than
non-Asian patients (67%), whereas test specificity was higher in non-Asian patients
(91% versus 71%).
Conclusion
Asian patients with IgG4-RD have more exuberant serum IgG4, IgG and polyclonal hypergammaglobulinemia
than non-Asian patients; the mechanism of this difference requires further study.
These findings have significant clinical importance and must be accounted for in the
diagnostic workup of patients in multiethnic settings.
Key Indexing Terms
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to The American Journal of the Medical SciencesAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- A new clinicopathological entity of IgG4-related autoimmune disease.J Gastroenterol. 2003; 38: 982-984
- IgG4-related disease.N Engl J Med. 2012; 366: 539-551
- IgG4-related disease with hypergammaglobulinemic hyperviscosity and retinopathy.Eur J Haematol. 2013; 90: 250-256
- Treatment of relapsing autoimmune pancreatitis with immunomodulators and rituximab: the Mayo Clinic experience.Gut. 2013; 62: 1607-1615
- The clinical course of patients with IgG4-related kidney disease.Kidney Int. 2013; 84: 826-833
- Polyclonal hyperviscosity syndrome in IgG4-related disease and associated conditions.Clin Case Rep. 2015; 3: 217-226
- A young woman with episodic angioedema, papilledema, and eosinophilia.Am J Hematol. 2010; 85: 124-127
- IgG4-related disease: dataset of 235 consecutive patients.Medicine (Baltimore). 2015; 94: e680
- Clinical characteristics of immunoglobulin G4-related disease: a prospective study of 118 Chinese patients.Rheumatology (Oxford). 2015; 54: 1982-1990
- IgG4-related disease: clinical and laboratory features in one hundred twenty-five patients.Arthritis Rheumatol. 2015; 67: 2466-2475
- High serum IgG4 concentrations in patients with sclerosing pancreatitis.N Engl J Med. 2001; 344: 732-738
- IgG4-related disease in Italy: clinical features and outcomes of a large cohort of patients.Scand J Rheumatol. 2016; 45: 135-145
- Consensus statement on the pathology of IgG4-related disease.Mod Pathol. 2012; 25: 1181-1192
- Development of an IgG4-RD responder index.Int J Rheumatol. 2012; 2012: 259408
- The diagnostic utility of serum IgG4 concentrations in IgG4-related disease.Ann Rheum Dis. 2015; 74: 14-18
- Value of serum IgG4 in the diagnosis of IgG4-related disease and in differentiation from rheumatic diseases and other diseases.Mod Rheumatol. 2012; 22: 419-425
- Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011.Mod Rheumatol. 2012; 22: 21-30
- Is rituximab effective for IgG4-related disease in the long term? Experience of cases treated with rituximab for 4 years.Ann Rheum Dis. 2015; 74: e46
- IgG4-related disease and lymphocyte-variant hypereosinophilic syndrome: a comparative case series.Eur J Haematol. 2017; 98: 378-387
- Elevated serum IgG4 levels in diagnosis, treatment response, organ involvement, and relapse in a prospective IgG4-related disease UK cohort.Am J Gastroenterol. 2016; 111: 733-743
Article info
Publication history
Published online: September 08, 2017
Accepted:
August 21,
2017
Received in revised form:
August 2,
2017
Received:
April 20,
2017
Footnotes
☆Funding: This work was supported by the Hal Kettleson Hematology Research Fund.
☆☆The authors have no conflicts of interest to disclose.
Identification
Copyright
© 2017 Southern Society for Clinical Investigation. Published by All rights reserved.
