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Abstract Number: 1239

Is Lymphocytic Sialadenitis IgG4-Related?

Nathalie Shehwaro1, Murielle Hourseau2, Thomas Papo3 and Karim Sacre4, 1Internal Medicine, University Paris-7, APHP, Bichat Hospital, Paris, France, 2Pathology, University Paris-7, APHP, Bichat Hospital, Paris, France, 3Internal Medicine, Bichat Hospital, Paris, Paris, France, 4Internal Medicine, University Paris-7, INSERM U699, APHP, Bichat Hospital, Paris, France

Meeting: 2014 ACR/ARHP Annual Meeting

Keywords: IgG4 Related Disease, Sjogren's syndrome and sialoadenitis

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Session Information

Title: Miscellaneous Rheumatic and Inflammatory Diseases

Session Type: Abstract Submissions (ACR)

Background/Purpose: To assess the prevalence of IgG4-related disease among patients with lymphocytic sialadenitis on labial salivary gland biopsy.

Methods: All labial salivary gland biopsies (LSGB) performed in a French University Hospital Center over a one-year period (2012) were retrospectively screened. IgG4 immunostaining was performed on all LSGB showing lymphocytic sialadenitis defined by a Chisholm score ≥3. Histopathological criteria for IgG4-related disease according to international criteria and final diagnosis associated with lymphocytic sialadenitis were analyzed in all cases. 

Results: Three hundred and fifty patients had a labial salivary gland biopsy (LSGB). Among them, 79 (23%) had a lymphocytic sialadenitis. Mean age was of 55.5±15.7 years old. Female/Male Sex ratio was 3.2/1. LSGB was performed because of sicca symptoms in most cases (48/79, 60.8%). Only one (1/79, 1.3%) LSGB showed histopathological features of IgG4-related disease in a patient who otherwise displayed obvious extra-salivary features of IgG4-related disease. Overall, the diagnoses associated with lymphocytic sialadenitis were Sjögren’s syndrome (29/79, 36.7%), other autoimmune disorders (besides Sjogren’s syndrome) (17/79, 21.5%), idiopathic pulmonary fibrosis (5/79, 6.3%), sarcoïdosis (3/79, 3.8%), B-cell hemopathy (3/79, 3.8%), hepatitis C infection (2/79, 2.5%), unclassified arthritis (1/79, 1.3%), idiopathic uveitis (1/79, 1.3%), multiple sclerosis (1/79, 1.3%) and tuberculosis (1/79, 1.3%). In 15 cases (19%), no diagnosis could be obtained despite extensive work-up. Of note, IgG4 staining was negative in all patients with unexplained lymphocytic sialadenitis.

Conclusion: The prevalence of IgG4-related disease among patients with lymphocytic sialadenitis on LSGB is very low. Systematic IgG4 immunostaining has no diagnostic value in patients with lymphocytic sialadenitis. On the other hand, lip biopsy, by being simple and safe, can be useful for a definite diagnosis when IgG4-related disease is suspected.


Disclosure:

N. Shehwaro,
None;

M. Hourseau,
None;

T. Papo,
None;

K. Sacre,
None.

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