IgG4-Related Dacryoadenitis

Background: Eye and periorbital infections are a common problem in the paediatric population and differential diagnosis should be considered in cases with atypical presentation. Herein, we describe a paediatric case of a probable IgG4-related disease (RD) presenting with orbital and eyelid swelling, successfully treated with steroids.

Case Description: A 9-year-old boy, with a past history of rhinitis and asthma, and family history of lupus (38 years-old mother) presented with progressively swelling and ptosis of the right eye that improved along the day, pruritus, conjunctival hyperaemia and ocular pain, without exudate or fever. There was no history of upper respiratory infection, local skin lesion, dental abscess or trauma. Visual acuity, funduscopic examination and the neurologic exam were normal. The computed tomography scan showed densification and swelling of the right peri-orbital tissues with pre-septal extension to the lacrimal gland, without signs of sinusitis or mastoiditis. After 5 days of antibiotics there was no clinical improvement, and he started treatment with steroids (prednisolone, 1,5mg/kg/day) with good clinical evolution. The laboratory investigation showed mild eosinophilia (810/uL), serum IgG4 324 mg/dl (reference upper limit of 169.9 mg/dl), negative autoantibodies (c-ANCA, p-ANCA, antinuclear, anti-double stranded DNA, and extractable nuclear antigen antibodies and rheumatoid factor); adequate C3 and C4 complement serum levels and serum immunoglobulins.

Conclusion: The exclusion of a proper pathogenesis to a periorbital cellulitis, the eosinophilia, high IgG4 serum levels, improvement of the oedema along the day, and the steroid responsiveness suggests the diagnosis of IgG4-related dacryoadenitis. Due to the typical picture and good outcome a biopsy was not performed. In children the ocular involvement is very common in patients with IgG4-RD (44%). It is important to remind this entity as a potential cause of periorbital inflammation.