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Abstract Number: 2199

Elevated IgG4 Serum Levels Among Primary Sjogren’s Syndrome Patients: Do They Unmask Underlying IgG4-Related Disease?

Clio P. Mavragani1, George Fragoulis2, Dimitra Rontogianni3, Maria Kanariou4 and Haralampos M. Moutsopoulos2, 1Department of Experimental Physiology, School of Medicine, University of Athens, Athens, Greece, Athens, Greece, 2Department of Pathophysiology, School of Medicine, University of Athens, Athens, Greece, 3Department of Pathology, Evangelismos General Hospital, Athens, Greece, 4Department of Immunology, "Aghia Sophia" Children's Hospital, Athens, Greece

Meeting: ACR/ARHP Annual Meeting 2012

Keywords:

Session Information

Title: Sjögren's Syndrome - Clinical

Session Type: Abstract Submissions (ACR)

Background/Purpose:

To determine IgG4 serum levels in a cohort of patients fulfilling the classification criteria for primary Sjogren’s syndrome (pSS) and to explore whether they associate with distinct clinical, serological and histopathological parameters.

Methods:

IgG4 levels were measured by nephelometry in sera from 140 consecutive pSS patients and 45 healthy controls of similar age and sex distribution. Immunohistochemical IgG4 analysis was performed on paraffin-embedded salivary gland tissues from patients with high and low IgG4 serum levels.

Results:

Raised IgG4 serum levels defined as higher than 135mg/dl were detected in 8 out of 81 pSS patients analyzed (9.9%) (“High-IgG4” group) and in none of controls (p=0.05). Compared to their counterparts with normal IgG4 serum levels, the “High-IgG4” subset is characterized by significantly increased prevalence of IgG4-related features such as autoimmune pancreatitis, cholangitis and interstitial nephritis (p=0.006), lower rates of ANA (p=0.05) and anti-Ro/SSA (p=0.05) positivity. Although not statistically significant, sicca features and anti-La/SSB positivity occurred less frequently in the “High-IgG4” group. Multivariate logistic regression analysis revealed interstitial nephritis (p=0.048), autoimmune cholangitis (p=0.012) or pancreatitis (p=0.027) and absence of ANA (p=0.005) as independent predictors of IgG4 serum levels. Positive staining for IgG4+ plasma cells was detected in one out of four available MSG biopsies in the “High-IgG4” group but in none of the control patients with normal IgG4 serum levels. Analysis of the whole group is in process.

Conclusion:

Raised IgG4 levels occur approximately in 10% of patients with pSS and characterize a subgroup with high prevalence of IgG4-related clinical and serological features. Whether these patients represent a distinct pSS subset or a misclassified IgG4-Related (IgG4-RD) disease group remains to be defined.

Disclosure:

C. P. Mavragani,
None;

G. Fragoulis,
None;

D. Rontogianni,
None;

M. Kanariou,
None;

H. M. Moutsopoulos,
None.

ACR Meeting Abstracts - https://acrabstracts.org/abstract/elevated-igg4-serum-levels-among-primary-sjogrens-syndrome-patients-do-they-unmask-underlying-igg4-related-disease/