IgG4-related ophthalmic disease presenting as posterior scleritis in a pediatric patient

Abstract

Purpose: To report IgG4-related ophthalmic disease (IgG4-ROD) presenting as posterior scleritis in a pediatric patient.

Observations: A 7-year-old girl presented with proptosis, painful eyelid swelling, and restricted extraocular movements (EOM) of her left eye (OS). Visual acuity (VA) was 20/20 in right eye (OD) and counting fingers (CF) at 1 foot in OS. Slit lamp examination revealed 2 + anterior chamber (AC) cells, optic disc edema (ODE) with elevated appearance of macula in OS. Optical coherence tomography (OCT) showed significant subretinal fluid (SRF) in macula, B-scan ultrasound (US) demonstrated T-sign in OS. Orbital MRI was also consistent with posterior scleritis and periorbital inflammation. Extensive systemic work-up was unremarkable. Thus, the patient was started on intravenous methylprednisolone (IVMP) 30 mg/kg/day for 3 days, along with topical therapy in OS, which led to an improvement of proptosis, EOM restriction, AC cells, as well as ODE and SRF in macula in OS. Fluorescein angiography (FA) showed leakage from optic disc in OS. The patient was then switched to oral prednisone with slow tapering and started on methotrexate (MTX). Given the recurrence of proptosis and painful eyelid swelling on systemic steroid tapering, serum IgG4 levels were ordered and found to be elevated at 149.9 mg/dL (range, 1-99). Therefore, the patient was diagnosed as 'possible' IgG4-ROD (based on diagnostic criteria) and started on infliximab (7.5 mg/kg) and IVMP monthly infusions with continuation of MTX 20 mg weekly and slower tapering of oral prednisone, which led to resolution of clinical findings, improvement of VA to 20/20 in OS.

Conclusion and importance: Posterior scleritis may be the initial presentation of IgG4-ROD in children. Refractory course is not uncommon. Biologics are effective in the long-term control of inflammation.

Keywords: IgG4-related ophthalmic disease; Infliximab; Pediatric; Posterior scleritis.

Fig. 1

Initial presentation of the patient with optic disc edema in left eye (A) and subretinal fluid at macula (B), as well as T-sign on B-scan (C) and MRI showing T2 and T1 hypointense and hyper-enhancing tissue along the left posterior retina and along the posterior aspect of the globe extending into the intraconal fat (D, red arrows)

Fig. 2

Following pulse intravenous methylprednisolone therapy, improved proptosis (A), optic disc edema (B), subretinal fluid at macula on optical coherence tomography (C) in the left eye along with leakage from optic disc on fluorescein angiography (D)

Fig. 3

At 3 months of follow-up, while on methotrexate therapy and oral prednisone tapering, recurrence of proptosis, periorbital edema and erythema (A), anterior scleral inflammation (B), despite unremarkable fundus exam (C) and optical coherence tomography (D) with stable mild leakage from optic disc in the left eye on fluorescein angiography (E)

Fig. 4

After first cycle of infliximab and intravenous methylprednisolone therapy, improved proptosis (A) with unremarkable fundus exam (C) and optical coherence tomography (D) as well as improved leakage from optic disc in the left eye on fluorescein angiography (E)

Fig. 5

After five cycles of infliximab and intravenous methylprednisolone therapy, unremarkable fundus exam (A), optical coherence tomography (B) and fluorescein angiography (C) in both eyes