Orbital IgG4-Related Disease: Clinical Features and Diagnosis

Abstract

Orbital IgG4-related disease, which can occur in adults of any age, is characterized by IgG4-positive lymphoplasmacytic infiltrations in ocular adnexal tissues. The signs and symptoms include chronic noninflammatory lid swelling and proptosis. Patients often have a history of allergic disease and elevated serum levels of IgG4 and IgE as well as hypergammaglobulinemia. Orbital IgG4-related disease must be differentiated from idiopathic orbital inflammation and ocular adnexal marginal zone B-cell lymphoma to ensure appropriate and effective treatment. Systemic steroid therapy decreases the size of the lesions, but relapse often occurs when systemic steroid therapy is discontinued.

Orbital Manifestations of Immunoglobulin G4–Related Disease in Bilateral Lacrimal Glands, Optic Nerves, Trigeminal Nerves, and Maxillary Sinuses

“…Its signs and symptoms include lid and periorbital swelling, which are mostly painless. Some patients have proptosis and ocular motility restrictions; few patients have visual loss [1][2][3]6 ; and patients commonly have bilateral involvement. 1,3 To date, scattered cases of orbital IgG4-RD with lesions involving the lacrimal glands, extraocular muscles, trigeminal nerves, cavernous sinuses, and optic nerve sheaths have been reported.…”

Orbital IgG4-associated diseases

“…In literature, one can find significant differences in medical procedures, particularly between Japanese and American physicians. Japanese groups recommend starting daily doses of prednisone around 0.6-1 mg/kg for 2-4 weeks [1,8,14,17,21] followed by gradual lowering of the dose during the next 3-6 months based on clinical response to 5 mg/day and subsequent long-term treatment with 2.5-5 mg/day for next 3 years. Treatment recommended by the Mayo Clinic starts with 40 mg/day of prednisone for 30 days followed by gradual decrease of the dose by 5 mg for next 2 months and ending the treatment after 11-12 weeks [16].…”

“…In 1892, Johan von Mikulicz-Radecki first described clinical manifestations in a case report of a 42-year-old farmer with symmetric bilateral lacrimal, parotid and submandibular gland enlargement associated with lymphocytic infiltration [1][2][3]. Since then, there have been additional cases of patients with the same clinical manifestation, labeled Mikulicz disease.…”

“…In 2001, several studies revealed a connection between Mikulicz disease and high levels of IgG4, which significantly lowered after glucocorticoid therapy [1,2,[6][7][8][9]. Based on these observations, Mikulicz disease is now classified as IgG4-related disease (IgG4-RD) [10].…”

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.…”