Background/Purpose: To characterize a single centre retrospective case series of patients with orbital inflammatory masses associated with autoimmune diseases including granulomatosis with polyangiitis(GPA)(formerly Wegener’s granulomatosis), eosinophilic granulomatosis with polyangiitis(eGPA)  or Immunoglobulin G4 related disease(IgG4-RD).

Methods: We identified 30 patients with orbital inflammatory masses 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 was 44 years(range 29-76).

14 patients were diagnosed with GPA, 11 patients had IgG4-RD, 1 patient with IgG4 lymphoma, 1 with eGPA, 1 unspecified vasculitis, 1 IgA dacryoadenitis, 1 non-specific granuloma.

7/11 patients with IgG4-RD had isolated orbital masses whereas all 14 GPA patients suffered extra-ocular manifestations (p=0.01), usually sino-nasal or pulmonary disease.

11/14 GPA patients had positive ANCA vs 2/11 patients with Ig4-RD (p=0.04). IgG4 levels were 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(p=0.05); immunoglobulin subclasses were not checked routinely in GPA.

All 11 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 3/11 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 3/11 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 6/11 IgG4-RD vs 1/14 GPA (p=0.02). All 30 patients had subsequent MRI to assess response to therapy.

Conclusion: IgG4-RD is an important differential diagnosis of orbital inflammation, especially if ANCA is negative. Unlike GPA which 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, however the majority were left with some chronic visual damage. Serial imaging is useful to document clinical response. Rituximab can specifically deplete autoreactive B lymphocytes producing IgG4 and future systematic studies are required to establish the optimum therapeutic strategy.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/autoimmune-associated-orbital-inflammatory-masses-and-response-to-immunosuppressive-therapy/