P53 Paediatric non-infectious dacryoadenitis: a single-centre experience

• Published in: Rheumatology (Oxford, England) England), 2019-10, Vol.58 (Supplement_4)
• Main Authors: Silva-Dinis, Joana, Solebo, Ameenat Lola, Abou-Rayyah, Yassir, Compeyrot-Lacassagne, Sandrine
• Format: Article
• Language: English
• ISSN: 1462-0324; 1462-0332
• DOI: 10.1093/rheumatology/kez416.020

Abstract Background Dacryoadenitis is an inflammatory enlargement of the lacrimal gland and can be idiopathic or associated with infections, malignant or inflammatory diseases like Sjögren’s disease (SSj) or granulomatosis with polyangiitis (GPA). It remains the most common orbital inflammatory condition in children. This study purposes to describe a paediatric cohort of non-infectious dacryoadenitis. Methods Identification of children who underwent lacrimal gland biopsy at our centre between 2000 and 2018 was done by searching the database using two key-words: “lacrimal” and “dacryoadenitis”. Patients with infectious or malignant dacryoadenitis were excluded. Medical notes were reviewed to collect data. Results Twenty-six patients underwent lacrimal gland biopsy at our centre between 2000 and 2018 and had inflammatory dacryoadenitis. 17 patients (65.4%) had non-specific chronic inflammation (NSCI) and 9 patients (34.6%) had an inflammatory systemic condition (Table 1). P53 Table 1Patients’ characteristics.NSCIGPASarcoidosisSSjogrenIgG4Number of patients17 (65.4%)4 (15.4%)3 (11.5%)1 (3.9%)1 (3.9%)Female, n (%)6 (35.3%)4 (100%)3 (100%)1 (100%)1 (100%)Median age (Y) (IQ)10.5 (4.9- 14.4)9.72 (8.7- 13.0)13.2 (11.45- 15.7)11.911.2Lacrimal gland involvementUnilateral13 (74.5%)3 (75.0%)2 (66.7%)00Bilateral4 (23.5%)1 (25.0%)1 (33.3%)1 (100%)1 (100%)Orbital symptomsPtosis4 (23.5%)1 (25.0%)000Erythema3 (17.5%)1 (25.0%)1 (33.3%)00Pain2 (11.8%)1 (25.0%)000Extra-orbital involvementExocrine glands001 (33.3%)00ENT03 (75.0%)000Renal1 (14.3%)2 (50.0%)01 (100%)1 (100%)Pulmonary1 (14.3%)1 (25.0%)1 (33.3%)01 (100%)Myositis1 (14.3%)0000Arthralgia1 (14.3%)01 (33.3%)00Thyroiditis1 (14.3%)0000CNS0001 (100%)0Lymphadenopaties00001 (100%) 4/13 children tested had raised ACE (2 sarcoidosis and 2 NSCI). 7/17 had raised amylase (2 sarcoidosis,1 SSj, 4 NSCI). 3/16 had raised IgG (IgG4 disease, GPA and NSCI). ANA was positive in 4/19 patients (NSCI, GPA, Sarcoidose, SSj) and ANCA was positive in 4/19 patients (GPA). Thirteen patients received steroids (oral or IV) and elevan received DMARDS (MMF, methotrexate, azathioprine and hydroxychloroquine). Four patients received biologic (rituximab, adalimumab or infliximab). Six patients underwent orbitotomy. Patients still on follow-up had good response to treatment. One patient with NSCI presented with dacryoadenitis and later developed auto-immune thyroiditis and polymyositis, with positive ANA and anti-SRP antibodies. He was treated with ste