SAT0552\u2005PAEDIATRIC NON-INFECTIOUS DACRYOADENITIS: THE GOSH EXPERIENCE

Abstract

Background: Dacryoadenitis is an inflammatory enlargement of the lacrimal gland and the most common orbital inflammatory condition in the paediatric population, although only 6 to 17% of orbital inflammatory disorders occur in children (1-4). Dacryoadenitis may be idiopathic or associated with infections or inflammatory diseases. Systematic inflammatory disorders are more typically associated with chronic than acute dacryoadenitis (3). We describe the largest paediatric cohort of biopsy proven non-infectious dacryoadenitis (4,5). Objectives: To describe lacrimal gland biopsy results To report associated clinical, laboratory features and treatment Methods: We identified 72 children who had lacrimal gland biopsy at GOSH between 25/09/2000 and 12/04/2018 using two key-words: “lacrimal” and “dacryoadenitis”. We excluded patients with infectious dacryoadenitis, benign tumors or malignancy. We retrospectively reviewed the medical notes, laboratory and histopathology results. Results: Twenty six patients had non-infectious dacryoadenitis with or without soft tissue involvement. All cases manifested as upper lid swelling. 19 (73.1%) patients had unilateral involvement. 12 (46.2%) patients were male. Mean age at time of biopsy was 10.5 years and median 13 years (range 1 – 17.7). Four patients had GPA (granulomatous polyangiitis), 4 patients sarcoidosis, 1 patient IgG4-related disease, 1 patient Sjögren with secondary anti-phospholipid syndrome and 16 patients non-specific chronic inflammation (NSCI). 2/4 patients with sarcoidosis had extra-ocular involvement. One patient with sarcoidosis had NOD2 mutation without Blau phenotype. One patient with GPA had extra-ocular involvement. 4/13 children tested had raised ACE including 2 sarcoidosis and 2 NSCI. 7/17 had raised amylase (2 sarcoidosis, 1 Sjögren, 4 NSCI). 7/20 had raised ESR (1 Sjögren, 5 NSCI and 1GPA with renal involvement). 3/16 had raised IgG (IgG4 disease, GPA with renal involvement and NSCI). 13 (50%) patients received systemic steroids. 11 (42.3%) patients received DMARDS (MMF, Methotrexate, Azathioprine and Hydroxychloroquine). 4 (15.4%) patients received a biologic: 3/4 rituximab (2 GPA and one IgG4-related disease) and one patient with NSCI received anti-TNF. 6 (23.1%) patients underwent orbitotomy. Patients still on follow-up had good response to treatment and did not experience any local complication. One patient with NSCI developed auto-immune thyroiditis and polymyositis. Conclusion: Most of the patients in our retrospective cohort study had NSCI and may be idiopathic. Few patients had a specific diagnosis on biopsy allowing targeted treatment. Patients with non-infectious dacryoadenitis may benefit from tissue biopsy to exclude infection and malignancy and streamline management. On-going monitoring is important for patients with NSCI to identify underlying inflammatory diseases at an early stage. References [1] Belanger C, et al. Inflammatory Disorders of the Orbit in Childhood: A Case Series. Am J Ophthalmol. 2010Oct;150(4):460-3 [2] Spindle J, Tang SX, Davies B, Wladis EJ, Piozzi E, Pellegrini M, Lally SE, Shields C, Shinder R. Pediatric Idiopathic Orbital Inflammation: Clinical Features of 30 Cases. Ophthalmic Plast Reconstr Surg. 2016 Jul-Aug;32(4):270-4 [3] Andrew NH, Kearney D, Sladden N, McKelvie P, Wu A, Sun MT, McNab A, Selva D. Idiopathic Dacryoadenitis: Clinical Features, Histopathology, and Treatment Outcomes. Am J Ophthalmol. 2016Mar;163:148-153 [4] Luemsamran P, Rootman J, White VA, Nassiri N, Heran MKS. The role of biopsy in lacrimal gland inflammation: A clinicopathologic study. Orbit. 2017Dec;36(6):411-418 [5] Boulter EL, Eleftheriou D, Sebire NJ, Edelsten C, Brogan PA. Inflammatory lesions of the orbit: a single paediatric rheumatology centre experience. Rheumatology2012. 51(6):1070–1075 Disclosure of Interests: Joana Silva-Dinis: None declared, Ameenat Lola Solebo: None declared, Yassir Abou-Rayyah: None declared, Sandrine Compeyrot-Lacassagne Grant/research support from: Abbvie