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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