IgG4-Related Disease

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is a multifocal inflammatory disorder that causes tumefactive lesions with a dense lymphoplasmacytic infiltrate rich in IgG4 plasma cells and storiform-pattern fibrosis. The clinical symptoms are relatively mild, and the condition is usually recognized by organ swelling and damage. When referring to the ophthalmic manifestations of IgG4-RD, the term IgG4-related ophthalmic disease (IgG4-ROD) is used. IgG4-ROD is characterized by bilateral lacrimal gland enlargement accompanied by 3 distinctive features: infraorbital nerve enlargement, extraocular myositis, and compressive optic neuropathy. IgG4 implies an underlying systemic disease process requiring evaluation to detect other systemic involvement. This includes hypophysitis and hypertrophic pachymeningitis, entities of neuro-ophthalmic interest. IgG4-ROD usually responds favorably to systemic corticosteroids but may be complicated by relapse during steroid taper. Rituximab has been shown to be effective for controlling steroid-refractory IgG4-RD. In contrast to IgG4-RD, an increasing number of cases of extranodal marginal B-cell lymphoma (MALT type) associated with IgG4-ROD have been described. IgG4 may be a risk factor for later emergence of low-grade B-cell lymphoma.

Magnetic resonance imaging indicator of the causes of optic neuropathy in IgG4-related ophthalmic disease

“…Previous data have suggested that IgG4-RD has a particular tropism for cranial nerves [24–26]. In addition, it has been reported that IONE can oppress the optic nerve at the orbital apex [27]. However, in this study, we observed no IONE oppression related cases, while most oppressions were caused by pseudo tumor.…”

Colon cancer and IgG4-related disease with orbital inflammation and bilateral optic perineuritis

“…[ 28 ] On the contrary, optic nerve involvement is uncommon in IgG4-RD. [ 29 – 34 ] It is most often unilateral [ 35 ] and due to compression of the optic nerve by enlarged orbital structures affected by IgG4-ROD; [ 36 – 38 ] in a series of patients with IgG4-ROD, compressive optic neuropathy was found on orbital imaging in 9.2% of the cases. [ 35 ] In some cases a meningeal infiltration of the optic nerve is found on imaging, suggesting perineuritis.…”

“…[ 32 ] In view of this only case, it seems that IgG4-related optic neuropathy may rarely be due to direct infiltration of the optic nerve by IgG4-related inflammation (by analogy with what is found in the biopsies of the branches of the trigeminal nerve, but this hypothesis is subject to confirmation by an actual optic nerve biopsy). [ 36 ]…”

Optic nerve involvement in immunoglobulin G4-related disease: A case report

“…Orbital involvement in IgG4-RD has been previously reported to affect nearly every orbital structure, including lacrimal glands, extra-ocular muscles and the trigeminal nerve ( 16 , 17 ). The term IgG4-related ophthalmic disease (IgG4-ROD) has been suggested for IgG4-RD with ophthalmic involvement ( 18 ). According to Kashii ( 18 ), IgG4-ROD is characterized by bilateral lacrimal gland enlargement accompanied by three distinctive features, including infraorbital nerve enlargement, extraocular myositis and compressive optic neuropathy.…”

“…The term IgG4-related ophthalmic disease (IgG4-ROD) has been suggested for IgG4-RD with ophthalmic involvement ( 18 ). According to Kashii ( 18 ), IgG4-ROD is characterized by bilateral lacrimal gland enlargement accompanied by three distinctive features, including infraorbital nerve enlargement, extraocular myositis and compressive optic neuropathy. Comparing the characteristics of the patient of the current study to this classification, we note that an upper eyelid mass was present, which was consistent with lacrimal gland enlargement; however, this disappeared spontaneously and prior to methylprednisolone treatment.…”