Purpose

IgG4 related disease, an immune-mediated fibroinflammatory condition is characterized by multiorgan inflammatory lesions. Vascular involvement has been confined to large vessels, with no reports of small vessel involvement. We report two patients with IgG4 related disease with small vessel retinal vasculitis characterized on fluorescein angiography.

Methods

Case reports of two patients with IgG4 disease with small vessel retinal vasculitis. Both underwent extensive laboratory work-up and multimodal imaging.

Results

Case 1 was a 17 -year-old boy who presented with global developmental delay and diminution of vision in both eyes for the past 6 weeks. Visual acuity was 6/60 in right and 5/60 in left eye. There was moderate anterior segment inflammation and vitritis in both eyes. Fundus fluorescein Angiography revealed fern leaf pattern of capillary leakage suggestive of small vessel retinal vasculitis. Patient had elevated serum IgG4 with raised IgG4 positive plasma cells and positive ANA. Case 2 was a 6-year-old girl who presented with proptosis and restriction of extraocular movement with decrease in visual acuity in left eye for past 2 years. Visual acuity was 6/9 in the right and 6/60 in her left eye with moderate anterior segment inflammation, posterior synechiae and vitritis left eye. CECT orbit showed heterogeneously enhancing ill-defined mass lesion in the intraconal space of left orbit.Fundus fluorescein Angiography revealed hyperfluorescence in late phase showing typical fern leaf pattern of capillary leakage suggestive of small vessel vasculitis in both cases. Serum IgG4 levels were raised. Laboratory tests for tuberculosis and serology for syphilis and HIV were negative in both patients. Both patients responded to treatment with oral steroids and immunosuppression.

Conclusions

IgG4 disease may present with small vessel retinal vasculitis.