The disease is represented in a broad clinical classification of idiopathic orbital inflammation termed by Birch-Hirchfield in 1905 as an
orbital pseudotumor condition.
By contrast, patients with "
orbital pseudotumor" or "idiopathic orbital inflammation," which is not related to IgG4, tend to be unilateral and usually present with an acute onset of pain, erythema, proptosis, and restriction of ocular motility (41).
Idiopathic inflammatory
orbital pseudotumor in childhood.
In adults, it is commonly related to Graves' ophthalmopathy,
orbital pseudotumor, and rhinosinusitis-related orbital infections.
Orbital pseudotumor is an important differentiating pathology.
Herpes zoster ophthalmicus with
orbital pseudotumor syndrome complicated by optic nerve infarction and cerebral granulomatous angiitis: MR-pathologic correlation.
Patients with IgG4-related ophthalmic disease usually involve the lacrimal gland and may cause diseases such as
orbital pseudotumor or sclerosing dacryoadenitis.
Idiopathic
orbital pseudotumor is characterized by the presence of non-granulomatous inflammation in the eye without a known systemic or local reason.
Pathologic involvement beyond the cavernous sinus, superior orbital fissure, or apex of the orbit occurs rarely, and the disorder is part of a continuum with idiopathic
orbital pseudotumor, with which it shares histopathology features.
Orbital pseudotumor. Am J Clin Oncol 2010; 33:304-306.
Painful ophthalmoplegia is the consequence of the mass effect on the cavernous sinus which includes trauma, vascular malformation, neoplasm, infection, and inflammation including Tolosa-Hunt syndrome, Wegener's granulomatosis, or
orbital pseudotumor [6].
Marenco de la Fuente, "Resistant
orbital pseudotumor treated with rituximab in a patient with systemic lupus erythematosus.