Background/Purpose:

IgG4-related disease (IgG4-RD) is an inflammatory disorder responsible for fibrosing, tumefactive lesions that can present in nearly any anatomic location.  The orbital manifestations of IgG4-RD, termed IgG4-related ophthalmic disease (IgG4-ROD), include lacrimal gland involvement in most cases.  However, the extraocular muscles, orbital soft tissues, sclera, and local nerves may also be affected.  We review the orbital manifestations of IgG4-RD based on the experience at our center and characterize the natural history, pathology, and treatment of these IgG4-RD complications. 

Methods:

We identified 27 patients in our IgG4-RD Registry with orbital manifestations.  Six cases were excluded because no pathology was available for review.  All 21 cases included had histopathologically-confirmed IgG4-RD diagnoses.  We then performed a retrospective medical records review. 

Results:

Patients with IgG4-ROD had a mean age of 50 years (range: 21-79) at the time of symptom onset and were approximately balanced with regard to gender distribution (male 57%, female 43%).The lacrimal gland, affected in 13/21 cases (62%) was the most commonly involved orbital structure.  Dacryoadenitis typically led to proptosis.  Most patients (71%) had bilateral ophthalmic disease as well as extra-orbital involvement.  The salivary glands, abnormal in 40% of those with IgG4-ROD, comprised the most common site of extra-orbital disease.  The average serum IgG4 concentration was 721 mg/dL (range: 28 to 4780; normal < 125).  The mean serum concentration was higher in patients with bilateral disease and in those with extra-orbital manifestations.  Among patients with dacryoadenitis, all were anti-Ro and anti-La negative.  The histopathological and immunostaining features of every biopsy specimen were consistent with consensus criteria.  Nine of 10 patients treated with rituximab responded to this therapy.

Conclusion:

Ophthalmic involvement is a common manifestation of IgG4-RD and can affect nearly every anatomic structure of the orbit.  Consideration of IgG4-RD and accurate diagnosis by biopsy have important implications for prognosis and treatment following the distinction of this condition from Sjögren syndrome (SjS), granulomatosis with polyangiitis (GPA, formerly Wegener’s), sarcoidosis, lymphoma, infection, and other disorders.  Rituximab holds promise as an effective steroid-sparing agent or therapy for steroid-resistant cases.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/ophthalmic-manifestations-of-igg4-related-disease-a-single-center-experience/