IgG4-related ophthalmic disease (IgG4-ROD) is the recommended term to describe orbital (eye socket) manifestations of the systemic condition IgG4-related disease, which is characterised by infiltration of

lymphocyte A lymphocyte is a type of white blood cell (leukocyte) in the immune system of most vertebrates. Lymphocytes include natural killer cells (which function in cell-mediated, cytotoxic innate immunity), T cells (for cell-mediated, cytotoxic ad ...

s and plasma cells and subsequent

fibrosis Fibrosis, also known as fibrotic scarring, is a pathological wound healing in which connective tissue replaces normal parenchymal tissue to the extent that it goes unchecked, leading to considerable tissue remodelling and the formation of perma ...

in involved structures. It can involve one or more of the orbital structures. Frequently involved structures include the

lacrimal gland The lacrimal glands are paired exocrine glands, one for each eye, found in most terrestrial vertebrates and some marine mammals, that secrete the aqueous layer of the tear film. In humans, they are situated in the upper lateral region of each or ...

s, extraocular muscles,

infraorbital nerve The infraorbital nerve is a branch of the maxillary nerve, itself a branch of the trigeminal nerve (CN V). It travels through the orbit and enters the infraorbital canal to exit onto the face through the infraorbital foramen. It provides sensory ...

, supraorbital nerve and

eyelid An eyelid is a thin fold of skin that covers and protects an eye. The levator palpebrae superioris muscle retracts the eyelid, exposing the cornea to the outside, giving vision. This can be either voluntarily or involuntarily. The human eye ...

s. It has also been speculated that ligneous conjunctivitis may be a manifestation of

IgG4-related disease IgG4-related disease (IgG4-RD), formerly known as IgG4-related systemic disease, is a chronic inflammatory condition characterized by tissue infiltration with lymphocytes and IgG4-secreting plasma cells, various degrees of fibrosis (scarring) and ...

(IgG4-RD). As is the case with other manifestations of

, a prompt response to steroid therapy is a characteristic feature of IgG4-ROD in most cases, unless significant fibrosis has already occurred. It basically can cause loss of vision and in other cases, cause skin irritation and loss of brain function

Symptoms

Breakdown of diagnosis in orbital lymphoproliferative disorders

Symptoms, if any, can be mild even in the presence of significant swelling or masses. Lacrimal gland involvement may cause swelling of the upper eyelid, or

proptosis Exophthalmos (also called exophthalmus, exophthalmia, proptosis, or exorbitism) is a bulging of the eye anteriorly out of the orbit. Exophthalmos can be either bilateral (as is often seen in Graves' disease) or unilateral (as is often seen i ...

if there is severe swelling. Other orbital masses or inflammation can result in visual disturbance (blurred vision, double vision, visual field impairment), restricted eye movements, pain or discomfort, numbness in the distribution of the supraorbital and/or infraorbital nerves, or

. IgG4-related ophthalmic disease has been estimated to account for approximately 25% of all cases of proptosis, eyelid swelling and other features of orbital swelling.

Cause

Diagnosis

The extent of inflammation that can occur in IgG4-ROD is well demonstrated on magnetic resonance imaging (MRI). Infraorbital nerve enlargement (IONE) is considered to be a particularly suspicious sign of IgG4-ROD, but seems to occur only when inflammation is in direct contact with the

infraorbital canal The infraorbital canal is a canal found at the base of the orbit that opens on to the maxilla. It is continuous with the infraorbital groove and opens onto the maxilla at the infraorbital foramen. The infraorbital nerve and infraorbital arte ...

. IONE is defined as the

diameter being greater than the

optic nerve In neuroanatomy, the optic nerve, also known as the second cranial nerve, cranial nerve II, or simply CN II, is a paired cranial nerve that transmits visual information from the retina to the brain. In humans, the optic nerve is derived fro ...

diameter in the

coronal plane The coronal plane (also known as the frontal plane) is an anatomical plane that divides the body into dorsal and ventral sections. It is perpendicular to the sagittal and transverse planes. Details The coronal plane is an example of a longit ...

Management

Nomenclature

Although IgG4-related ophthalmic disease is the recommended name for all orbital manifestations of

, more precise terminology for the various anatomical variations of the condition can be used. This includes: * IgG4-related dacryoadenitis (

s) * IgG4-related dacryocystitis (

lacrimal sac The lacrimal sac or lachrymal sac is the upper dilated end of the nasolacrimal duct, and is lodged in a deep groove formed by the lacrimal bone and frontal process of the maxilla. It connects the lacrimal canaliculi, which drain tears from the ...

) * IgG4-related orbital myositis ( extraocular muscles) * IgG4-related orbital nerve lesions * IgG4-related optic neuropathy ( optic nerve) * IgG4-related orbital inflammation (orbital soft tissue) * IgG4-related pan-orbital inflammation (all of the various types of orbital structures) Names previously used in the diagnosis of cases now considered to be IgG4-ROD have included

idiopathic orbital inflammatory disease Idiopathic orbital inflammatory (IOI) disease refers to a marginated mass-like enhancing soft tissue involving any area of the orbit. It is the most common painful orbital mass in the adult population, and is associated with proptosis, cranial ner ...

and orbital pseudotumor.

References

External links

{{Medical resources , ICD10 = {{ICD10, H05.1 , MeSH = , Orphanet = 449563 IgG4-related disease Eye diseases Steroid-responsive inflammatory conditions