Orbital inflammation with IgG4-positive plasma cells: Manifestation of IgG4 systemic disease

José Antonio Plaza, James A. Garrity, Ahmet Dogan, Anuradha Ananthamurthy, Thomas Elmer Witzig, Diva R. Salomão

Research output: Contribution to journalArticle

109 Citations (Scopus)

Abstract

Objective: To describe clinical, radiographic, and morphologic findings in patients with IgG4-positive cells present on orbital biopsy specimens. Design: Retrospective review (from January 1, 1993, through December 31, 2006) of patients with orbital biopsy specimens that excluded lymphoma; comparison of patients with and without IgG4-positive cells on immunostaining. Results: Of 21 patients, 11 had increased IgG4-positive cells (defined as >10 cells on biopsy). Symptoms included eyelid or periocular swelling (8 patients) or proptosis (3 patients), with bilateral involvement in 6 patients. Computed tomographic imaging displayed lacrimal gland mass in 10 patients; 6 patients had lesions in other organs. Two patients had increased serum IgG4 levels. In 10 patients without IgG4-positive cells (≤10 cells on biopsy), 6 had proptosis, 1 had eyelid swelling, 2 had eyelid mass, and 1 had diplopia, all unilateral. None had systemic symptoms. Patients with IgG4-positive cells had longer symptom duration, and their biopsy specimens showed more background fibrosis, lymphoid hyperplasia, plasma cells, and eosinophils. Conclusions: The clinical appearance, high incidence of bilateral disease, association with lesions in other organs, and increased IgG4 serum levels in some patients-with an increased number of IgG4-positive cells in the biopsy specimen, which shows more background fibrosis, lymphoid hyperplasia, plasma cells, and eosinophils-indicate that these patients have an orbital manifestation of IgG4-associated systemic disease.

Original languageEnglish (US)
Pages (from-to)421-428
Number of pages8
JournalArchives of Ophthalmology
Volume129
Issue number4
DOIs
StatePublished - Apr 2011

Fingerprint

Plasma Cells
Immunoglobulin G
Inflammation
Biopsy
Eyelids
Exophthalmos
Eosinophils
Hyperplasia
Fibrosis
Lacrimal Apparatus
Diplopia
Serum
Lymphoma

ASJC Scopus subject areas

  • Ophthalmology

Cite this

Orbital inflammation with IgG4-positive plasma cells : Manifestation of IgG4 systemic disease. / Plaza, José Antonio; Garrity, James A.; Dogan, Ahmet; Ananthamurthy, Anuradha; Witzig, Thomas Elmer; Salomão, Diva R.

In: Archives of Ophthalmology, Vol. 129, No. 4, 04.2011, p. 421-428.

Research output: Contribution to journalArticle

Plaza, José Antonio ; Garrity, James A. ; Dogan, Ahmet ; Ananthamurthy, Anuradha ; Witzig, Thomas Elmer ; Salomão, Diva R. / Orbital inflammation with IgG4-positive plasma cells : Manifestation of IgG4 systemic disease. In: Archives of Ophthalmology. 2011 ; Vol. 129, No. 4. pp. 421-428.
@article{e8be50a62cf84bb3bd78cd465bccaf9d,
title = "Orbital inflammation with IgG4-positive plasma cells: Manifestation of IgG4 systemic disease",
abstract = "Objective: To describe clinical, radiographic, and morphologic findings in patients with IgG4-positive cells present on orbital biopsy specimens. Design: Retrospective review (from January 1, 1993, through December 31, 2006) of patients with orbital biopsy specimens that excluded lymphoma; comparison of patients with and without IgG4-positive cells on immunostaining. Results: Of 21 patients, 11 had increased IgG4-positive cells (defined as >10 cells on biopsy). Symptoms included eyelid or periocular swelling (8 patients) or proptosis (3 patients), with bilateral involvement in 6 patients. Computed tomographic imaging displayed lacrimal gland mass in 10 patients; 6 patients had lesions in other organs. Two patients had increased serum IgG4 levels. In 10 patients without IgG4-positive cells (≤10 cells on biopsy), 6 had proptosis, 1 had eyelid swelling, 2 had eyelid mass, and 1 had diplopia, all unilateral. None had systemic symptoms. Patients with IgG4-positive cells had longer symptom duration, and their biopsy specimens showed more background fibrosis, lymphoid hyperplasia, plasma cells, and eosinophils. Conclusions: The clinical appearance, high incidence of bilateral disease, association with lesions in other organs, and increased IgG4 serum levels in some patients-with an increased number of IgG4-positive cells in the biopsy specimen, which shows more background fibrosis, lymphoid hyperplasia, plasma cells, and eosinophils-indicate that these patients have an orbital manifestation of IgG4-associated systemic disease.",
author = "Plaza, {Jos{\'e} Antonio} and Garrity, {James A.} and Ahmet Dogan and Anuradha Ananthamurthy and Witzig, {Thomas Elmer} and Salom{\~a}o, {Diva R.}",
year = "2011",
month = "4",
doi = "10.1001/archophthalmol.2011.16",
language = "English (US)",
volume = "129",
pages = "421--428",
journal = "JAMA Ophthalmology",
issn = "2168-6165",
publisher = "American Medical Association",
number = "4",

}

TY - JOUR

T1 - Orbital inflammation with IgG4-positive plasma cells

T2 - Manifestation of IgG4 systemic disease

AU - Plaza, José Antonio

AU - Garrity, James A.

AU - Dogan, Ahmet

AU - Ananthamurthy, Anuradha

AU - Witzig, Thomas Elmer

AU - Salomão, Diva R.

PY - 2011/4

Y1 - 2011/4

N2 - Objective: To describe clinical, radiographic, and morphologic findings in patients with IgG4-positive cells present on orbital biopsy specimens. Design: Retrospective review (from January 1, 1993, through December 31, 2006) of patients with orbital biopsy specimens that excluded lymphoma; comparison of patients with and without IgG4-positive cells on immunostaining. Results: Of 21 patients, 11 had increased IgG4-positive cells (defined as >10 cells on biopsy). Symptoms included eyelid or periocular swelling (8 patients) or proptosis (3 patients), with bilateral involvement in 6 patients. Computed tomographic imaging displayed lacrimal gland mass in 10 patients; 6 patients had lesions in other organs. Two patients had increased serum IgG4 levels. In 10 patients without IgG4-positive cells (≤10 cells on biopsy), 6 had proptosis, 1 had eyelid swelling, 2 had eyelid mass, and 1 had diplopia, all unilateral. None had systemic symptoms. Patients with IgG4-positive cells had longer symptom duration, and their biopsy specimens showed more background fibrosis, lymphoid hyperplasia, plasma cells, and eosinophils. Conclusions: The clinical appearance, high incidence of bilateral disease, association with lesions in other organs, and increased IgG4 serum levels in some patients-with an increased number of IgG4-positive cells in the biopsy specimen, which shows more background fibrosis, lymphoid hyperplasia, plasma cells, and eosinophils-indicate that these patients have an orbital manifestation of IgG4-associated systemic disease.

AB - Objective: To describe clinical, radiographic, and morphologic findings in patients with IgG4-positive cells present on orbital biopsy specimens. Design: Retrospective review (from January 1, 1993, through December 31, 2006) of patients with orbital biopsy specimens that excluded lymphoma; comparison of patients with and without IgG4-positive cells on immunostaining. Results: Of 21 patients, 11 had increased IgG4-positive cells (defined as >10 cells on biopsy). Symptoms included eyelid or periocular swelling (8 patients) or proptosis (3 patients), with bilateral involvement in 6 patients. Computed tomographic imaging displayed lacrimal gland mass in 10 patients; 6 patients had lesions in other organs. Two patients had increased serum IgG4 levels. In 10 patients without IgG4-positive cells (≤10 cells on biopsy), 6 had proptosis, 1 had eyelid swelling, 2 had eyelid mass, and 1 had diplopia, all unilateral. None had systemic symptoms. Patients with IgG4-positive cells had longer symptom duration, and their biopsy specimens showed more background fibrosis, lymphoid hyperplasia, plasma cells, and eosinophils. Conclusions: The clinical appearance, high incidence of bilateral disease, association with lesions in other organs, and increased IgG4 serum levels in some patients-with an increased number of IgG4-positive cells in the biopsy specimen, which shows more background fibrosis, lymphoid hyperplasia, plasma cells, and eosinophils-indicate that these patients have an orbital manifestation of IgG4-associated systemic disease.

UR - http://www.scopus.com/inward/record.url?scp=79953906790&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=79953906790&partnerID=8YFLogxK

U2 - 10.1001/archophthalmol.2011.16

DO - 10.1001/archophthalmol.2011.16

M3 - Article

C2 - 21482868

AN - SCOPUS:79953906790

VL - 129

SP - 421

EP - 428

JO - JAMA Ophthalmology

JF - JAMA Ophthalmology

SN - 2168-6165

IS - 4

ER -