EULAR 2016 | Daily Highlights
CHARACTERISTICS OF PRIMARY SJÖGREN’S SYNDROME PATIENTS WITH IGG4-POSITIVE PLASMA CELLS IN LABIAL SALIVARY GLANDSAbstract: OP0185
Authors: C. Liu1, H. Zhang1,*, G. Yao1, W. Chen1, L. Sun1
Co Authors: 1Department of Rheumatology and Immunology, The Affiliated Drum Tower Hospital of Nanjing University Medical School, Nanjing, China
Primary Sjögren’s syndrome (pSS) is a systemic autoimmune disease affecting predominantly exocrine glands such as salivary and lacrimal glands. Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a new clinical concept of multi-organ diseases, which is characterized pathologically by the infiltration of IgG4-bearing plasma cells into involved organs. However, some pSS patients showed infiltration of IgG4+ plasma cells in exocrine glands.
We aim to investigate the characteristics of primary Sjögren’s syndrome (pSS) patients with IgG4+ plasma cells in labial salivary glands (LSGs).
336 patients with pSS were enrolled. The LSG tissues from pSS patients were stained with monoclonal antibodies to IgG4 and IgG?Patients were divided into three subgroups according to histopathologic infiltration degree of IgG4+ plasma cells. The clinical and serological characteristics of patients were compared.
336 patients were divided into three groups according to infiltration degree of IgG4+ plasma cells: slight-IgG4 group (n=194), moderate-IgG4 group (n=106) and high-IgG4 group (n=36). The results showed that the higher IgG4+ plasma cells expression, the lower positive rates of serum anti-SSA, anti-SSB, anti-nuclear antibodies (ANA) and rheumatoid factor (RF). The comparison of extraglandular manifestations of three groups indicated that patients in high-IgG4 expression group showed a highest frequency of interstitial lung disease (30.6%) and tubulointerstitial nephritis (13.9%), whereas a lowest frequency of leucopaenia (13.9%), thrombocytopaenia (11.1%) and abnormal thyroidal function (0%).
The results showed that a considerable proportion of pSS patients could manifest abundant IgG4+ plasma cells infiltration in LSGs. These patients have unique laboratory and clinical characteristics. These findings may help further understand pSS, IgG4-RD, and the role of IgG4+ plasma cells in autoimmune diseases.
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Disclosure of Interest
Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) are a relatively new entity with potential multiorgan involvement mediated by infiltration of IgG4-bearing plasma cells. For Sjögren`s-Syndrome, IgG4-RD can be an important differential diagnosis, as swelling of the parotid gland can be a manifestation of both diseases. Current guidelines carefully differentiate the histological findings. For example, for a histological definition of IgG4-RD, large numbers of IgG4-bearing plasma cells are required. The current study shows that the labial salivary glands in Sjögren`s Syndrome can contain varying degrees of IgG4-bearing plasma cells. Remarkably, high numbers of IgG4-bearing plasma cells as in IgG4-RD were seen in up to 10% of patients fulfilling Sjögren`s classification criteria. These data indicate that the two disease likely overlap or that there is a continuum with cases in between the two entities.
Prof. Dr. Oliver Distler
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