IgG4-related orbital disease: a meta-analysis and review

Abstract

ABSTRACT. IgG4-related orbital disease (IgG4-ROD) is a recently described condition that may account for a significant proportion of idiopathic lymphoplasmacytic or sclerotic orbital lesions. This study is the first meta-analysis of published cases and reveals several differences between IgG4-related disease affecting the orbit and that affecting the pancreas. IgG4-ROD affects a slightly younger group of patients, affects men and women approximately equally, is commonly associated with salivary gland lesions, is associated with a relatively higher serum IgG4 and may confer an increased risk of non-Hodgkin Lymphoma. Its pathogenesis may involve an immune response to antigen exposure in the upper aerodigestive tract.

Orbital inflammatory disease management

“…The disease can affect multiple tissues in the orbit, including the lacrimal gland, extraocular muscles, and soft tissue [21][22][23][24]. IgG4-RD is a relatively new entity, and although controversial, it may account or a sizeable proportion of what was previously thought to be idiopathic orbital inflammation.…”

Infraorbital nerve enlargement due to IgG4-related disease

“…1 Cranial nerve involvement, including IONE, has rarely been reported as part of the disease. [2][3][4] In a recent study, the incidence of IONE was significantly higher in patients with IgG4-related orbital disease than in patients with non-IgG4-related lymphoproliferative orbital disorders.…”

“…1 Cranial nerve involvement has rarely been reported. We describe a case of infraorbital nerve enlargement (IONE) due to IgG4-related disease.…”

Applying the consensus statement on the pathology of IgG4-related disease to lacrimal gland lesions

“…To our knowledge, only five lacrimal cases featuring obliterative phlebitis have been reported, 3 and only two of these had 4100 IgG4 þ cells/hpf. Obliterative phlebitis and storiform fibrosis are rare in both lacrimal and salivary IgG4-related disease, 1 and this distribution of organ involvement often occurs concurrently.…”

“…For lacrimal lesions, a cutoff of 100 IgG4 þ cells/hpf is proposed, markedly higher than the cutoff of 10 suggested in the comprehensive diagnostic criteria by Umehara et al 2 We recently undertook a meta-analysis of all published cases of IgG4-related orbital disease (n ¼ 133) and classified them using a cutoff of 10 IgG4 þ cells/hpf. 3 When the cases were reclassified using the diagnostic criteria proposed by Deshpande et al, 1 the resultant cohort had some interesting characteristics. Of the lacrimal cases with an IgG4 þ /IgG þ ratio 440% (n ¼ 72), 23 (32%) had 4100 IgG4 þ cells/hpf.…”