Clinicopathological features of IgG4-related disease complicated with orbital involvement. Issue 3 (May 2014)

• Record Type: Journal Article
• Authors: Hagiya, Chihiro; Tsuboi, Hiroto; Yokosawa, Masahiro; Hagiwara, Shinya; Hirota, Tomoya; Takai, Chinatsu; Asashima, Hiromitsu; Miki, Haruka; Umeda, Naoto; Horikoshi, Masanobu; Kondo, Yuya; Sugihara, Makoto; Ogishima, Hiroshi; Suzuki, Takeshi; Hiraoka, Takahiro; Kaji, Yuichi; Matsumoto, Isao; Oshika, Tetsuro; Sumida, Takayuki
• Is Part Of: Modern rheumatology. Volume 24:Issue 3(2014)
• Page Start: 471
• Page End: 476
• Publication Date: 2014-05
• Subjects: Rheumatology -- Periodicals; 616.723005
• DOI: 10.3109/14397595.2013.844307
• Languages: English
• ISSNs: 1439-7595

Abstract

<abstract> <title>Abstract</title> <p> <italic>Objective.</italic> IgG4-related disease (IgG4-RD) is characterized by IgG4-positive plasmacytic infiltration and fibrosis in various organs. Orbital involvement in IgG4-RD includes lacrimal glands, extra-ocular muscles, trigeminal nerve and other parts of the orbit. Immunohistochemical staining is used to diagnose IgG4-RD in patients with orbital inflammation. The purpose of this retrospective study was to clarify the clinicopathological features of IgG4-RD complicated with orbital involvement.</p> <p> <italic>Methods.</italic> We examined the clinical features, pathological findings and response to treatment in nine patients with IgG4-RD who underwent orbital tissue biopsy between April 2010 and August 2012 at the University of Tsukuba Hospital.</p> <p> <italic>Results.</italic> Among the nine patients, eight had dacryoadenitis, one had infraorbital nerve swelling, and another one had IgG4-related orbital inflammation. Involvement of other organs was identified in all patients, including involvement of the salivary glands, lymph nodes, lung, kidney and para-aorta. In all patients, biopsy samples from orbital tissues showed lymphoplasmacytic infiltration and fibrosis, and IgG4-positive/IgG-positive plasmacyte ratio of > 40%. All patients were treated with prednisolone (0.6 mg/kg/day) and responded well in early phase, although relapse was noted in two patients following tapering of prednisolone, evident by swelling of lacrimal glands.</p> <p> <italic>Conclusion.</italic> Patients with IgG4-RD complicated with orbital involvement often present with involvement of other organs. The histopathological findings of orbital tissue match the characteristic features of IgG4-RD. Corticosteroid is effective for orbital and systemic involvement in IgG4-RD.</p> </abstract>