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AB0565 Routine Investigations for Circulating Igg4 and/or Igg4+ Salivary Gland Histopathology Fail to Reveal Undiagnosed Cases of Igg4 Related Disease in Sicca Patients not Fulfilling the Diagnosis of Sjogren's Syndrome
  1. E. Astorri1,
  2. C. Croia1,
  3. G. Picarelli2,
  4. N. Sutcliffe3,
  5. A. Tappuni4,
  6. M. Bombardieri1,
  7. R. Priori2
  1. 1Experimental Medicine and Rheumatology, QMUL, London, United Kingdom
  2. 2Department of Internal Medicine and Medical Specialties, Rheumatology, Sapienza University of Rome, Rome, Italy
  3. 3Department of Rheumatology, Barts Health NHS Trust and Queen Mary University of London
  4. 4Institute of Dentistry, Barts and The London School of Medicine and Dentistry, Queen Mary University of London, London, United Kingdom

Abstract

Background Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is an immune-mediated condition characterized by dense IgG4+ plasmacytic infiltration of diverse organs, fibrosis, tumefactive lesions and eosinophilia. Salivary and lacrimal glands are frequent targets that can present with enlargement and severe hypofunction. The diagnosis of IgG4-RD is challenging because of its serological and histopathological overlaps with other diseases, in particular, a differential diagnosis with Sjogren's syndrome (SS) can sometimes be achieved only on the basis of a labial salivary gland biopsy.

Objectives In this study we evaluated whether some of the patients with sicca symptoms not fulfilling SS classification criteria, were in fact affected by IgG4-RD.

Methods A total of 81 Sicca and 50 SS patients were retrospectively evaluated for IgG4-RD-associated signs and symptoms. Additionally, IgG4 and total-IgG staining was performed on lip gland biopsies from all patients while IgG4 serum levels were evaluated on the Italian cohort.

Results Only 2 of the Sicca patients had positive IgG4 staining but did not fulfil the diagnostic criteria. The serum analysis found 3 sicca patients with raised IgG4 (≥1.35 g/L) but none had a concomitant positive histology nor IgG4-RD related symptoms.

Conclusions In our cohort of patients with sicca symptoms not fulfilling SS criteria no case could be accounted as an undiagnosed IgG4-RD based upon IgG4 staining on labial salivary gland biopsies. This study also suggests that in the absence of a clear clinical suspicious of an IgG4-RD, a routine IgG4 staining on labial salivary gland biopsies is not of clinical utility.

References

  1. Yamamoto M, Harada S, Ohara M, Suzuki C, Naishiro Y, Yamamoto H, et al. Clinical and pathological differences between Mikulicz's disease and Sjogren's syndrome. Rheumatology (Oxford). 2005 Feb;44(2):227-34. PubMed PMID: 15509627.

  2. Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol. 2012 Feb;22(1):21-30. PubMed PMID: 22218969.

Disclosure of Interest None declared

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