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SAT0342 Features of Orbital Inflammatory Disease and Response To Immunosuppressive Therapy
  1. A. Casian1,
  2. S. Sangle (joint first author)1,
  3. R. Malaiya2,
  4. P. Lutalo3,
  5. L. Nel1,
  6. B. Menon4,
  7. H. Verma5,
  8. M. Stanford6,
  9. D. D'Cruz1
  1. 1Lupus Unit, Guy's and St. Thomas' NHS Foundation Trust
  2. 2Rheumatology, Guy's and St Thomas' NHS Foundation Trust
  3. 3Rheumatology, University Hospital Lewisham
  4. 4Rheumatology
  5. 5Radiology
  6. 6Ophthalmology, Guy's and St. Thomas' NHS Foundation Trust, London, United Kingdom


Objectives To characterize a single centre retrospective case series of patients with infra-orbital inflammatory masses with autoimmune disease including granulomatosis with polyangiitis (GPA) (formerly Wegener's granulomatosis), eGPA (eosinophillic granulomatosis with polyangiitis) or Immunoglobulin G4 (IgG4) related disease (IgG4-RD).

Methods We identified 30 patients with infra-orbital inflammation on MRI imaging. Clinical and laboratory data was collected from electronic clinical records. Comprehensive Diagnostic Criteria were used for IgG4-RD and Chapel Hill criteria for GPA and eGPA. Statistical analysis was performed by GraphPad software; continuous variables were compared between IgG4-RD and GPA groups using non-parametric Mann-Whitney test and categorical variables were compared by Fisher's exact test.

Results The study included 21 Caucasian, 6 Asian and 3 patients of African descent. There were 19 female and 11 male patients. The median age of the patients was 44 years (range 29–76).

13 patients were diagnosed with GPA, 1 with eosinophillic granulomatosis with polyangitis 9eGPA), 11 patients had IgG-RD, 1 patient with lymphoma, 2 other vasculitis, 1 IgA dacryoadenitis, 1 non-specific granuloma.

7/12 patients with IgG4-RD had isolated infra-orbital masses whereas all 14 GPA patients suffered extra-ocular manifestations (p=0.01), usually sino-nasal or pulmonary.

11/14 GPA patients had positive ANCA vs 2/12 patients with Ig4-RD (p=0.04). IgG4 level was elevated pre-treatment in IgG4 RD patients (median 2.46 g/l (range 1.2–23.7)) and dropped to 1.25 g/l (range 0.37–10.4) after therapy; immunoglobulin subclasses were not checked routinely in GPA.

All 12 patients with IgG4-RD underwent diagnostic orbital biopsy vs 3/14 GPA (p=0.0001).

All 30 patients were treated with corticosteroids (used alone in 3/12 IgG4-RD patients). The median number of DMARDs ever used to treat GPA was 3 vs 1 DMARD for IgG4-RD (p=0.001). Rituximab was effectively administered to 10/14 GPA patients vs 3/12 IgG4-RD (p=0.04), is planned for 2 further IgG4-RD patients and approval was refused for 1 case. Surgical debulking was undertaken in 6/12 IgG4-RD vs 1/14 GPA (p=0.03). All 40 patients had subsequent MRI to assess response to therapy.

Conclusions IgG4-RD is an important differential diagnosis of infra-orbital inflammation, especially if ANCA is negative. Unlike GPA that was associated with extra-ocular manifestations in all patients, IgG4-RD was more likely to present with isolated orbital inflammation and to require biopsy or surgical debulking as the diagnosis was initially uncertain. Treatment with corticosteroids +/− DMARDs was effective. Rituximab can specifically deplete the pool of autoreactive B lymphocytes producing IgG4 and future systematic studies are required to establish the optimum therapeutic strategy.

Disclosure of Interest None declared

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