Ocular adnexal IgG4-related disease has uniform clinicopathology

• Published in: Pathology international 2008-08, Vol.58 (8), p.465-470
• Main Authors: Sato, Yasuharu, Ohshima, Koh-ichi, Ichimura, Kouichi, Sato, Masakazu, Yamadori, Ichiro, Tanaka, Takehiro, Takata, Katsuyoshi, Morito, Toshiaki, Kondo, Eisaku, Yoshino, Tadashi
• Format: Article
• Language: English
• Subjects: Adult; Aged; Aged, 80 and over; clinicopathological features; Dacryocystitis - immunology; Dacryocystitis - pathology; Female; Gene Rearrangement, B-Lymphocyte, Heavy Chain; Humans; Hypergammaglobulinemia - genetics; Hypergammaglobulinemia - immunology; Hypergammaglobulinemia - pathology; IgH gene rearrangement; Immunoglobulin G - blood; Lacrimal Apparatus - immunology; Lacrimal Apparatus - pathology; Lacrimal Apparatus Diseases - genetics; Lacrimal Apparatus Diseases - immunology; Lacrimal Apparatus Diseases - pathology; Lymphocytes - immunology; Lymphocytes - pathology; Lymphoid Tissue - immunology; Lymphoid Tissue - pathology; Male; marginal zone B-cell lymphoma; Middle Aged; ocular adnexal IgG4-related disease; Orbital Diseases - immunology; Orbital Diseases - pathology; Plasma Cells - immunology; Plasma Cells - pathology
• ISSN: 1320-5463; 1440-1827
• DOI: 10.1111/j.1440-1827.2008.02257.x

IgG4‐related disease is a recently proposed clinical entity with several unique clinicopathological features. Ocular adnexal IgG4‐related disease, however, has not well been clarified. The purpose of the present study was to examine 21 patients (10 men, 11 women; age range, 39–86 years) with ocular adnexal IgG4‐related disease. In 17 out of 21 patients (81%), the lacrimal glands were involved and bilateral lacrimal gland swelling was frequently observed (n = 12; 70.6%). In contrast, the conjunctiva was not involved in any of the patient. Histology was uniform with marked lymphoplasmacytic infiltration admixed with dense fibrosis, similar to previous reports of IgG4‐related disease. Immunostaining detected numerous aggregates of IgG4‐positive plasma cells. Serum IgG4 was higher than normal in 10 of the 13 patients tested, although it was measured after treatment in almost all cases. Interestingly, immunoglobulin heavy chain gene rearrangement was detected in two of 17 patients (12%) examined. The present results show that ocular adnexal IgG4‐related disease has uniform clinicopathology: that is, disease involving the bilateral lacrimal glands with lymphoid hyperplasia and fibrosis, but not the conjunctiva. And presence of immunoglobulin heavy chain gene rearrangement suggests the possibility of B‐cell lymphoma arising in a background of IgG4‐related chronic inflammation.