IgG4-related disease in idiopathic sclerosing orbital inflammation.
Br J Ophthalmol, 2015/11;99(11):1493-7.
Sa HS[1], Lee JH[2], Woo KI[2], Kim YD[2]
Affiliations
PMID: 25947555DOI: 10.1136/bjophthalmol-2014-305528
Impact factor: 5.908
Abstract
aims: To investigate the frequency of IgG4-related disease (IgG4-RD) among patients previously diagnosed with idiopathic sclerosing orbital inflammation (ISOI), and to compare the clinical features and treatment outcomes of patients with ISOI associated with IgG4-RD and those without IgG4.
methods: Retrospective clinicopathological series of 24 patients with ISOI diagnosed between June 2001 and June 2010. Biopsy specimens were immunostained for IgG-expressing and IgG4-expressing cells. Clinical data of patients with IgG4-RD and ISOI unrelated to IgG4 were obtained from patient records.
results: Of 24 patients, 11 patients (45.8%) were identified with IgG4-RD. 10 patients (10/11, 90.9%) presented with bilateral lacrimal gland enlargement, and seven of those also had submadibular gland enlargement. One patient (1/11, 9.1%) presented with a superior orbital mass. All patients were successfully treated with steroids and/or radiotherapy or had an indolent clinical course. 13 patients (54.2%) were identified with ISOI unrelated to IgG4. Eight patients (8/13, 61.5%) showed unilateral orbital involvement, and nine patients (9/13, 69.2%) had orbital lesions not involving the lacrimal glands. Treatment modalities for ISOI unrelated to IgG4 were varied and less effective: eight patients (61.5%) relapsed following initial treatment with steroids or radiation, and additional therapies were required to enter remission.
conclusions: IgG4-RD may be identified frequently in patients with ISOI, and distinguishing features may be bilateral lacrimal gland enlargement with associated submandibular gland enlargement. Patients with IgG4-RD may have better treatment outcomes with less aggressive treatment modalities than those with ISOI unrelated to IgG4. An additional workup for IgG4-RD should be considered in all histopathological biopsy specimens suspicious of ISOI.
Keywords: Inflammation; Lacrimal gland; Orbit; Pathology
MeSH terms
Adult; Aged; Autoimmune Diseases; Female; Glucocorticoids; Humans; Immunoglobulin G; Immunosuppressive Agents; Lymphoproliferative Disorders; Male; Middle Aged; Orbit; Orbital Pseudotumor; Plasma Cells; Positron-Emission Tomography; Radiotherapy; Retrospective Studies; Sclerosis; Treatment Outcome; Young Adult
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