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FRI0621 Prevalence of different orbital anatomic structures affection in igg4-related ophthalmic disease: single center experience
  1. V Vasilyev1,
  2. E Sokol1,
  3. T Safonova2,
  4. S Palshina1,
  5. N Kokosadze3,
  6. A Kovrigina4
  1. 1Nasonova Research Institute of Rheumatology, Moscow, Russia
  2. 2Research Institute of Ophthalmic diseases
  3. 3N.N. Blochin Russian Cancer Research Center
  4. 4Hematology Research Center, Moscow, Russian Federation


Background According to 2009 Japanese nationwide survey of IgG4-related disease (IgG4-RD), orbit is the leading site of affection [1]. However diagnostic criteria and nomenclature of IgG4-RD were developed a few years later [2,3].

Objectives To evaluate the peculiarities of clinical, laboratory, histological and immunohistochemical presentation of IgG4-related ophthalmic disease (IgG4-ROD).

Methods During 2004 – 2016, 82 patients were diagnosed with IgG4-RD 53 of whom had IgG4-ROD (men – 17, women - 36). The diagnoses of IgG4-RD and IgG4-ROD were established using comprehensive diagnostic criteria [2,3]. In all patients full clinical, ophtalmological, dental and serological (rheumatoid factor, C-reactive protein, IgG, IgG4, IgM, IgA, ANA, anti-Ro/La, C3/C4 complement) examination was carried. In all cases diagnosis was verified pathomorphologicaly with immunohystochemical staining (anti-CD 138, CD 68, IgG, IgG4, κ-chain, λ-chain), but only in 43 patients the diagnosis of IgG4-RD was established on orbital tissues biopsy. Some patients at baseline were tested on B-cell clonality in tissue (on frozen tissue section or paraffin embedded) by PCR analysis of immunoglobulin V-D-J genes heavy chain rearrangements (FR1, FR2, FR3); monoclonal secretion was tested in serum protein electrophoresis.

Results 40 patients with IgG4-ROD had orbital lesions at the onset of the disease and 13 developed them after 2 - 9 years from the onset. The frequency of different orbital anatomic structures involvement see in table 1. 15 (28%) patients had isolated orbital lesions while 38 (72%) had systemic IgG4-RD with simultaneous involvement of 2 - 7 other organs. 6 (14%) patients had unilateral orbital lesions. 23 (53%) patients with IgG4-ROD had associated IgG4-related sialoadenitis and nasal lesions. Most common pathomorphological features of IgG4-ROD see in table 2. The most common laboratory features of IgG4-ROD were elevation of: total serum IgG (44%), serum IgG4 (88%), serum IgE (61%) and monoclonal serum secretion (23%).

Conclusions In our cohort of IgG4-RD patients orbit is the leading site of the disease (64.5% of patients) and the disease onset (75% of patients). The majority of patients have bilateral orbital lesions (89%) and systemic course of the disease with other organ involvement (72%). Most often affected orbital anatomicstructures are lacrimal glands, extraocular muscles, retrobulbar infiltration with optic nerve thickening and fibroinflammatory lesions of eyelids. MALT-lymphoma of lacrimal gland and local AL-amyloidosis can complicate the long history of IgG4-ROD. Monoclonal serum secretion and B-cell clonality in the tissue in 23% of patients can potentially act as a background for lymphoma formation.


  1. Uchida K, Masamune A, Shimosegawa T et al. Prevalence of IgG4-related disease in Japan based on nationwide survey in 2009. IntJ.Rheumatol. 2012;2012:358371.

  2. Umehara H, Okazaki K, Masaki Y et al. A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details. ModRheumatol. 2012; 22: 1–14.

  3. Stone JH, Khosroshahi A, Deshpande V et al. Recommendations for the Nomenclature of IgG4-Related Disease and Its Individual Organ System Manifestations. Arth.Rheum. 2012; 64:3061–3067.


Disclosure of Interest None declared

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