Ocular adnexal IgG4-related lymphoplasmacytic infiltrative disorder.
OBJECTIVE
To determine the clinicopathological characteristics of patients with infiltration of IgG4-positive plasma cells into the ocular adnexa.


METHODS
We designed a prospective study to evaluate 24 patients with ocular adnexal lymphoplasmacytic infiltrative lesions, including sclerosing inflammation and reactive lymphoid hyperplasia. We analyzed peripheral blood and biopsy specimens from all patients. The classification criteria for placement in the IgG4-related group included having both an elevated serum level of IgG4 of 135 mg/dL or greater and an IgG4:IgG ratio of infiltrating plasma cells of 30% or greater.


RESULTS
Ten patients met the classification criteria (IgG4-related group), 9 patients did not meet the criteria (IgG4-unrelated group), and 5 patients met 1 but not both criteria (indeterminate group). Patients in the IgG4-related group had significantly higher bilateral involvement (P = .02), a higher number of allergic diseases (P = .01), and elevated IgE serum levels (P = .01). Of the 10 patients in the IgG4-related group, 3 also had polyclonal hypergammaglobulinemia, 6 had systemic lymphadenopathy or salivary gland enlargement, and 1 developed autoimmune pancreatitis. Patients in the IgG4-unrelated group did not have these serum and/or systemic abnormalities.


CONCLUSIONS
The IgG4-related and IgG4-unrelated groups have different patterns of tissue involvement and systemic disease associations and possibly different prognoses.
Kubota
Toshinobu
T
Department of Ophthalmology, National Hospital Organization, Nagoya Medical Center, 4-1-1, Sannomaru, Naka-ku, Nagoya-shi, Aichi-ken 460-0001, Japan. ganiky@nnh.hosp.go.jp
Moritani
Suzuko
S
Katayama
Masao
M
Terasaki
Hiroko
H
D000328:Adult; D000368:Aged; D003229:Conjunctival Diseases / Q000097:blood / Q000000981:diagnostic imaging / Q000473:pathology*; D005141:Eyelid Diseases / Q000097:blood / Q000000981:diagnostic imaging / Q000473:pathology*; D005260:Female; D019788:Fluorodeoxyglucose F18; D005710:Gallium Radioisotopes; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D008232:Lymphoproliferative Disorders / Q000097:blood / Q000000981:diagnostic imaging / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010950:Plasma Cells / Q000473:pathology; D049268:Positron-Emission Tomography; D011446:Prospective Studies; D019310:Pseudolymphoma / Q000097:blood / Q000000981:diagnostic imaging / Q000473:pathology*; D019275:Radiopharmaceuticals; D014057:Tomography, X-Ray Computed; D055815:Young Adult
D005710:Gallium Radioisotopes; D007074:Immunoglobulin G; D019275:Radiopharmaceuticals; D019788:Fluorodeoxyglucose F18