Article Text

THU0578 Spectrum of the diseases with orbital involvement in rheumatology: single-center study
  1. V Vasilyev1,
  2. V Gorodetskiy1,
  3. T Safonova2,
  4. N Probatova3,
  5. N Kokosadze3,
  6. A Pavlovskaya3,
  7. A Kovrigina4,
  8. I Gaiduk5
  1. 1Nasonova Research Institute of Rheumatology
  2. 2Research Institute of Ophthalmic diseases
  3. 3N.N. Blochin Russian Cancer Research Center
  4. 4Hematology Research Center
  5. 5A.I. Evdokimov Moscow State Dental University, Moscow, Russian Federation


Background It is assumed that in ophthalmological clinical practice lymphoproliferative disorders in patients with affection of different orbital organs and tissues constitute 25–55% and 21% of patients have IgG4-related ophthalmic disease [1,2].

Objectives To evaluate the most common conditions affecting the orbit in rheumatologic clinical practice.

Methods During 2004 – 2016 years 138 patients (male – 33, female - 105) with eyelid edema and/or tumefactive lesions in the orbit were examined in Nasonova Research Institute of Rheumatology. In all patients full clinical, ophthalmological, 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). Diagnosis was established on the basis of pathomorphological examination of different tissues: orbit – 79 patients, parotid salivary gland – 40, submandibular salivary gland – 14, nasal – 4, lymph nodes – 3 and other – 6. In 79 cases (54%) the diagnosis was established on the results of orbital biopsy.

Results Different non-neoplastic diseases were diagnosed in 108 patients (78.5%) and 30 had different conditions of hematological spectrum, including malignant conditions in 25 (18.2%) patients (see table 1). Some patients at baseline had simultaneous involvement of the major salivary glands (23 patients with IgG4-related disease, 35 with sarcoidosis, 12 with non-Hodgkin lymphomas and 2 with AL-amyloidosis). The most rare conditions affecting the orbit were Cogan's syndrome, relapsing polychondritis, Erdheim-Chester disease, NK/T-cell nasal lymphoma and calcifying aponeurotic fibroma.

Conclusions In rheumatologic practice in 78.5% of patients with orbital involvement different non-neoplastic conditions are diagnosed: IgG4-related ophthalmic disease (35.0%), granulematous lesions (29%). The most common hematological disorders in rheumatologic clinic are non-Hodgkin lymphomas (17.5%) and histiocytosis (3.5%).


  1. Japanese study group of IgG4-related ophthalmic desease. A prevalence study 0f IgG4-related ophthalmic disease in Japan.JpnJOphthalmol.2013;57: 573–579.

  2. Shields JA et al. Survey of 1264 patients with orbital tumors and simulating lesions: the 2002 Montgomery Lecture, part I. Ophthalmology.2004;111: 997–1008.

  3. Goto H et al. Diagnostic criteria for IgG4-related ophthalmic disease. JpnJOphthalmol.2015;59:1–7.


Disclosure of Interest None declared

Statistics from

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.