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THU0405 Characterization of the group of patients with IgG4-related systemic disease in russian population
  1. S. Sedyshev1,
  2. V. Vasilyev1,
  3. A. Kovrigina2
  1. 1Research Institute of Rheumatology of Rams
  2. 2Hematology Research Centre, Moscow, Russian Federation


Objectives to describe a group ofpatients with IgG4-related systemic disease (IgG4-RSD), followed up in the Institute of Rheumatology, Moscow, Russia

Methods Between 2008 and 2011 25 patients were diagnosed with IgG4-RSD. Of these, male - 12, female - 13, mean age - 43.6 years (22 - 71). The diagnosis was based on clinical features, evaluation of serum IgG4 level (>1,35 g/l), histological and immunomorphological examination of affected tissue specimens using IgG, IgG4, CD20, CD138 and κ/λ stainig.

Results As part of the IgG4-RSD 32% (n=8) of patients were diagnosed with multifocal fibrosclerosis, IgG4-associated sclerosing orbit pseudotumor (sclerosing dacryoadenitis, solitary fibrotic mass, fibroplasia of extraocular muscles and orbit fat)–28% (n=7), retroperitoneal fibrosis (Ormond’s disease) with periaortic mass, often compressing ureter–16% (n=4), Miculicz’s disease–8% (n=2), Küttner’s tumor–4% (n=1), autoimmune pancreatitis (AIP) (type I), mediastinal fibrosclerosis and juvenile xanthogranuloma of the orbit–by 4% (n=1) respectively. Among affected areas were orbit (n=17), salivary (parotid and submandibular) glands (n=8), retroperitoneum (n=5), sinuses (n=3), mediastinum (n=2), pancreas (n=2), lymph nodes (n=1), biliary ducts (n=1). Serum IgG4 was elevated >1,35g/l in 65% of patients (1,5–16,5 g/l). IgG4-staining of plasma cells was diagnostic in 100% of cases with maximum IgG4/IgG ratio – 50%. All cells were polyclonal. Highest levels of IgG4 were detected in cases of Miculicz’s disease. Histology showed the absence of fibroplasia, MALT-tissue formation with no lymphoepithelial lesions and the abundance of IgG4-expressing plasma cells.

Conclusions In Russian IgG4-RSD cohort multifocal forms prevail predominantly with orbital affection. Relatively few patients with AIP were found.

Disclosure of Interest None Declared

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