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<meta name="DC.Title" content="Immunoglobulin G4–Related Disease of the Orbit: Imaging Features in 27 Patients" />
<meta name="DC.Identifier" content="10.3174/ajnr.A3865" />
<meta name="DC.Date" content="2014-03-13" />
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<meta name="DC.Description" content="BACKGROUND AND PURPOSE: Immunoglobulin G4–related disease is a systemic fibroinflammatory process of unknown etiology, characterized by tissue infiltration by immunoglobulin G4 plasma cells. The purpose of this study was to retrospectively identify the spectrum of imaging features seen in immunoglobulin G4–related disease of the orbit.

MATERIALS AND METHODS: This study included 27 patients with biopsy-proved immunoglobulin G4–related disease of the orbit and either a CT or MR imaging of the orbits. These CT or MR imaging examinations were evaluated for the following: extraocular muscle size, extraocular muscle tendon enlargement, lacrimal gland enlargement, infiltrative process in the orbital fat (increased attenuation on CT or abnormal signal on MR imaging), infraorbital nerve enlargement, mucosal thickening in the paranasal sinuses, and extension of orbital findings intracranially.

RESULTS: Extraocular muscles were enlarged in 24 of 27 (89%) patients, 21 (88%) bilaterally. In 32 of 45 (71%) affected orbits, the lateral rectus was the most enlarged muscle. In 26 (96%) patients, the tendons of the extraocular muscles were spared. Nineteen (70%) patients had lacrimal gland enlargement. Twelve (44%) patients had an infiltrative process within the orbital fat. Infraorbital nerve enlargement was seen in 8 (30%) patients. Twenty-four (89%) patients had sinus disease. Cavernous sinus or Meckel cave extension was seen in 3 (11%) patients.

CONCLUSIONS: In patients with extraocular muscle enlargement, particularly when the tendons are spared and the lateral rectus is the most enlarged, and even more so when other noted findings are present, immunoglobulin G4–related disease should be a leading differential consideration, even over more commonly known etiologies of extraocular muscle enlargement.

### Abbreviations

IgG4
:   immunoglobulin G4" />
<meta name="DC.Contributor" content="C.A. Tiegs-Heiden" />
<meta name="DC.Contributor" content="L.J. Eckel" />
<meta name="DC.Contributor" content="C.H. Hunt" />
<meta name="DC.Contributor" content="F.E. Diehn" />
<meta name="DC.Contributor" content="K.M. Schwartz" />
<meta name="DC.Contributor" content="D.F. Kallmes" />
<meta name="DC.Contributor" content="D.R. Salomão" />
<meta name="DC.Contributor" content="T.E. Witzig" />
<meta name="DC.Contributor" content="J.A. Garrity" />
<meta name="article:published_time" content="2014-03-13" />
<meta name="article:section" content="HEAD &amp;amp; NECK" />
<meta name="citation_title" content="Immunoglobulin G4–Related Disease of the Orbit: Imaging Features in 27 Patients" />
<meta name="citation_abstract" lang="en" content="&lt;h3&gt;BACKGROUND AND PURPOSE:&lt;/h3&gt;
&lt;p&gt;Immunoglobulin G4–related disease is a systemic fibroinflammatory process of unknown etiology, characterized by tissue infiltration by immunoglobulin G4 plasma cells. The purpose of this study was to retrospectively identify the spectrum of imaging features seen in immunoglobulin G4–related disease of the orbit.&lt;/p&gt;&lt;h3&gt;MATERIALS AND METHODS:&lt;/h3&gt;
&lt;p&gt;This study included 27 patients with biopsy-proved immunoglobulin G4–related disease of the orbit and either a CT or MR imaging of the orbits. These CT or MR imaging examinations were evaluated for the following: extraocular muscle size, extraocular muscle tendon enlargement, lacrimal gland enlargement, infiltrative process in the orbital fat (increased attenuation on CT or abnormal signal on MR imaging), infraorbital nerve enlargement, mucosal thickening in the paranasal sinuses, and extension of orbital findings intracranially.&lt;/p&gt;&lt;h3&gt;RESULTS:&lt;/h3&gt;
&lt;p&gt;Extraocular muscles were enlarged in 24 of 27 (89%) patients, 21 (88%) bilaterally. In 32 of 45 (71%) affected orbits, the lateral rectus was the most enlarged muscle. In 26 (96%) patients, the tendons of the extraocular muscles were spared. Nineteen (70%) patients had lacrimal gland enlargement. Twelve (44%) patients had an infiltrative process within the orbital fat. Infraorbital nerve enlargement was seen in 8 (30%) patients. Twenty-four (89%) patients had sinus disease. Cavernous sinus or Meckel cave extension was seen in 3 (11%) patients.&lt;/p&gt;&lt;h3&gt;CONCLUSIONS:&lt;/h3&gt;
&lt;p&gt;In patients with extraocular muscle enlargement, particularly when the tendons are spared and the lateral rectus is the most enlarged, and even more so when other noted findings are present, immunoglobulin G4–related disease should be a leading differential consideration, even over more commonly known etiologies of extraocular muscle enlargement.&lt;/p&gt;" />
<meta name="citation_journal_title" content="American Journal of Neuroradiology" />
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<meta name="citation_publication_date" content="2014/03/13" />
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<meta name="citation_author" content="C.A. Tiegs-Heiden" />
<meta name="citation_author_institution" content="From the Departments of Radiology (C.A.T.-H., L.J.E., C.H.H., F.E.D., K.M.S., D.F.K.), Pathology (D.R.S.), Hematology (T.E.W.), and Ophthalmology (D.R.S., J.A.G.), Mayo Clinic, Rochester, Minnesota." />
<meta name="citation_author" content="L.J. Eckel" />
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<meta name="citation_author_institution" content="From the Departments of Radiology (C.A.T.-H., L.J.E., C.H.H., F.E.D., K.M.S., D.F.K.), Pathology (D.R.S.), Hematology (T.E.W.), and Ophthalmology (D.R.S., J.A.G.), Mayo Clinic, Rochester, Minnesota." />
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<meta name="citation_author" content="K.M. Schwartz" />
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<meta name="citation_author" content="T.E. Witzig" />
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<meta name="twitter:title" content="Immunoglobulin G4–Related Disease of the Orbit: Imaging Features in 27 Patients" />
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<meta name="twitter:description" content="BACKGROUND AND PURPOSE: Immunoglobulin G4–related disease is a systemic fibroinflammatory process of unknown etiology, characterized by tissue infiltration by immunoglobulin G4 plasma cells. The purpose of this study was to retrospectively identify the spectrum of imaging features seen in immunoglobulin G4–related disease of the orbit.

MATERIALS AND METHODS: This study included 27 patients with biopsy-proved immunoglobulin G4–related disease of the orbit and either a CT or MR imaging of the orbits. These CT or MR imaging examinations were evaluated for the following: extraocular muscle size, extraocular muscle tendon enlargement, lacrimal gland enlargement, infiltrative process in the orbital fat (increased attenuation on CT or abnormal signal on MR imaging), infraorbital nerve enlargement, mucosal thickening in the paranasal sinuses, and extension of orbital findings intracranially.

RESULTS: Extraocular muscles were enlarged in 24 of 27 (89%) patients, 21 (88%) bilaterally. In 32 of 45 (71%) affected orbits, the lateral rectus was the most enlarged muscle. In 26 (96%) patients, the tendons of the extraocular muscles were spared. Nineteen (70%) patients had lacrimal gland enlargement. Twelve (44%) patients had an infiltrative process within the orbital fat. Infraorbital nerve enlargement was seen in 8 (30%) patients. Twenty-four (89%) patients had sinus disease. Cavernous sinus or Meckel cave extension was seen in 3 (11%) patients.

CONCLUSIONS: In patients with extraocular muscle enlargement, particularly when the tendons are spared and the lateral rectus is the most enlarged, and even more so when other noted findings are present, immunoglobulin G4–related disease should be a leading differential consideration, even over more commonly known etiologies of extraocular muscle enlargement.

### Abbreviations

IgG4
:   immunoglobulin G4" />
<meta name="og-title" property="og:title" content="Immunoglobulin G4–Related Disease of the Orbit: Imaging Features in 27 Patients" />
<meta name="og-url" property="og:url" content="http://ajnr-stage2.stage.highwire.org/content/early/2014/03/13/ajnr.A3865" />
<meta name="og-site-name" property="og:site_name" content="American Journal of Neuroradiology" />
<meta name="og-description" property="og:description" content="BACKGROUND AND PURPOSE: Immunoglobulin G4–related disease is a systemic fibroinflammatory process of unknown etiology, characterized by tissue infiltration by immunoglobulin G4 plasma cells. The purpose of this study was to retrospectively identify the spectrum of imaging features seen in immunoglobulin G4–related disease of the orbit.

MATERIALS AND METHODS: This study included 27 patients with biopsy-proved immunoglobulin G4–related disease of the orbit and either a CT or MR imaging of the orbits. These CT or MR imaging examinations were evaluated for the following: extraocular muscle size, extraocular muscle tendon enlargement, lacrimal gland enlargement, infiltrative process in the orbital fat (increased attenuation on CT or abnormal signal on MR imaging), infraorbital nerve enlargement, mucosal thickening in the paranasal sinuses, and extension of orbital findings intracranially.

RESULTS: Extraocular muscles were enlarged in 24 of 27 (89%) patients, 21 (88%) bilaterally. In 32 of 45 (71%) affected orbits, the lateral rectus was the most enlarged muscle. In 26 (96%) patients, the tendons of the extraocular muscles were spared. Nineteen (70%) patients had lacrimal gland enlargement. Twelve (44%) patients had an infiltrative process within the orbital fat. Infraorbital nerve enlargement was seen in 8 (30%) patients. Twenty-four (89%) patients had sinus disease. Cavernous sinus or Meckel cave extension was seen in 3 (11%) patients.

CONCLUSIONS: In patients with extraocular muscle enlargement, particularly when the tendons are spared and the lateral rectus is the most enlarged, and even more so when other noted findings are present, immunoglobulin G4–related disease should be a leading differential consideration, even over more commonly known etiologies of extraocular muscle enlargement.

### Abbreviations

IgG4
:   immunoglobulin G4" />
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      <div class="highwire-cite-overline"><span  class="highwire-cite-metadata-article-type highwire-cite-metadata">Research Article</span><span class="separator-pipe"></span><span  class="highwire-cite-metadata-art-cat highwire-cite-metadata"><span class="wrapper">HEAD &amp; NECK</span></span></div>
  
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      <h1 class="highwire-cite-title" id="page-title">Immunoglobulin G4–Related Disease of the Orbit: Imaging Features in 27 Patients</h1>  
    	<div class="highwire-cite-authors"><span  class="highwire-citation-authors"><span class="highwire-citation-author first" data-delta="0">C.A. Tiegs-Heiden</span>, <span class="highwire-citation-author" data-delta="1">L.J. Eckel</span>, <span class="highwire-citation-author" data-delta="2">C.H. Hunt</span>, <span class="highwire-citation-author" data-delta="3">F.E. Diehn</span>, <span class="highwire-citation-author" data-delta="4">K.M. Schwartz</span>, <span class="highwire-citation-author" data-delta="5">D.F. Kallmes</span>, <span class="highwire-citation-author" data-delta="6">D.R. Salomão</span>, <span class="highwire-citation-author" data-delta="7">T.E. Witzig</span> and <span class="highwire-citation-author" data-delta="8">J.A. Garrity</span></span></div>
  
    	<div class="highwire-cite-metadata"><span  class="highwire-cite-metadata-journal highwire-cite-metadata">American Journal of Neuroradiology </span><span  class="highwire-cite-metadata-date highwire-cite-metadata">March 2014,  </span><span  class="highwire-cite-metadata-doi highwire-cite-metadata">DOI: https://doi.org/10.3174/ajnr.A3865 </span></div>
  
  
  
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<div id="hw-article-author-popups-top-node-50567--6218131155" style="display: none;"><div class="author-tooltip-0"><div class="author-tooltip-name">C.A. Tiegs-Heiden </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'>From the Departments of Radiology (C.A.T.-H., L.J.E., C.H.H., F.E.D., K.M.S., D.F.K.), Pathology (D.R.S.), Hematology (T.E.W.), and Ophthalmology (D.R.S., J.A.G.), Mayo Clinic, Rochester, Minnesota.</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=C.A.%2BTiegs-Heiden%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Tiegs-Heiden%20C&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link last"><a href="/search/author1%3AC.A.%2BTiegs-Heiden%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li></ul></div><div class="author-tooltip-1"><div class="author-tooltip-name">L.J. Eckel </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'>From the Departments of Radiology (C.A.T.-H., L.J.E., C.H.H., F.E.D., K.M.S., D.F.K.), Pathology (D.R.S.), Hematology (T.E.W.), and Ophthalmology (D.R.S., J.A.G.), Mayo Clinic, Rochester, Minnesota.</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=L.J.%2BEckel%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Eckel%20L&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link last"><a href="/search/author1%3AL.J.%2BEckel%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li></ul></div><div class="author-tooltip-2"><div class="author-tooltip-name">C.H. Hunt </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'>From the Departments of Radiology (C.A.T.-H., L.J.E., C.H.H., F.E.D., K.M.S., D.F.K.), Pathology (D.R.S.), Hematology (T.E.W.), and Ophthalmology (D.R.S., J.A.G.), Mayo Clinic, Rochester, Minnesota.</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=C.H.%2BHunt%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Hunt%20C&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link last"><a href="/search/author1%3AC.H.%2BHunt%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li></ul></div><div class="author-tooltip-3"><div class="author-tooltip-name">F.E. Diehn </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'>From the Departments of Radiology (C.A.T.-H., L.J.E., C.H.H., F.E.D., K.M.S., D.F.K.), Pathology (D.R.S.), Hematology (T.E.W.), and Ophthalmology (D.R.S., J.A.G.), Mayo Clinic, Rochester, Minnesota.</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=F.E.%2BDiehn%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Diehn%20F&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link last"><a href="/search/author1%3AF.E.%2BDiehn%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li></ul></div><div class="author-tooltip-4"><div class="author-tooltip-name">K.M. Schwartz </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'>From the Departments of Radiology (C.A.T.-H., L.J.E., C.H.H., F.E.D., K.M.S., D.F.K.), Pathology (D.R.S.), Hematology (T.E.W.), and Ophthalmology (D.R.S., J.A.G.), Mayo Clinic, Rochester, Minnesota.</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=K.M.%2BSchwartz%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Schwartz%20K&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link last"><a href="/search/author1%3AK.M.%2BSchwartz%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li></ul></div><div class="author-tooltip-5"><div class="author-tooltip-name">D.F. Kallmes </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'>From the Departments of Radiology (C.A.T.-H., L.J.E., C.H.H., F.E.D., K.M.S., D.F.K.), Pathology (D.R.S.), Hematology (T.E.W.), and Ophthalmology (D.R.S., J.A.G.), Mayo Clinic, Rochester, Minnesota.</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=D.F.%2BKallmes%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Kallmes%20D&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link last"><a href="/search/author1%3AD.F.%2BKallmes%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li></ul></div><div class="author-tooltip-6"><div class="author-tooltip-name">D.R. 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<p id="p-1"><strong>BACKGROUND AND PURPOSE:</strong> Immunoglobulin G4–related disease is a systemic fibroinflammatory process of unknown etiology, characterized by tissue infiltration by immunoglobulin G4 plasma cells. The purpose of this study was to retrospectively identify the spectrum of imaging features seen in immunoglobulin G4–related disease of the orbit.</p></div>
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<p id="p-2"><strong>MATERIALS AND METHODS:</strong> This study included 27 patients with biopsy-proved immunoglobulin G4–related disease of the orbit and either a CT or MR imaging of the orbits. These CT or MR imaging examinations were evaluated for the following: extraocular muscle size, extraocular muscle tendon enlargement, lacrimal gland enlargement, infiltrative process in the orbital fat (increased attenuation on CT or abnormal signal on MR imaging), infraorbital nerve enlargement, mucosal thickening in the paranasal sinuses, and extension of orbital findings intracranially.</p></div>
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<p id="p-3"><strong>RESULTS:</strong> Extraocular muscles were enlarged in 24 of 27 (89%) patients, 21 (88%) bilaterally. In 32 of 45 (71%) affected orbits, the lateral rectus was the most enlarged muscle. In 26 (96%) patients, the tendons of the extraocular muscles were spared. Nineteen (70%) patients had lacrimal gland enlargement. Twelve (44%) patients had an infiltrative process within the orbital fat. Infraorbital nerve enlargement was seen in 8 (30%) patients. Twenty-four (89%) patients had sinus disease. Cavernous sinus or Meckel cave extension was seen in 3 (11%) patients.</p></div>
<div id="sec-4" class="subsection">
<p id="p-4"><strong>CONCLUSIONS:</strong> In patients with extraocular muscle enlargement, particularly when the tendons are spared and the lateral rectus is the most enlarged, and even more so when other noted findings are present, immunoglobulin G4–related disease should be a leading differential consideration, even over more commonly known etiologies of extraocular muscle enlargement.</p>
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<h3>Abbreviations</h3>
<dl id="def-list-1"><dt id="def-item-1">IgG4<span class="def-item-dt-sep"></span></dt><dd>immunoglobulin G4 </dd></dl>
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    	<div class="highwire-cite-metadata"><span  class="highwire-cite-metadata-journal highwire-cite-metadata">American Journal of Neuroradiology </span><span  class="highwire-cite-metadata-date highwire-cite-metadata">Mar 2014, </span><span  class="highwire-cite-metadata-doi highwire-cite-metadata"><span class="label">DOI:</span> 10.3174/ajnr.A3865 </span></div>
  
  
  
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      <div class="highwire-cite-title">Immunoglobulin G4–Related Disease of the Orbit: Imaging Features in 27 Patients</div>  
    	<div class="highwire-cite-authors"><span  class="highwire-citation-authors"><span class="highwire-citation-author first" data-delta="0"><span class="nlm-given-names">C.A.</span> <span class="nlm-surname">Tiegs-Heiden</span></span>, <span class="highwire-citation-author" data-delta="1"><span class="nlm-given-names">L.J.</span> <span class="nlm-surname">Eckel</span></span>, <span class="highwire-citation-author" data-delta="2"><span class="nlm-given-names">C.H.</span> <span class="nlm-surname">Hunt</span></span>, <span class="highwire-citation-author" data-delta="3"><span class="nlm-given-names">F.E.</span> <span class="nlm-surname">Diehn</span></span>, <span class="highwire-citation-author" data-delta="4"><span class="nlm-given-names">K.M.</span> <span class="nlm-surname">Schwartz</span></span>, <span class="highwire-citation-author" data-delta="5"><span class="nlm-given-names">D.F.</span> <span class="nlm-surname">Kallmes</span></span>, <span class="highwire-citation-author" data-delta="6"><span class="nlm-given-names">D.R.</span> <span class="nlm-surname">Salomão</span></span>, <span class="highwire-citation-author" data-delta="7"><span class="nlm-given-names">T.E.</span> <span class="nlm-surname">Witzig</span></span>, <span class="highwire-citation-author" data-delta="8"><span class="nlm-given-names">J.A.</span> <span class="nlm-surname">Garrity</span></span></span></div>
  
    	<div class="highwire-cite-metadata"><span  class="highwire-cite-metadata-journal highwire-cite-metadata">American Journal of Neuroradiology </span><span  class="highwire-cite-metadata-date highwire-cite-metadata">Mar 2014, </span><span  class="highwire-cite-metadata-doi highwire-cite-metadata"><span class="label">DOI:</span> 10.3174/ajnr.A3865 </span></div>
  
  
  
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